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Yang YH, Zhang YM, Liu AH, Luo GC, Liu ZJ, Xie JW. Perinephric myxoid pseudotumor of fat: the first case report in China with an updated literature review. Front Oncol 2025; 15:1543068. [PMID: 40308488 PMCID: PMC12041072 DOI: 10.3389/fonc.2025.1543068] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2024] [Accepted: 03/27/2025] [Indexed: 05/02/2025] Open
Abstract
The perinephric myxoid pseudotumor of fat (PMPF) is an uncommon benign neoplasm characterized by a favorable prognosis. To date, 21 cases of PMPFs have been reported worldwide, and their detailed characteristics have not been fully elucidated. We report the 22nd case of PMPF, which is the first case in China and happened in a 54-year-old male with a 5.6 cm mass located in the lower inner aspect of the right kidney. The patient underwent a minimally invasive robot-assisted laparoscopic resection of the renal mass. Postoperative histopathological, immunohistochemical, and genetic testing results confirmed the diagnosis of PMPF. No evidence of recurrence was found during a follow-up period of 6 months postoperatively based on clinical and imaging data. This is a report of an unusual case of PMPF with an up-to-date review. According to the latest systematic review and clinical confirmation, PMPF is strongly associated with chronic kidney disease, which is further confirmed by our case. For PMPF, combining imaging methods and immunohistochemical staining is suggested, which may prove beneficial in clinical practice.
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Affiliation(s)
- Yu-Hang Yang
- Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Yue-Ming Zhang
- Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - An-Hao Liu
- Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Guang-Cheng Luo
- Department of Urology, Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Zheng-Jin Liu
- Department of Pathology, Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Jian-Wei Xie
- Department of Urology, Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
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Tanemoto F, Mimura I, Abe H, Nangaku M. A case of PLA2R-positive membranous nephropathy with subsequent development of IgG4-related disease. CEN Case Rep 2025; 14:280-290. [PMID: 39514162 PMCID: PMC11958855 DOI: 10.1007/s13730-024-00941-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2024] [Accepted: 10/07/2024] [Indexed: 11/16/2024] Open
Abstract
Membranous nephropathy (MN) is a common cause of adult-onset nephrotic syndrome. It is also known as a minor but established renal manifestation of Immunoglobulin G4-related disease (IgG4-RD). Previous reports suggest that MN can also be an initial manifestation of IgG4-RD, all of which are phospholipase A2 receptor (PLA2R)-negative MN. We describe a case of PLA2R-positive MN that subsequently developed other manifestations of IgG4-RD. A 60-year-old male with nephrotic syndrome was diagnosed as primary MN with positive staining for PLA2R on the initial renal biopsy, which remained in partial remission with supportive therapy using angiotensin II receptor blocker (ARB) without steroid. About 1 year later, a renal mass was detected during an annual checkup, and contrast-enhanced computed tomography revealed low-density masses in bilateral kidneys and the head of the pancreas. The findings of endoscopic biopsy of the pancreatic mass were consistent with autoimmune pancreatitis (AIP) and the second renal biopsy showed the findings of MN with tubulointerstitial nephritis, both of which led to a diagnosis of IgG4-RD. The second renal biopsy also showed positive PLA2R. The patient received oral glucocorticoid therapy for IgG4-RD, which improved IgG4-related AIP and renal masses and also resulted in complete remission of MN. To our knowledge, this is the first reported case of PLA2R-positive MN with subsequent development of IgG4-RD. It is sometimes difficult to determine whether PLA2R-positive MN occurring with IgG4-RD is primary MN or secondary MN associated with IgG4-RD. The possibility of developing IgG4-RD should be considered even when preceding MN is PLA2R-positive, suggesting of primary MN.
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Affiliation(s)
- Fumiaki Tanemoto
- Division of Nephrology and Endocrinology, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Imari Mimura
- Division of Nephrology and Endocrinology, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
| | - Hiroyuki Abe
- Division of Pathology, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Masaomi Nangaku
- Division of Nephrology and Endocrinology, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan
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Minezaki C, Uchida H, Todaka K, Koumatsu N, Okita J, Kudo A, Nakata T, Fukuda A, Fukunaga N, Uesugi N, Shibata H. An Atypical Cystic Renal Mass in a Patient with IgG4-Related Kidney Disease: A Case Report. Intern Med 2025:4691-24. [PMID: 40159154 DOI: 10.2169/internalmedicine.4691-24] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 04/02/2025] Open
Abstract
We herein report a case of IgG4-related kidney disease (IgG4-RKD) with an atypical cystic renal mass in a 73-year-old man. Computed tomography (CT) revealed diffuse renal enlargement and a cystic mass in the left kidney. Blood tests revealed elevated IgG and IgG4 levels. A renal biopsy led to the IgG4-RKD diagnosis. The cystic mass was deemed to be benign based on the clinical, imaging, and ultrasonography findings. No malignancy or abscess formation was noted. Both the renal function and IgG4 levels improved after steroid treatment; moreover, the mass shrunk, suggesting an IgG4-RKD etiology. Cystic renal masses with capsule formation should be considered in patients presenting with IgG4-RKD.
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Affiliation(s)
- Chisato Minezaki
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan
| | - Hiroki Uchida
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan
| | - Kouhei Todaka
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan
| | - Nobuchika Koumatsu
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan
| | - Jun Okita
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan
| | - Akiko Kudo
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan
| | - Takeshi Nakata
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan
| | - Akihiro Fukuda
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan
| | - Naoya Fukunaga
- Department of Nephrology, Oita Prefectural Hospital, Japan
| | - Noriko Uesugi
- Department of Pathology, Fukuoka University of Medicine, Japan
| | - Hirotaka Shibata
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan
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Kudsi M, Tarcha R, Khalayli N, Rabah N, Rabah K, Alghawe FA. Progression to end-stage renal disease due to IgG4-related nephritis: a case report. Oxf Med Case Reports 2025; 2025:omae179. [PMID: 39839693 PMCID: PMC11748429 DOI: 10.1093/omcr/omae179] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2023] [Revised: 08/26/2024] [Accepted: 11/11/2024] [Indexed: 01/23/2025] Open
Abstract
IgG4-related disease (IgG4-RD) is a rare but increasingly recognised condition that can involve multiple organs, including the kidneys which often presents as tubulointerstitial nephritis. Treatment with glucocorticoids is the first line of therapy, but other options may be needed in refractory cases. This case report explores a 68-year-old female, diagnosed with the patient initially responded to glucocorticoids but had a relapse, leading to progressive renal insufficiency and ultimately death. Our case is a rare case observing the progression to end-stage kidney disease from IgG4-RD, and the first case of which the patient had died in a short period.
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Affiliation(s)
- Maysoun Kudsi
- Professor of Rheumatology, Faculty of Medicine, Damascus University, Damascus, Syria
| | - Raghad Tarcha
- Department of Rheumatology, Faculty of Medicine, Damascus University, Damascus, Syria
| | - Naram Khalayli
- Faculty of Medicine, Damascus University, Damascus, Syria
| | - Nour Rabah
- 6 year medical student, Faculty of Medicine, Syrian Private University, Syria
| | - Karam Rabah
- Faculty of Medicine, Syrian Private University, Syria
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Kawano M. IgG4-related Disease: Recent Topics on Immunological Aspects of This Disorder and Their Application in New Treatment Strategies. Intern Med 2025; 64:31-39. [PMID: 38369350 PMCID: PMC11781911 DOI: 10.2169/internalmedicine.3154-23] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/01/2023] [Accepted: 12/17/2023] [Indexed: 02/20/2024] Open
Abstract
IgG4-related disease (IgG4-RD) is a systemic and chronic inflammatory disorder that can affect every part of the body. The formation of tertiary lymphoid tissues (TLT) in the affected organs may be a key phenomenon in understanding the pathogenesis of this disease because T follicular helper (Tfh) 2 cells play an important role in IgG4 class switching within TLT in the affected organs or tissues. TLT formation leads to the formation of masses or swelling of the affected organs. Interleukin (IL)-4 and IL-10 are critical cytokines for IgG4-class switching and are produced in TLT. Other factors, such as CD4-positive (CD4+) cytotoxic T cells, M2 macrophages, and LAG3+ Tfh cells, have been identified as disease-specific contributors to lesion formation. In this review, I describe the current knowledge necessary to understand the pathogenesis of this disease and recent developments in treatment strategies beyond B-cell depletion therapy.
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Affiliation(s)
- Mitsuhiro Kawano
- Innovative Clinical Research Center, Department of Nephrology and Rheumatology, Kanazawa University Hospital, Japan
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Vaquer-Grimalt P, García IM, Antón E, Escarda A, Vanrell M, Bonet L, Sastre L. IgG4-related disease mimicking a liver abscess. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2024; 116:707-708. [PMID: 38305673 DOI: 10.1007/978-3-031-60855-1_5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
Abstract
IgG4-related disease (IGRD) is a complex medical condition affecting multiple organs, including the liver. The condition is characterized by excessive production of IgG4 antibodies, leading to chronic inflammation and tissue damage. We present a case of a 37-year-old man with a history of chronic pancreatitis was diagnosed with a liver mass. Initial treatment included piperacillin and tazobactam, but the patient's condition worsened. An ultrasound-guided biopsy revealed increased IgG4 positive cells, leading to the diagnosis of an inflammatory pseudotumor associated with IGRD. The patient was treated with prednisone taper therapy, and the liver mass resolved after six months of corticoid treatment.
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Affiliation(s)
| | | | - Ester Antón
- Aparato Digestivo, Hospital Universitario Son Espases, España
| | - Aina Escarda
- Hepatología, Hospital Universitario Son Espases, España
| | | | - Lucía Bonet
- Hepatología, Hospital Universitario Son Espases, España
| | - Lydia Sastre
- Hepatología, Hospital Universitario Son Espases, España
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Yamamoto M, Kanda M, Mizushima I, Kanno A, Umemura T, Ikeura T, Kodama Y, Dobashi H, Tanaka Y, Masamune A, Moriyama M, Saeki T, Matsui S, Origuchi T, Masaki Y, Asada M, Umehara H, Seno H, Naitoh I, Yamamoto S, Iwasaki E, Kubota K, Tanoue S, Nishino T, Tsuboi H, Matsumoto Y, Isayama H, Goto H, Notohara K, Uchida K, Kawabe K, Yamada K, Kasashima S, Takahira M, Sato Y, Kawachi I, Yamaguchi I, Okazaki K, Nakamura S, Matsuda F, Ishikawa H, Kawano M. Clinical profile of IgG4-related disease in Japan based on the rare disease data registry. Immunol Med 2024:1-11. [PMID: 39607031 DOI: 10.1080/25785826.2024.2430812] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2024] [Accepted: 10/14/2024] [Indexed: 11/29/2024] Open
Abstract
We started a registry for cases of immunoglobulin (Ig)G4-related disease (IgG4-RD) in December 2019 to clarify the clinical profile of IgG4-RD. In this study, clinical information from 854 cases registered by February 16, 2024 was analyzed from multiple perspectives. Diagnosis of IgG4-RD was made in 808 cases, comprising 638 definite, 38 probable, and 132 possible. The mean ± SD age at time of enrollment of the 808 cases was 67.9 ± 11.3 years, with 68.8% being male. The pancreas was the most frequently affected organ (49.8%), followed by the submandibular glands (46.2%) and lacrimal glands (30.6%). This study reconfirmed the pancreas and head-and-neck region as major affected areas in IgG4-RD. Clinically, submandibular adenitis and autoimmune pancreatitis often occur together in the same patient, but no association between the two organs was observed in our analysis. Regarding diagnosis, the comprehensive diagnostic criteria were most commonly used (63.6%). Storiform fibrosis and phlebitis obliterans were detected at different frequencies in different organs. In summary, this registry study identified clinical, imaging, hematologic, and pathologic findings in 808 Japanese patients with IgG4-RD. The frequency of affected organs and their characteristic pathological findings will be particularly useful for future practice.
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Affiliation(s)
- Motohisa Yamamoto
- The Institute of Medical Science, The University of Tokyo, Tokyo, Japan
| | | | | | | | | | | | | | | | - Yoshiya Tanaka
- University of Occupational and Environmental Health, Fukuoka, Japan
| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | - Takayoshi Nishino
- Tokyo Women's Medical University Yachiyo Medical Center, Chiba, Japan
| | | | - Yasushi Matsumoto
- National Hospital Organization Kanazawa Medical Center, Ishikawa, Japan
| | | | | | - Kenji Notohara
- Kurashiki Central Hospital, Ohara Health Care Foundation, Okayama, Japan
| | | | - Ken Kawabe
- National Hospital Organization Kyushu Medical Center, Fukuoka, Japan
| | | | | | | | | | | | | | | | | | | | | | - Mitsuhiro Kawano
- Kanazawa University, Ishikawa, Japan
- Kanazawa Medical University, Ishikawa, Japan
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8
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Towheed ST, Zanjir W, Ren KYM, Garland J, Clements-Baker M. Renal Manifestations of IgG4-Related Disease: A Concise Review. Int J Nephrol 2024; 2024:4421589. [PMID: 38957780 PMCID: PMC11217581 DOI: 10.1155/2024/4421589] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2023] [Revised: 04/28/2024] [Accepted: 06/10/2024] [Indexed: 07/04/2024] Open
Abstract
IgG4-related disease (IgG4-RD) is an immune-mediated disorder marked by fibro-inflammatory masses that can infiltrate multiple organ systems. Due to its relatively recent discovery and limited understanding of its pathophysiology, IgG4-related disease may be difficult to recognize and is consequently potentially underdiagnosed. Renal involvement is becoming regarded as one of the key features of this disease. To date, the most well-recognized renal complication of IgG4-related disease is tubulointerstitial nephritis, but membranous glomerulonephritis, renal masses, and retroperitoneal fibrosis have also been reported. This concise review has two objectives. First, it will briefly encapsulate the history, epidemiology, and presentation of IgG4-related disease. Second, it will examine the reported renal manifestations of IgG4-related disease, exploring the relevant histology, imaging, clinical features, and treatment considerations. This synthesis will be highly relevant for nephrologists, rheumatologists, general internists, and renal pathologists to raise awareness and help improve early recognition of IgG4-related kidney disease (IgG4-RKD).
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Luo S, Guo L, Yang Z, Shen R, Zhang T, Wang M, Zhou Q, Wang H, Li X, Chen J, Wang R. Deciphering three predominant biopsy-proven phenotypes of IgG4-associated kidney disease: a retrospective study. Clin Kidney J 2024; 17:sfae111. [PMID: 38783966 PMCID: PMC11114465 DOI: 10.1093/ckj/sfae111] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/24/2023] [Indexed: 05/25/2024] Open
Abstract
Background IgG4-associated kidney disease (IgG4-RKD) encompasses a spectrum of disorders, predominantly featuring tubulointerstitial nephritis (TIN) and membranous glomerulonephropathy (MGN). The limited understanding of the co-occurrence of IgG4-RD-TIN with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) poses a diagnostic and therapeutic challenge. Methods We examined 49 cases, comprising 21 cases of IgG4-RD-TIN (group A), 10 cases of IgG4-RD-TIN accompanied with MGN (group B), and 18 cases of IgG4-RD-TIN concurrent with AAV (group C), at the First Affiliated Hospital of Zhejiang University, China, from June 2015 to December 2022. Results The mean age and gender of the three IgG4-RKD subtypes were not statistically significant. IgG4-RD-TIN exhibited higher serum creatinine and a higher incidence of hypocomplementemia (group A 47.6%, group B 30%, group C 16.7%). IgG4-RD-TIN-MGN was characterized by proteinuria (group A 0.3 g/d, group B 4.0 g/d, group C 0.8 g/d, P < 0.001) and hypoalbuminemia. IgG4-RD-TIN-AAV exhibited hypohemoglobinemia (group A 103.45 g/l, group B 119.60 g/l, group C 87.94 g/l, P < 0.001) and a high level of urine erythrocytes. The primary treatment for IgG4-RD-TIN was steroids alone, whereas IgG4-RD-TIN-MGN and IgG4-RD-TIN-AAV necessitated combination therapy. Group A experienced two relapses, whereas groups B and C had no relapses. There was no significant difference in patient survival among the three groups, and only two cases in group C suffered sudden death. Conclusions This study provides valuable insights into clinical manifestations, auxiliary examination features, pathological characteristics, and prognosis of IgG4-RD-TIN, IgG4-RD-TIN-MGN, and IgG4-RD-TIN concurrent AAV. Large-scale studies are required to validate these findings.
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Affiliation(s)
- Sulin Luo
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Key Laboratory of Kidney Disease Prevention and Control Technology, Zhejiang Province, China
- National Key Clinical Department of Kidney Diseases, China
- Institute of Nephrology, Zhejiang University, Hangzhou, China
- Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Hangzhou, China
| | - Luying Guo
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Key Laboratory of Kidney Disease Prevention and Control Technology, Zhejiang Province, China
- National Key Clinical Department of Kidney Diseases, China
- Institute of Nephrology, Zhejiang University, Hangzhou, China
- Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Hangzhou, China
| | - Zhenzhen Yang
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Department of Nephrology, Huzhou Central Hospital, Huzhou, Zhejiang Province, China
| | - Rongfang Shen
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Affiliated Hospital of Shaoxing University, Shaoxing, Zhejiang Province, China
| | - Tianlu Zhang
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Key Laboratory of Kidney Disease Prevention and Control Technology, Zhejiang Province, China
- National Key Clinical Department of Kidney Diseases, China
- Institute of Nephrology, Zhejiang University, Hangzhou, China
- Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Hangzhou, China
| | - Meifang Wang
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Key Laboratory of Kidney Disease Prevention and Control Technology, Zhejiang Province, China
- National Key Clinical Department of Kidney Diseases, China
- Institute of Nephrology, Zhejiang University, Hangzhou, China
- Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Hangzhou, China
| | - Qin Zhou
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Key Laboratory of Kidney Disease Prevention and Control Technology, Zhejiang Province, China
- National Key Clinical Department of Kidney Diseases, China
- Institute of Nephrology, Zhejiang University, Hangzhou, China
- Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Hangzhou, China
| | - Huiping Wang
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Key Laboratory of Kidney Disease Prevention and Control Technology, Zhejiang Province, China
- National Key Clinical Department of Kidney Diseases, China
- Institute of Nephrology, Zhejiang University, Hangzhou, China
- Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Hangzhou, China
| | - Xiayu Li
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Key Laboratory of Kidney Disease Prevention and Control Technology, Zhejiang Province, China
- National Key Clinical Department of Kidney Diseases, China
- Institute of Nephrology, Zhejiang University, Hangzhou, China
- Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Hangzhou, China
| | - Jianghua Chen
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Key Laboratory of Kidney Disease Prevention and Control Technology, Zhejiang Province, China
- National Key Clinical Department of Kidney Diseases, China
- Institute of Nephrology, Zhejiang University, Hangzhou, China
- Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Hangzhou, China
| | - Rending Wang
- Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Key Laboratory of Kidney Disease Prevention and Control Technology, Zhejiang Province, China
- National Key Clinical Department of Kidney Diseases, China
- Institute of Nephrology, Zhejiang University, Hangzhou, China
- Zhejiang Clinical Research Center of Kidney and Urinary System Disease, Hangzhou, China
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10
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Needleman A, Sheaff M, Pepper RJ, Evans RDR. Immunoglobulin G4-related disease presenting with nephrotic syndrome due to minimal change disease: a case report. J Med Case Rep 2024; 18:192. [PMID: 38641649 PMCID: PMC11031906 DOI: 10.1186/s13256-024-04494-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2023] [Accepted: 03/06/2024] [Indexed: 04/21/2024] Open
Abstract
BACKGROUND Immunoglobulin G4-related disease is an inflammatory disease affecting multiple organs including the kidney. Immunoglobulin G4-related kidney disease most commonly manifests as a tubulointerstitial nephritis and is associated with glomerular disease in a proportion of cases. Membranous nephropathy is the most frequent glomerular lesion. Herein, we report the first documented case of immunoglobulin G4-related disease presenting with nephrotic syndrome owing to minimal change disease. CASE PRESENTATION A 67-year-old South Asian male presented to our service with systemic upset and leg swelling. He had heavy proteinuria (urine protein:creatinine ratio 1042 mg/mmol) and was hypoalbuminemic (17 g/L) and hypercholersterolemic (9.3 mmol/L), consistent with the nephrotic syndrome. His serum creatinine was 140 μmol/L, and he was hypocomplementemic (C3 0.59 g/L, C4 < 0.02 g/L) with raised immunoglobulin G4 subclass levels (5.29 g/L). Kidney biopsy demonstrated minimal change disease alongside a plasma-cell-rich tubulointerstitial nephritis with strong positive staining for immunoglobulin G4. A diagnosis of minimal change disease in the setting of immunoglobulin G4-related disease was made. He was commenced on oral prednisolone at 60 mg daily but suffered infectious complications, including necrotizing fasciitis within 3 weeks of starting treatment, ultimately resulting in his death 52 days after initial presentation. CONCLUSION This case highlights the potential for immunoglobulin G4-related disease to be associated with a spectrum of glomerular pathologies including minimal change disease. It adds to the differential diagnosis of secondary causes of minimal change disease, and moreover, aids as an important reminder of the potential complications of high-dose steroids used in its treatment.
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Affiliation(s)
- Amy Needleman
- UCL Centre for Kidney and Bladder Health, Royal Free Hospital, Pond Street, London, NW3 2QG, UK
| | - Michael Sheaff
- Department of Histopathology, Bart's Health NHS Trust, London, UK
| | - Ruth J Pepper
- UCL Centre for Kidney and Bladder Health, Royal Free Hospital, Pond Street, London, NW3 2QG, UK
| | - Rhys D R Evans
- UCL Centre for Kidney and Bladder Health, Royal Free Hospital, Pond Street, London, NW3 2QG, UK.
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11
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Czarnywojtek A, Agaimy A, Pietrończyk K, Nixon IJ, Vander Poorten V, Mäkitie AA, Zafereo M, Florek E, Sawicka-Gutaj N, Ruchała M, Ferlito A. IgG4-related disease: an update on pathology and diagnostic criteria with a focus on salivary gland manifestations. Virchows Arch 2024; 484:381-399. [PMID: 38316669 DOI: 10.1007/s00428-024-03757-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/01/2023] [Revised: 01/12/2024] [Accepted: 01/29/2024] [Indexed: 02/07/2024]
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ disorder characterized by a highly variable clinical presentation depending on the affected organ/s, extent of tumefactive fibroinflammatory lesions, and associated functional impairment. The disease pursues a chronic, relapsing, often asymptomatic course and hence may pose a significant diagnostic challenge. Diagnostic delay can lead to progressive fibrosis and irreversible organ damage resulting into significant morbidity and even mortality. Given its broad clinical spectrum, physicians of all specialties may be the first clinicians facing this diagnostic challenge. Outside the pancreatobiliary system, the head and neck represents the major site of IgG4-RD with variable organ-specific diffuse or mass-forming lesions. In up to 75% of cases, elevated serum IgG4 levels are observed, but this figure possibly underestimates the fraction of seronegative cases, as the disease manifestations may present metachronously with significant intervals. Together with negative serology, this can lead to misdiagnosis of seronegative cases. A standardized nomenclature and diagnostic criteria for IgG4-RD were established in 2012 and revised in 2020 facilitating scientific research and expanding the range of diseases associated with IgG4 abnormalities. In addition to orbital pseudotumor, dacryoadenitis, Riedel thyroiditis, sinonasal manifestations, and rare miscellaneous conditions, IgG4-related sialadenitis is one of the most frequent presentations in the head and neck region. However, controversy still exists regarding the relationship between sialadenitis and IgG4-RD. This review focuses on the clinicopathological features of IgG4-related sialadenitis and its contemporary diagnostic criteria.
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Affiliation(s)
- Agata Czarnywojtek
- Department of Pharmacology, Poznan University of Medical Sciences, 60-806, Poznan, Poland
- Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355, Poznan, Poland
| | - Abbas Agaimy
- Institute of Pathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg (FAU), 91054, Erlangen, Germany
| | | | - Iain J Nixon
- Department of Otorhinolaryngology Head and Neck Surgery, NHS Lothian, Edinburgh, EH8 9YL, UK
| | - Vincent Vander Poorten
- Otorhinolaryngology-Head and Neck Surgery, KU Leuven University Hospitals, 3000, Leuven, Belgium
- Department of Oncology, Section Head and Neck Oncology, KU Leuven, 3000, Leuven, Belgium
| | - Antti A Mäkitie
- Department of Otorhinolaryngology-Head and Neck Surgery, University of Helsinki and Helsinki University Hospital, and the Research Program in Systems Oncology, Faculty of Medicine, University of Helsinki, 00014, Helsinki, Finland
| | - Mark Zafereo
- Department of Head & Neck Surgery, MD Anderson Cancer Center, Houston, TX, 77005, USA
| | - Ewa Florek
- Laboratory of Environmental Research, Department of Toxicology, Poznan University of Medical Sciences, 60-806, Poznan, Poland.
| | - Nadia Sawicka-Gutaj
- Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355, Poznan, Poland
| | - Marek Ruchała
- Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355, Poznan, Poland
| | - Alfio Ferlito
- International Head and Neck Scientific Group, 35100, Padua, Italy
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12
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Gilani SI, Buglioni A, Cornell LD. IgG4-related kidney disease: Clinicopathologic features, differential diagnosis, and mimics. Semin Diagn Pathol 2024; 41:88-94. [PMID: 38246802 DOI: 10.1053/j.semdp.2023.12.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/14/2023] [Accepted: 12/11/2023] [Indexed: 01/23/2024]
Abstract
IgG4-related kidney disease (IgG4-RKD) encompasses all forms of kidney disease that are part of IgG4-related disease (IgG4-RD). First recognized as IgG4-related tubulointerstitial nephritis (IgG4-TIN), and then IgG4-related membranous glomerulonephritis (IgG4-MGN), we now recognize additional patterns of interstitial nephritis, glomerular disease, and vascular disease that can be seen as part of IgG4-RKD. The clinical presentation is variable and can include acute or chronic kidney injury, proteinuria or nephrotic syndrome, mass lesion(s), and obstruction. While usually associated with other organ involvement by IgG4-RD, kidney-alone involvement is present in approximately 20 % of IgG4-RKD. Compared to IgG4-RD overall, patients with IgG4-RKD are more likely to show increased serum IgG4 or IgG, and more likely to have hypocomplementemia. In this review, we extensively cover other types of autoimmune and plasma cell-rich interstitial nephritis, mass forming inflammatory diseases of the kidney, and other mimics of IgG4-TIN, in particular ANCA-associated disease.
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Affiliation(s)
- Sarwat I Gilani
- Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at San Antonio, TX, USA
| | - Alessia Buglioni
- Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
| | - Lynn D Cornell
- Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
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13
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Mizushima I, Saeki T, Kobayashi D, Sawa N, Hayashi H, Taniguchi Y, Nakata H, Yamada K, Matsui S, Yasuno T, Masutani K, Nagasawa T, Takahashi H, Ubara Y, Yanagita M, Kawano M. Improved Renal Function in Initial Treatment Improves Patient Survival, Renal Outcomes, and Glucocorticoid-Related Complications in IgG4-Related Kidney Disease in Japan. Kidney Int Rep 2024; 9:52-63. [PMID: 38312790 PMCID: PMC10831353 DOI: 10.1016/j.ekir.2023.10.016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2023] [Revised: 09/26/2023] [Accepted: 10/16/2023] [Indexed: 02/06/2024] Open
Abstract
Introduction We aimed to clarify long-term renal prognosis, complications of malignancy, glucocorticoid (GC) toxicity, and mortality in immunoglobulin G4 (IgG4)-related kidney disease (IgG4-RKD). Methods Reviewing the medical records of 95 patients with IgG4-RKD, we investigated clinical and pathologic features at baseline, the course of renal function, complications of malignancy, GC toxicity, and mortality during follow-up (median 71 months). The standardized incidence ratio (SIR) of malignancy and standardized mortality ratio were calculated using national statistics. Factors related to outcomes were assessed by Cox regression analyses. Results At diagnosis, the median estimated glomerular infiltration rate (eGFR) was 46 ml/min per 1.73 m2. GC achieved initial improvement. Additional renal function recovery within 3-months of initial treatment occurred in patients with highly elevated serum IgG and IgG4 levels and hypocomplementemia. During follow-up, 68%, 17%, and 3% of the patients had chronic kidney disease (CKD), >30% eGFR decline, and end-stage renal disease (ESRD), respectively. Age-adjusted and sex-adjusted Cox regression analyses indicated that eGFR (hazard ratio [HR], 0.71) and extensive fibrosis (HR, 2.58) at treatment initiation had a significant impact on the time to CKD. Ten patients died, and the standardized mortality ratio was 0.94. The SIR of malignancy was 1.52. The incidence rate (IR) of severe infection was 1.80/100 person-years. Cox regression analyses showed that the best eGFR within 3 months after treatment initiation were associated with lower mortality (HR 0.67) and fewer severe infections (HR 0.63). Conclusion This study suggests that more renal function recovery through early treatment initiation may improve patient survival, renal outcomes, and some GC-related complications in IgG4-RKD.
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Affiliation(s)
- Ichiro Mizushima
- Department of Rheumatology, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan
| | - Takako Saeki
- Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan
| | - Daisuke Kobayashi
- Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata City, Japan
| | - Naoki Sawa
- Department of Nephrology Center, Toranomon Hospital, Kawasaki, Japan
| | - Hiroki Hayashi
- Department of Nephrology, Fujita Health University School of Medicine, Toyoake, Japan
| | - Yoshinori Taniguchi
- Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University, Nankoku-shi, Japan
| | - Hirosuke Nakata
- Department of Nephrology, Graduate School of Medicine, Kyoto University, Kyoto City, Japan
| | - Kazunori Yamada
- Department of Hematology and Immunology, Kanazawa Medical University, Kahoku-gun, Japan
| | - Shoko Matsui
- Health Administration Center, University of Toyama, Toyama City, Japan
| | - Tetsuhiko Yasuno
- Division of Nephrology and Rheumatology, Department of Internal Medicine, Faculty of Medicine, Fukuoka University, Jonan-Ku, Japan
| | - Kosuke Masutani
- Division of Nephrology and Rheumatology, Department of Internal Medicine, Faculty of Medicine, Fukuoka University, Jonan-Ku, Japan
| | - Tasuku Nagasawa
- Division of Nephrology, Endocrinology, and Vascular Medicine, Tohoku University Hospital, Sendai, Japan
| | - Hiroki Takahashi
- Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Yoshifumi Ubara
- Department of Nephrology Center, Toranomon Hospital, Kawasaki, Japan
| | - Motoko Yanagita
- Department of Nephrology, Graduate School of Medicine, Kyoto University, Kyoto City, Japan
| | - Mitsuhiro Kawano
- Department of Rheumatology, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan
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14
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Shanbhogue KP, Ramani N, Surabhi VR, Balasubramanya R, Prasad SR. Tumefactive Nonneoplastic Proliferative Pseudotumors of the Kidneys and Urinary Tract: CT and MRI Findings with Histopathologic Correlation. Radiographics 2023; 43:e230071. [PMID: 37971934 DOI: 10.1148/rg.230071] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2023]
Abstract
A diverse spectrum of pathologically distinct, nonneoplastic, proliferative conditions of the kidneys and urinary tract demonstrate a expansile growth pattern similar to that of neoplasms. The renal pseudotumors include myriad causes of infections as well as rare noninfectious causes such as sarcoidosis, amyloidosis, and immunoglobulin G4-related disease (IgG4-RD). Rare entities such as cystitis cystica, endometriosis, nephrogenic adenoma, and pseudosarcomatous myofibroblastic proliferation and distinct types of prostatitis comprise tumefactive nontumorous disorders that affect specific segments of the urinary tract. The pseudotumors of the kidneys and urinary tract demonstrate characteristic histopathologic and epidemiologic features, as well as protean clinical manifestations, natural history, and imaging findings. Many patients present with genitourinary tract-specific symptoms or systemic disease. Some cases may be incidentally discovered at imaging. Some entities such as perinephric myxoid pseudotumors, IgG4-RD, fibroepithelial polyp, and nephrogenic adenoma display specific anatomic localization and disease distribution. Imaging features of multisystem disorders such as tuberculosis, sarcoidosis, and IgG4-RD provide supportive evidence that may allow precise diagnosis. Fungal pyelonephritis, xanthogranulomatous pyelonephritis, IgG4-RD, actinomycosis, and endometriosis show markedly low signal intensity on T2-weighted MR images. Although some pseudotumors exhibit characteristic imaging findings that permit correct diagnosis, laboratory correlation and histopathologic confirmation are required for definitive characterization in most cases. A high index of suspicion is a prerequisite for diagnosis. Accurate diagnosis is critical for instituting optimal management while preventing use of inappropriate therapies or interventions. Surveillance CT and MRI are frequently used for monitoring the response of pseudotumors to therapy. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.
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Affiliation(s)
- Krishna Prasad Shanbhogue
- From the Departments of Radiology (K.P.S., V.R.S., R.B., S.R.P.) and Pathology (N.R.), Michael E. DeBakey VA Medical Center, Houston, Tex; Department of Radiology, NYU Langone Health, New York, NY (K.P.S.); Department of Radiology, Thomas Jefferson University Hospital, Philadelphia, Pa (R.B.); and Department of Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler St, Unit 1473, Houston, TX 77030 (V.R.S., S.R.P.)
| | - Nisha Ramani
- From the Departments of Radiology (K.P.S., V.R.S., R.B., S.R.P.) and Pathology (N.R.), Michael E. DeBakey VA Medical Center, Houston, Tex; Department of Radiology, NYU Langone Health, New York, NY (K.P.S.); Department of Radiology, Thomas Jefferson University Hospital, Philadelphia, Pa (R.B.); and Department of Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler St, Unit 1473, Houston, TX 77030 (V.R.S., S.R.P.)
| | - Venkateswar R Surabhi
- From the Departments of Radiology (K.P.S., V.R.S., R.B., S.R.P.) and Pathology (N.R.), Michael E. DeBakey VA Medical Center, Houston, Tex; Department of Radiology, NYU Langone Health, New York, NY (K.P.S.); Department of Radiology, Thomas Jefferson University Hospital, Philadelphia, Pa (R.B.); and Department of Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler St, Unit 1473, Houston, TX 77030 (V.R.S., S.R.P.)
| | - Rashmi Balasubramanya
- From the Departments of Radiology (K.P.S., V.R.S., R.B., S.R.P.) and Pathology (N.R.), Michael E. DeBakey VA Medical Center, Houston, Tex; Department of Radiology, NYU Langone Health, New York, NY (K.P.S.); Department of Radiology, Thomas Jefferson University Hospital, Philadelphia, Pa (R.B.); and Department of Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler St, Unit 1473, Houston, TX 77030 (V.R.S., S.R.P.)
| | - Srinivasa R Prasad
- From the Departments of Radiology (K.P.S., V.R.S., R.B., S.R.P.) and Pathology (N.R.), Michael E. DeBakey VA Medical Center, Houston, Tex; Department of Radiology, NYU Langone Health, New York, NY (K.P.S.); Department of Radiology, Thomas Jefferson University Hospital, Philadelphia, Pa (R.B.); and Department of Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler St, Unit 1473, Houston, TX 77030 (V.R.S., S.R.P.)
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15
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McArthur M, Patel M. A pictorial review of genitourinary infections and inflammations. Clin Imaging 2023; 104:110013. [PMID: 37918136 DOI: 10.1016/j.clinimag.2023.110013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/22/2023] [Revised: 10/03/2023] [Accepted: 10/23/2023] [Indexed: 11/04/2023]
Abstract
Various infectious and inflammatory diseases affect the genitourinary system. This paper provides a review of multiple common and uncommon infectious and inflammatory conditions affecting the genitourinary system and some associated complications. These include acute infectious cystitis, emphysematous cystitis, acute pyelonephritis, emphysematous pyelonephritis, renal and perinephric abscesses, pyonephrosis, xanthogranulomatous pyelonephritis, epididymo-orchitis, vasitis, prostatitis, pelvic inflammatory disease, renal hydatid infection, renal tuberculosis, actinomycosis, Erdheim-Chester Disease, IgG4-Related Kidney Disease, urethritis and urethral strictures, ureteritis cystica, and genitourinary fistulas. Radiologists should be aware of these diseases' complications and management. Uncommon conditions must be considered when evaluating the genitourinary system.
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Affiliation(s)
- Mark McArthur
- University of California, Los Angeles, United States.
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16
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Nagahata K, Osanami A, Nakamura H, Amaike H, Kanda M, Takahashi H. IgG4-related tubulointerstitial nephritis: renal capsule-like rim. QJM 2023; 116:953-954. [PMID: 37369024 DOI: 10.1093/qjmed/hcad157] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/26/2023] [Indexed: 06/29/2023] Open
Affiliation(s)
- K Nagahata
- Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, South West 16, Chuo-ku, Sapporo, Hokkaido 060-8543, Japan
| | - A Osanami
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, Hokkaido 060-8543, Japan
| | - H Nakamura
- Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, South West 16, Chuo-ku, Sapporo, Hokkaido 060-8543, Japan
| | - H Amaike
- Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, South West 16, Chuo-ku, Sapporo, Hokkaido 060-8543, Japan
| | - M Kanda
- Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, South West 16, Chuo-ku, Sapporo, Hokkaido 060-8543, Japan
| | - H Takahashi
- Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, South West 16, Chuo-ku, Sapporo, Hokkaido 060-8543, Japan
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17
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Groh M, Habert P, Ebbo M, Muller R, Gaigne L, Gaubert JY, Schleinitz N. [IgG4-related disease: A proteiform pathology with frequent chest manifestations]. Rev Mal Respir 2023; 40:768-782. [PMID: 37858433 DOI: 10.1016/j.rmr.2023.10.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2023] [Accepted: 09/11/2023] [Indexed: 10/21/2023]
Abstract
INTRODUCTION While IgG4-related disease (IgG4-RD) was initially described in the early 2000s, its polymorphic clinical manifestations were previously reported under different names ; they have in common the presence of IgG4+ oligoclonal plasma cells and fibrosis. STATE OF THE ART Ruling out certain differential diagnoses, the diagnosis of IgG4-RD is based on a bundle of clinical, biological and histological features. Chest involvement is variable and can affect the mediastinum, bronchi, parenchyma, pleura and/or, more rarely, bones and (pericardium, aorta, coronary…) vascular structures. The most frequent radiological manifestations are peribronchovascular thickening, mediastinal lymphadenopathy, and nodular or interstitial patterns. Pleural involvement and posterior mediastinal fibrosis are less frequent, while thoracic paravertebral tissue thickening is highly specific. Systemic corticosteroids are the cornerstone of treatment. In case of relapse or as frontline therapy in case of risk factors for relapse and/or poor tolerance of corticosteroids), a steroid-sparing agent (most often rituximab) is added, and biannual maintenance infusions are associated with a lower risk of relapse. PERSPECTIVES An international consensus has recently led to the development of classification criteria that should standardize the diagnostic approach and homogenize the enrolment of patients in epidemiological as well as therapeutic studies. Other treatments are also under evaluation, including biologics targeting T2 inflammation, CD-19 (inebilizumab, obexelimab), SLAMF7 (elotuzumab) surface proteins, Bruton's tyrosine kinase, and the JAK/STAT pathway. CONCLUSIONS Substantial progress has been made over recent years in understanding IgG4-RD pathophysiology, and personalized patient care seems to be an achievable medium-term goal.
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Affiliation(s)
- M Groh
- Centre de références des syndromes hyperéosinophiliques (CEREO), service de médecine Interne, hôpital Foch, 92150 Suresnes, France; Inserm, U1286 - INFINITE-Institute for Translational Research in Inflammation, Université de Lille, CHU de Lille, 59000 Lille, France
| | - P Habert
- Service de radiologie, hôpital Nord, APHM, Aix-Marseille université, Marseille, France; LIIE (Experimental Interventional Imaging Laboratory), Aix-Marseille Université, 13000 Marseille, France
| | - M Ebbo
- Service de médecine Interne, hôpital La Timone, APHM, Aix-Marseille Université, 13005 Marseille, France
| | - R Muller
- Service de médecine Interne, hôpital La Timone, APHM, Aix-Marseille Université, 13005 Marseille, France
| | - L Gaigne
- Service de médecine Interne, hôpital La Timone, APHM, Aix-Marseille Université, 13005 Marseille, France
| | - J-Y Gaubert
- Service de radiologie, hôpital La Timone, APHM, Aix-Marseille université, 13005 Marseille, France
| | - N Schleinitz
- Service de médecine Interne, hôpital La Timone, APHM, Aix-Marseille Université, 13005 Marseille, France.
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18
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Mamizu H, Ohta T, Yanai K, Yamazaki R, Mamizu M, Ishikawa D, Kawakami H, Furukawa T, Ishida T. Refractory Eosinophilic Granulomatosis with Polyangiitis Complicated with IgG4-related Disease Showing Different Treatment Responses for Each Organ. Intern Med 2023; 62:2995-3000. [PMID: 36823081 PMCID: PMC10641191 DOI: 10.2169/internalmedicine.1302-22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/16/2022] [Accepted: 01/10/2023] [Indexed: 02/25/2023] Open
Abstract
We herein report a 75-year-old woman who presented with dyspnea and purpura. She was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) based on axonal damage observed in the left tibial nerve and skin and lung pathologies. Lung pathology showed IgG4-positive plasma cells, considered a complication of IgG4-related disease (IgG4-RD). Computed tomography revealed thickening of the abdominal aorta and a poor contrast area in the left kidney, which was indicative of IgG4-RD. Steroid administration improved the IgG4-RD. However, the EGPA resisted treatment; therefore, immunosuppressive drugs and mepolizumab were administered. Refractory EGPA complicated with IgG4-RD showed different treatment responses for each organ.
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Affiliation(s)
- Hikaru Mamizu
- Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
| | - Takeshi Ohta
- Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
| | - Kensuke Yanai
- Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
| | - Ryo Yamazaki
- Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
| | - Maiko Mamizu
- Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
| | - Daisuke Ishikawa
- Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
| | - Hidenori Kawakami
- Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
| | - Toshiki Furukawa
- Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
| | - Takashi Ishida
- Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
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19
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Mima A, Lee R, Murakami A, Gotoda H, Akai R, Lee S. Case Report: IgG4-related kidney disease complicated by interstitial pneumonia. F1000Res 2023; 12:1045. [PMID: 37744768 PMCID: PMC10511842 DOI: 10.12688/f1000research.131818.1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/22/2023] [Indexed: 09/26/2023] Open
Abstract
Immunoglobulin G4 (IgG4)-related disease is a systemic inflammatory disorder characterized by tubulointerstitial nephritis with IgG4-positive plasma cell infiltration. We report the case of an 84-year-old male who presented with a history of dyspnea on exertion and cough. The lymph nodes were palpated in the axilla. Urinalysis revealed mild proteinuria and increased levels of NAG and β2-microglobulin. Blood tests showed hyperglobulinemia with a marked elevation of serum IgG4 levels. Chest computed tomography showed bilateral ground-glass and reticular opacities in the lower and peripheral portions of the lungs. Ga-67 scintigraphy showed kidney uptake. The patient was diagnosed with IgG4-related kidney disease based on the renal pathology indicative of typical tubulointerstitial nephritis with extensive IgG4-positive plasma cell infiltration. The patient was treated with prednisolone and showed a prompt response in his clinical condition. The patient achieved normalization of serum IgG4 levels 6 months after the initiation of treatment. Although IgG4-related disease is thought to be potentially associated with organ fibrosis, there are few reports on combination of interstitial pneumonia and IgG4-related kidney disease. Our case report presents a possible pattern of IgG4-related disease.
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Affiliation(s)
- Akira Mima
- Department of Nephrology, Osaka Medical and Pharmaceutical University, Takatsuki, Japan
| | - Rina Lee
- Department of Nephrology, Osaka Medical and Pharmaceutical University, Takatsuki, Japan
| | - Ami Murakami
- Department of Nephrology, Osaka Medical and Pharmaceutical University, Takatsuki, Japan
| | - Hidemasa Gotoda
- Department of Nephrology, Osaka Medical and Pharmaceutical University, Takatsuki, Japan
| | - Ryosuke Akai
- Department of Nephrology, Osaka Medical and Pharmaceutical University, Takatsuki, Japan
| | - Shinji Lee
- Department of Nephrology, Osaka Medical and Pharmaceutical University, Takatsuki, Japan
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20
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Suenaga A, Sawa N, Ikuma D, Oba Y, Sekine A, Yamanouchi M, Hasegawa E, Mizuno H, Suwabe T, Kono K, Shintani-Domoto Y, Kinowaki K, Ohashi K, Suzuki Y, Miyazono M, Takemura T, Yamaguchi Y, Ubara Y. Immunoglobulin G4-related Tubulointerstitial Nephritis with Simultaneous Resolution of Plasma Cell Infiltration and Fibrosis after Steroid Treatment. Intern Med 2023; 62:2699-2706. [PMID: 36725037 PMCID: PMC10569928 DOI: 10.2169/internalmedicine.1121-22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/13/2022] [Accepted: 12/11/2022] [Indexed: 02/03/2023] Open
Abstract
We performed 3 kidney biopsies in a 71-year-old man. At the first biopsy, we made the diagnosis of immunoglobulin G4 (IgG4)-related interstitial nephritis characterized by the simultaneous presence of IgG4-positive plasma cells and characteristic fibrosis with a bird's-eye pattern. At the second biopsy, rather than finding fibrosis as a post-inflammatory scar, we noted that steroid treatment had caused the simultaneous disappearance of IgG4-positive plasma cells and fibrosis and had restored the normal tubular structure. The third biopsy showed the recurrence of the disease with inflammatory cells accompanied by fibrosis. These findings suggest that IgG4-positive plasma cells and fibrosis occur simultaneously.
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Affiliation(s)
- Atsuhiko Suenaga
- Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Japan
- Department of Nephrology, Saga University School of Medicine, Japan
| | - Naoki Sawa
- Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Japan
- Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan
| | - Daisuke Ikuma
- Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Japan
| | - Yuki Oba
- Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Japan
| | - Akinari Sekine
- Department of Nephrology and Rheumatology, Toranomon Hospital, Japan
| | - Masayuki Yamanouchi
- Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Japan
| | - Eiko Hasegawa
- Department of Nephrology, Saga University School of Medicine, Japan
- Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan
| | - Hiroki Mizuno
- Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Japan
| | - Tatsuya Suwabe
- Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Japan
| | - Kei Kono
- Department of Pathology, Toranomon Hospital, Japan
| | | | | | - Kenichi Ohashi
- Department of Pathology, Toranomon Hospital, Japan
- Department of Human Pathology, Tokyo Medical and Dental University, Japan
| | | | - Motoaki Miyazono
- Department of Nephrology, Saga University School of Medicine, Japan
| | - Tamiko Takemura
- Department of Pathology, Kanagawa Cardiovascular and Respiratory Center, Japan
| | | | - Yoshifumi Ubara
- Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Japan
- Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan
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21
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Seo I, Kim Y. IgG4-Related Diseases Involving Bilateral Renal Pelvises Successfully Treated with Steroid Therapy. SAUDI JOURNAL OF KIDNEY DISEASES AND TRANSPLANTATION 2023; 34:449-454. [PMID: 38995304 DOI: 10.4103/1319-2442.397207] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/13/2024] Open
Abstract
Immunoglobulin (Ig) G4-related diseases are very rare diseases and are difficult to diagnose and treat. Here, we report a rare condition, an IgG4-related disease involving the bilateral renal pelvises, which was successfully treated with steroid therapy. A 64-year-old female was admitted with left flank pain and hematuria. Computed tomographic images showed irregular enhancing masses around the bilateral renal pelvises and lymph nodes in the left para-aortic area. A histopathological examination of a renal needle biopsy revealed numerous cells that were positive for IgG and more than 10 IgG4-positive cells per high-powered field. The IgG4/IgG ratio was more than 40%. Her serum IgG4 level was elevated to 1440 mg/dL, and her IgG4/IgG ratio was 82.1%. After a diagnosis of IgG4 sclerosing disease, a double-J ureteral stent was inserted and steroid therapy was performed for 20 weeks. Her symptoms dramatically improved, and the follow-up images showed a resolved state. There was no relapse of the disease for more than 6 months after the removal of the stent.
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Affiliation(s)
- Illyoung Seo
- Department of Urology, Institute of Wonkwang Medical Science, Wonkwang University School of Medicine and Hospital, Iksan, South Korea
| | - Youri Kim
- Department of Radiology, Institute of Wonkwang Medical Science, Wonkwang University School of Medicine and Hospital, Iksan, South Korea
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22
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Palamaris K, Stylianou K, Destouni M, Stofas A, Theodoropoulou H, Kroustalakis N, Dermitzaki EK, Petrakis I, Pleros C, Theochari I, Sarantis P, Paliouras C, Gakiopoulou H. Tubulointerstitial Nephritis and Uveitis Syndrome: A Report of 6 Cases with Renal Biopsy and Electron Microscopy Evaluation. Nephron Clin Pract 2023; 148:204-214. [PMID: 37611557 DOI: 10.1159/000533402] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2022] [Accepted: 06/24/2023] [Indexed: 08/25/2023] Open
Abstract
Tubulointerstitial nephritis with uveitis syndrome is a rare, immune-mediated entity, characterized by oculo-renal inflammation. Diagnosis requires the exclusion of all other causes of tubulointerstitial nephritis (TIN). We present 6 patients with clinical, laboratory, and renal biopsy findings denotative of tubulointerstitial nephritis with uveitis syndrome. All our patients experienced ocular and renal manifestations, defined by bilateral uveitis and photosensitivity, along with a decline of renal function. In some patients, increased serum creatinine was accompanied by non-nephrotic range proteinuria, glucosuria or "full-blown" Fanconi syndrome. The rest of the laboratory evaluation was normal apart from the presence of elevated erythrocyte sedimentation rate and increased urine β2-microglobulin, as well as normochromic, normocytic anemia in some cases. All patients underwent renal biopsy. Histochemical (PAS, Masson, silver, Congo-red) and immunohistochemical stains for immune cell populations (CD3, CD20, CD4, CD8, PGM1, CD138) and for the assessment of β2-microglobulin were conducted. Electron microscopy examination of the biopsies was also performed. Follow-up, ranging from 18 months to 10 years, was available for 4 patients. Histological evaluation revealed interstitial inflammatory infiltration consisting mainly of lymphocytes, with a T-cell predominance, along with several macrophages. Inflammation severity varied among different patients, with some showing scarce foci of immune cell clusters, while others demonstrated a dense, diffuse interstitial infiltration. Interestingly, in 2 cases, a granulomatous pattern, characterized by non-necrotic, ill-defined granulomas was detected. Tubulitis was also encountered in some patients. A divergence was noted regarding the chronicity index, with different levels of tubular atrophy, interstitial fibrosis, and global glomerulosclerosis among different cases. β2-Microglobulin immunohistochemical evaluation revealed a substantial diminishment of cytoplasmic staining in tubular epithelial cells compared to control kidneys. The most notable finding derived from electron microscopy examination was the presence, in 1 patient, of scattered granular electron-dense deposits along some tubular basement membranes. First-line treatment included steroids, supplemented in some cases by additional immunosuppressive agents. Three patients experienced a partial or complete response, while progressive renal damage was observed in a case with severe chronic lesions and persistence of inflammation-triggering factor. Our cases seem to represent progressive stages within the continuum of disease evolution. Patients with more prominent inflammation might represent a more initial state, while those with a more severe chronicity index, probably depict more advanced stages. While the predominance of T-cells predicates a cell-mediated autoimmune mechanism, as the driving force of the disease occurrence, the presence of immune complexes in more advanced stages might indicate the involvement of humoral immunity as a late event during the disease course.
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Affiliation(s)
- Kostas Palamaris
- First Department of Pathology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece,
| | - Kostas Stylianou
- Nephrology Department, Heraklion University Hospital, Heraklion, Greece
| | - Maria Destouni
- First Department of Pathology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
| | - Anastasios Stofas
- First Department of Pathology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
| | | | | | | | - Ioannis Petrakis
- Nephrology Department, Heraklion University Hospital, Heraklion, Greece
| | - Christo Pleros
- Nephrology Department, Heraklion University Hospital, Heraklion, Greece
| | - Irene Theochari
- First Department of Pathology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
| | - Panagiotis Sarantis
- Department of Biological Chemistry, National and Kapodistrian University of Athens, Athens, Greece
| | | | - Harikleia Gakiopoulou
- First Department of Pathology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
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23
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Shimosegawa T. Clinical Manifestation of Type 1 Autoimmune Pancreatitis. THE PANCREAS 2023:546-553. [DOI: 10.1002/9781119876007.ch70] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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24
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Soma Y, Kato M, Shimura G, Kamio M, Iida M. Renal biopsy diagnosis of IgG4-related kidney disease with minor hematuria and mild renal dysfunction: lessons for the clinical nephrologist. J Nephrol 2023; 36:639-642. [PMID: 36441499 DOI: 10.1007/s40620-022-01506-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2022] [Accepted: 10/13/2022] [Indexed: 11/29/2022]
Affiliation(s)
- Yu Soma
- Department of Nephrology, Yokosuka General Uwamachi Hospital, Yokosuka, Japan.
| | - Miku Kato
- Department of Nephrology, Yokosuka General Uwamachi Hospital, Yokosuka, Japan
| | - Gaku Shimura
- Department of Nephrology, Yokosuka General Uwamachi Hospital, Yokosuka, Japan
| | - Manabu Kamio
- Department of Internal Medicine, Yokosuka General Uwamachi Hospital, Yokosuka, Japan
| | - Maki Iida
- Department of Pathology, Yokosuka General Uwamachi Hospital, Yokosuka, Japan
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25
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Nguyen T, Brodsky S, Maroz N. Progression to End-Stage Renal Disease Due to IgG4-Related Nephritis Refractory to Rituximab. Cureus 2023; 15:e36327. [PMID: 37077588 PMCID: PMC10108657 DOI: 10.7759/cureus.36327] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/18/2023] [Indexed: 03/20/2023] Open
Abstract
An 81-year-old woman was referred to nephrology for a follow-up on progressive chronic kidney disease. She has a past medical history of hypertension, T2DM, breast cancer, and secondary hyperparathyroidism related to renal disease. A renal biopsy showed patchy interstitial fibrosis and tubular atrophy with an increased number of IgG4-positive plasma cells. A diagnosis of IgG4-related kidney disease was made based on clinical presentation and pathology. The patient ultimately required the initiation of hemodialysis, despite the administration of steroids and rituximab.
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26
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He PH, Liu LC, Zhou XF, Xu JJ, Hong WH, Wang LC, Liu SJ, Zeng JH. IgG4-related kidney disease complicated with retroperitoneal fibrosis: A case report. World J Clin Cases 2023; 11:1656-1665. [PMID: 36926395 PMCID: PMC10011980 DOI: 10.12998/wjcc.v11.i7.1656] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/08/2022] [Revised: 01/11/2023] [Accepted: 02/15/2023] [Indexed: 03/02/2023] Open
Abstract
BACKGROUND IgG4-related disease (IgG4-RD) is a chronic fibrotic disease mediated by immunity recognized by clinicians in recent years. When the kidney is involved, it is called IgG4-related kidney disease (IgG4-RKD). IgG4-related tubulointerstitial nephritis (IgG4-TIN) is a representative manifestation of IgG4-RKD. IgG4-TIN can cause obstructive nephropathy complicated by retroperitoneal fibrosis (RPF). Cases of IgG4-TIN complicated with RPF are rare. Glucocorticoids are the first-line therapeutic medication for IgG4-RD and can significantly improve renal function.
CASE SUMMARY Herein, we report the case of a 56-year-old man with IgG4-RKD complicated with RPF. The patient presented to the hospital with complaints of elevated serum creatinine (Cr), nausea, and vomiting. During hospitalization, Cr was 1448.6 µmol/L, and serum IgG4 was increased. A total abdominal computed tomography (CT) scan and enhanced CT scan obviously indicated RPF. Although this patient had a long course and renal insufficiency, we performed a kidney biopsy. Renal biopsy showed that the renal tubulointerstitium had focal plasma cell infiltration and increased lymphocyte infiltration accompanied by fibrosis. After combining the biopsy results with immunohistochemistry, it was found that the absolute number of positive IgG4+ cells per high power field exceeded 10, and the ratio of IgG4/IgG was over 40%. Finally, the patient was diagnosed with IgG4-TIN complicated with RPF and given glucocorticoids as long-term maintenance therapy, helping him keep out of dialysis. After a follow-up of 19 mo, the patient had recovered well. Previous literature on IgG4-RKD and RPF was retrieved from PubMed to characterize the clinical and pathological features and to identify the diagnosis and treatment of IgG4-RKD.
CONCLUSION Our case report demonstrates the clinical characteristics of IgG4-RKD complicated with RPF. Serum IgG4 is a favorable indicator for screening. Performing renal biopsy actively plays a vital role in diagnosis and treatment, even if the patient has a long course and manifests with renal insufficiency. It is remarkable to treat IgG4-RKD with glucocorticoids. Hence, early diagnosis and targeted therapy are essential for reversing renal function and improving extrarenal manifestations in patients with IgG4-RKD.
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Affiliation(s)
- Pei-Hua He
- The Second Clinical Medical College, Guangzhou University of Chinese Medicine, Guangzhou 510006, Guangdong Province, China
| | - Li-Chang Liu
- Department of Nephrology, Zhuhai Hospital of Guangdong Provincial Hospital of Chinese Medicine, Zhuhai 519015, Guangdong Province, China
| | - Xing-Fu Zhou
- The Second Clinical Medical College, Guangzhou University of Chinese Medicine, Guangzhou 510006, Guangdong Province, China
| | - Jun-Jie Xu
- The Second Clinical Medical College, Guangzhou University of Chinese Medicine, Guangzhou 510006, Guangdong Province, China
| | - Wei-Hong Hong
- Department of Nephrology, Zhuhai Hospital of Guangdong Provincial Hospital of Chinese Medicine, Zhuhai 519015, Guangdong Province, China
| | - Li-Chun Wang
- Department of Nephrology, Zhuhai Hospital of Guangdong Provincial Hospital of Chinese Medicine, Zhuhai 519015, Guangdong Province, China
| | - Su-Jun Liu
- Department of Nephrology, Zhuhai Hospital of Guangdong Provincial Hospital of Chinese Medicine, Zhuhai 519015, Guangdong Province, China
| | - Jia-Hao Zeng
- Department of Nephrology, Zhuhai Hospital of Guangdong Provincial Hospital of Chinese Medicine, Zhuhai 519015, Guangdong Province, China
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27
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Kawano M, Saeki T, Ubara Y, Matsui S. Recent advances in IgG4-related kidney disease. Mod Rheumatol 2023; 33:242-251. [PMID: 35788361 DOI: 10.1093/mr/roac065] [Citation(s) in RCA: 13] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2022] [Revised: 06/01/2022] [Accepted: 06/21/2022] [Indexed: 11/14/2022]
Abstract
Recent advances in the management and understanding of immunoglobulin (Ig)G4-related kidney disease (RKD) have emphasized the importance of urgent treatment in IgG4-related tubulointerstitial nephritis. On the other hand, to avoid long-term glucocorticoid toxicity, strategies for early withdrawal of steroids or combination of immunosuppressants, such as rituximab, and the minimum dose of steroids have been pursued. However, disease recurrence after reducing or stopping steroid therapy hampers early withdrawal of glucocorticoid maintenance therapy. In addition, knowledge has accumulated in diagnostic approaches including differential diagnosis of anti-neutrophil cytoplasmic antibodies-associated vasculitis, idiopathic multicentric Castleman's disease, and Rosai-Dorfman disease with kidney lesion, which leads to earlier and precise diagnosis of IgG4-RKD. This review summarizes recent progress in the differential diagnosis of IgG4-RKD and related treatment strategies and recent topics of hypocomplementaemia, membranous glomerulonephritis, and IgG4-related pyelitis and periureteral lesion.
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Affiliation(s)
- Mitsuhiro Kawano
- Department of Rheumatology, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan
| | - Takako Saeki
- Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan
| | - Yoshifumi Ubara
- Department of Nephrology and Rheumatology, Toranomon Hospital, Kawasaki, Japan
| | - Shoko Matsui
- Health Administration Center, University of Toyama, Toyama, Japan
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28
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Lee JE, Hwang SH. A Case of IgG4-Related Disease With Sinonasal Involvement Presenting With Decreased Visual Acuity. JOURNAL OF RHINOLOGY 2023; 30:48-52. [PMID: 39664706 PMCID: PMC11524364 DOI: 10.18787/jr.2023.00008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2023] [Revised: 01/30/2023] [Accepted: 02/01/2023] [Indexed: 04/01/2023] Open
Abstract
IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by IgG4-positive plasma cell and T lymphocyte infiltration of multiple organs. It commonly involves the pancreas, lacrimal glands, and salivary glands, and it has been rarely reported in the sinonasal cavity. We herein report the case of a 47-year-old male patient whose chief complaint was decreased visual acuity. A tumefactive mass was found on imaging studies, originating from the sinonasal cavity and invading the orbit, kidney, and meninges. The mass was resected through endoscopic sinus surgery and was pathologically confirmed to be IgG4-RD. The patient was treated with steroid therapy and showed clinical improvement.
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Affiliation(s)
- Ju Eun Lee
- Department of Otolaryngology-Head and Neck Surgery, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
| | - Se Hwan Hwang
- Department of Otolaryngology-Head and Neck Surgery, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
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29
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Kogami M, Abe Y, Ando T, Makiyama A, Yamaji K, Tamura N. Performance of classification and diagnostic criteria for IgG4-related disease and comparison of patients with and without IgG4-related disease. Sci Rep 2023; 13:2509. [PMID: 36782006 PMCID: PMC9925424 DOI: 10.1038/s41598-023-29645-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2022] [Accepted: 02/08/2023] [Indexed: 02/15/2023] Open
Abstract
IgG4-related disease (IgG4-RD) was recently described in Japan. It is characterised by extensive organ involvement with tissue fibrosis. We assessed the performance of the 2019 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification criteria and the 2020 revised comprehensive diagnostic (RCD) criteria as well as differences between patients with and without IgG4-RD. In this retrospective, single-centre study of 50 patients admitted with suspected IgG4-RD, we evaluated the sensitivity and specificity of both criteria. We also compared clinical characteristics and laboratory data of patients with IgG4-RD (n = 42) and patients without IgG4-RD (n = 8). The ACR/EULAR classification criteria had 88.1% sensitivity and 87.5% specificity for IgG4-RD diagnosis. The RCD criteria had 100% sensitivity and 50% specificity. Patients with IgG4-RD had significantly more affected organs (p = 0.002). Patients with a single affected organ and IgG4-RD had significantly higher serum IgG4/IgG ratios (p = 0.027), lower serum C-reactive protein levels (p = 0.020), and lower total haemolytic complement activity (p = 0.044) than those without IgG4-RD. The ACR/EULAR classification criteria have high specificity and the RCD criteria have high sensitivity for diagnosing IgG4-RD. The number of affected organs is important for diagnosing IgG4-RD.
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Affiliation(s)
- Masahiro Kogami
- Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo, Japan.
| | - Yoshiyuki Abe
- Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo, Japan
| | - Taiki Ando
- Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo, Japan
| | - Ayako Makiyama
- Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo, Japan
| | - Ken Yamaji
- Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo, Japan
| | - Naoto Tamura
- Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo, Japan
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30
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Yoshida M, Mizushima I, Tsuge S, Takahashi Y, Zoshima T, Nishioka R, Hara S, Ito K, Kawano M. Development of IgG4-related pancreatitis and kidney disease 7 years after the onset of undiagnosed lymphadenopathy: A case report. Mod Rheumatol Case Rep 2023; 7:192-196. [PMID: 35950792 DOI: 10.1093/mrcr/rxac065] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2022] [Revised: 07/17/2022] [Accepted: 08/05/2022] [Indexed: 06/15/2023]
Abstract
This report describes a patient diagnosed with immunoglobulin G4 (IgG4)-related pancreatitis and kidney disease 7 years after the onset of undiagnosed lymphadenopathy. A 48-year-old Japanese woman presented with fatigue and leg oedema. Computed tomography showed perigastric lymphadenopathy, for which she underwent a laparoscopic biopsy of the perigastric lymph nodes. Although histopathological examination of the lymph nodes did not lead to a definitive diagnosis, serological tests revealed elevated serum IgG4 levels (558 mg/dl) and IgG4 immunostaining of the lymph nodes showed IgG4-positive plasma cell infiltration, leading to the suspicion of IgG4-related disease. Further workup revealed no organ lesion other than lymphadenopathy. At age 55 years, despite having no subjective symptoms, contrast-enhanced computed tomography showed low-density lesions in the tail of the pancreas and the left kidney. Histopathological examination showed lymphocyte infiltration, consisting of a mixture of plasma cells and eosinophils, in both organs and obliterative phlebitis in the pancreas. IgG4 immunostaining of the kidney specimens showed 160 IgG4-positive cells per high-powered field, with the IgG4+/IgG+ cell ratio being almost 100%, leading to a diagnosis of IgG4-related pancreatitis and kidney disease. Treatment with prednisolone for 2 months resulted in lesion improvement. Although the diagnosis of IgG4-related lymphadenopathy is often challenging in patients with lymphadenopathy alone, findings in the present patient emphasise the importance of long-term follow-up, as it may allow early detection of involvement of other organs by IgG4-related disease.
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Affiliation(s)
- Misaki Yoshida
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| | - Ichiro Mizushima
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| | - Shunsuke Tsuge
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| | | | - Takeshi Zoshima
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| | - Ryo Nishioka
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| | - Satoshi Hara
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| | - Kiyoaki Ito
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| | - Mitsuhiro Kawano
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
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31
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Imai S, Tahara N, Igata S, Tahara A, Bekki M, Sugiyama Y, Maeda-Ogata S, Honda A, Otsuka H, Ushijima T, Okabe Y, Kaida H, Abe T, Tanaka H, Fukumoto Y, Tayama E. Vascular/perivascular inflammation in IgG4-related disease. J Nucl Cardiol 2022; 29:2920-2933. [PMID: 34704218 DOI: 10.1007/s12350-021-02812-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2021] [Accepted: 09/12/2021] [Indexed: 01/29/2023]
Abstract
BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is characterized by the infiltration of IgG4-positive plasma cells and fibrosclerotic inflammation in multiple organs. Although vascular complications are present in some patients with IgG4-RD, vascular and/or perivascular inflammatory activity compared to control subjects remains unknown. This study sought to investigate vascular/perivascular inflammation in IgG4-RD patients compared to control subjects using 18F-fluorodeoxyglucose-positron emission tomography combined with computed tomography (FDG-PET/CT). METHODS We examined 37 consecutive patients diagnosed as IgG4-RD (29 males, mean age of 64.3 ± 8.3 years old), who underwent FDG-PET/CT. Thirty-seven age- and gender-matched subjects without IgG4-RD were employed as controls. Vascular/perivascular inflammation was quantified by blood-normalized standardized uptake value, known as a target-to-background ratio (TBR). RESULTS All IgG4-RD patients presented with multiple region involvements. Twelve (32.4%) of the IgG4-RD patients had vascular complications, all of which appeared in the abdominal aorta. IgG4-RD patients had significantly higher TBR values in the descending aorta, abdominal aorta, and common iliac artery than control subjects. Also, IgG4-RD patients with vascular complication exhibited higher TBR values in the infra-renal aorta and common iliac artery than those without vascular complication. CONCLUSIONS We found that vascular FDG activity is significantly elevated in IgG4-RD patients regardless of vascular complication than control subjects. FDG-PET/CT is a useful modality for assessing vascular/perivascular inflammation, which may contribute vascular complication in IgG4-RD patients.
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Affiliation(s)
- Shinichi Imai
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Nobuhiro Tahara
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan.
| | - Sachiyo Igata
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Atsuko Tahara
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Munehisa Bekki
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Yoichi Sugiyama
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Shoko Maeda-Ogata
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Akihiro Honda
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Hiroyuki Otsuka
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Tomoyuki Ushijima
- Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan
| | - Yoshinobu Okabe
- Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan
| | - Hayato Kaida
- Department of Radiology, Kindai University Faculty of Medicine, Osakasayama, Japan
| | - Toshi Abe
- Department of Radiology, Kurume University School of Medicine, Kurume, Japan
| | - Hiroyuki Tanaka
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Yoshihiro Fukumoto
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Eiki Tayama
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
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32
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Sumimoto K, Uchida K, Ikeura T, Hirano K, Yamamoto M, Takahashi H, Nishino T, Mizushima I, Kawano M, Kamisawa T, Saeki T, Maguchi H, Ushijima T, Shiokawa M, Seno H, Goto H, Nakamura S, Okazaki K. Nationwide epidemiological survey of immunoglobulin G4-related disease with malignancy in Japan. J Gastroenterol Hepatol 2022; 37:1022-1033. [PMID: 35229347 DOI: 10.1111/jgh.15809] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/22/2021] [Revised: 01/24/2022] [Accepted: 02/08/2022] [Indexed: 01/11/2023]
Abstract
BACKGROUND AND AIM To clarify the clinicoepidemiological characteristics of immunoglobulin G4 (IgG4)-related disease (IgG4-RD) with malignancy, a nationwide epidemiological survey was conducted. METHODS Immunoglobulin G4-related disease patients with malignancy who had visited selected hospitals in Japan were surveyed. The study consisted of two stages: the number of IgG4-RD patients with malignancy was estimated by the first questionnaire and their clinicoepidemiological characteristics were assessed by the second questionnaire. RESULTS The frequencies of autoimmune pancreatitis (AIP), IgG4-related sialadenitis, IgG4-related eye disease, IgG4-related kidney disease, and IgG4-related retroperitoneal fibrosis were 44.7%, 20.8%, 14.0%, 5.16%, and 5.12%, respectively. The overall prevalence of malignant disease in IgG4-RD cases was estimated to be 10 900 per 100 000 cases, which was significantly higher than that of malignant disease in the general population. The prevalence of malignant lymphoma in IgG4-RD cases was the highest and was estimated to be 1985 per 100 000 cases. IgG4-related kidney disease had the highest frequency of malignant disease (17.1%). In data from 200 patients, 61 (30.5%) cases of cancer were found 2 years or more before the IgG4-RD diagnosis, 92 cases (46%) during the 1 year preceding or following IgG4-RD diagnosis, and 62 cases of cancer (31%) 2 or more years following IgG4-RD diagnosis. CONCLUSIONS The nationwide survey for IgG4-RD with malignancy in Japan showed that IgG4-RD may be related with malignant diseases.
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Affiliation(s)
- Kimi Sumimoto
- The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
| | - Kazushige Uchida
- Department of Gastroenterology and Hepatology, Kochi Medical School, Kochi University, Nankoku, Japan
| | - Tsukasa Ikeura
- The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
| | - Kenji Hirano
- Department of Gastroenterology, Tokyo Takanawa Hospital, Tokyo, Japan
| | - Motohisa Yamamoto
- The Institute of Medical Science, The University of Tokyo, Tokyo, Japan
| | - Hiroki Takahashi
- Department of Rheumatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Takayoshi Nishino
- Department of Gastroenterology, Tokyo Women's Medical University Yachiyo Medical Center, Tokyo, Japan
| | - Ichiro Mizushima
- Division of Rheumatology, Department of Internal Medicine, Kanazawa University Hospital, Kanazawa, Japan
| | - Mitsuhiro Kawano
- Division of Rheumatology, Department of Internal Medicine, Kanazawa University Hospital, Kanazawa, Japan
| | | | - Takako Saeki
- Department of Internal Medicine, Nagaoka Red Cross Hospital, Niigata, Japan
| | - Hiroyuki Maguchi
- Education and Research Center, Teine-Keijinkai Hospital, Sapporo, Hokkaido, Japan
| | - Tomoyuki Ushijima
- Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Fukuoka, Japan
| | - Masahiro Shiokawa
- Division of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, Kyoto, Japan
| | - Hiroshi Seno
- Division of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, Kyoto, Japan
| | - Hiroshi Goto
- Department of Ophthalmology, Tokyo Medical University, Tokyo, Japan
| | - Seiji Nakamura
- Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Fukuoka, Japan
| | - Kazuichi Okazaki
- The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
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Zongfei J, Lingli C, Ying S, Lingying M, Lijuan Z, Dongmei L, Xiaomin D, Yingyong H, Huiyong C, Lili M, Lindi J. Clinical and pathological predictors of relapse in IgG4-related disease. Arthritis Res Ther 2022; 24:106. [PMID: 35546243 PMCID: PMC9092827 DOI: 10.1186/s13075-022-02792-z] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2021] [Accepted: 04/30/2022] [Indexed: 12/24/2022] Open
Abstract
OBJECTIVES In IgG4-related disease, the relationship between pathological findings and relapse has not been well established. This study aimed to identify the clinical and pathological predictors of disease relapse in IgG4-RD. METHODS Patients with newly diagnosed IgG4-RD (n = 71) were enrolled between January 2011 and April 2020; all cases were pathologically confirmed. The clinical and pathological features were recorded in a database at baseline and each follow-up visit. Patients were followed up at least once a month via outpatient clinic examinations and telephone calls. Univariate and multivariate Cox regression analyses and receiver operating curve (ROC) analysis were used to identify the predictors of disease relapse and to assess their predictive value. RESULTS Over a median follow-up of 26 (range, 6-123) months, 3/71 (4.2%) patients died. Of the remaining 68 patients, 47 (69.1%) patients had achieved clinical remission and 21 (30.9%) had suffered relapse at the last follow-up. The independent predictors of relapse were IgG4 ≥ 6.5 g/L (HR = 2.84, 95% CI: 1.11-7.23), IgG ≥ 20.8 g/L (HR = 4.11, 95% CI: 1.53-11.06), IgG4-RD responder index (RI) ≥ 9 (HR = 3.82, 95% CI: 1.28-11.37), and severe IgG4+ plasma cell infiltration (HR = 6.32, 95% CI: 1.79-22.41). A prognostic score developed using three of the identified predictors (IgG ≥ 20.8 g/L, IgG4-RD RI ≥ 9, and severe IgG4+ plasma cell infiltration) showed good value for predicting impending relapse (AUC, 0.806). CONCLUSIONS In patients with IgG4-RD, IgG4 ≥ 6.5 g/L, IgG ≥ 20.8 g/L, IgG4-RD responder index (RI) ≥ 9, and severe IgG4+ plasma cell infiltration are predictors of relapse.
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Affiliation(s)
- Ji Zongfei
- Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China
| | - Chen Lingli
- Department of Pathology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China
| | - Sun Ying
- Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China
| | - Ma Lingying
- Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China
| | - Zhang Lijuan
- Department of Rheumatology, Xiamen Branch, Zhongshan Hospital, Fudan University, 668 Jinhu Road, Xiamen, Fujian Province, China
| | - Liu Dongmei
- Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China
| | - Dai Xiaomin
- Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China
| | - Hou Yingyong
- Department of Pathology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China
| | - Chen Huiyong
- Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China
| | - Ma Lili
- Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China
- Center of Clinical Epidemiology and Evidence-based Medicine, Fudan University, Shanghai, China
| | - Jiang Lindi
- Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China.
- Center of Clinical Epidemiology and Evidence-based Medicine, Fudan University, Shanghai, China.
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Long-Term Follow-Up and Immunomonitoring of Relapsing Type 1 Autoimmune Pancreatitis Treated With Rituximab. Pancreas 2022; 51:452-462. [PMID: 35835119 DOI: 10.1097/mpa.0000000000002048] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
OBJECTIVES To evaluate the efficacy and safety of rituximab in relapsing type 1 autoimmune pancreatitis especially the long-term clinical and immunologic impacts. METHODS All consecutive patients with type 1 autoimmune pancreatitis were retrospectively included. The rituximab protocol was induction therapy of 375 mg·m -2 intravenous weekly for 4 weeks, followed by 500 mg intravenous every 6 months for 2 years. The follow-up included clinical examinations, biological tests, positron emission tomography scan, and immunomonitoring of lymphocyte CD 19+. RESULTS Among the 43 patients included, 15 received rituximab induction therapy, followed by maintenance in 10 cases because of 1 or more relapses after steroids (whether or not followed by immunosuppressants) and multiple organ involvement. All patients had a clinical, biological and morphological response, a deep and persistent drop in serum immunoglobulin G4 levels, an extinction of both pancreatic and extra pancreatic hypermetabolic positron emission tomography scan signals, and a depletion of B lymphocyte CD19+. No relapse occurred during the follow-up (62.8 ± standard error of the mean of 11.1 months). CONCLUSIONS Rituximab is an effective treatment for type 1 autoimmune pancreatitis that provides a rapid strong clinical, biological, and morphological response, which persists after discontinuation without any safety issues.
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Faz-Muñoz D, Hinojosa-Azaola A, Mejía-Vilet JM, Uribe-Uribe NO, Rull-Gabayet M, Muñoz-Castañeda WR, Vargas-Parra NJ, Martín-Nares E. ANCA-associated vasculitis and IgG4-related disease overlap syndrome: a case report and literature review. Immunol Res 2022; 70:550-559. [PMID: 35449491 PMCID: PMC9023041 DOI: 10.1007/s12026-022-09279-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2021] [Accepted: 04/09/2022] [Indexed: 12/11/2022]
Abstract
Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides are infrequent autoimmune diseases characterized by inflammation of the walls of small vessels leading to tissue and endothelial damage. On the other hand, IgG4-related disease is a fibroinflammatory disease characterized histologically by lymphoplasmacytic infiltrates with IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis that may affect nearly every organ of the body. There are similarities in clinical, serological, radiological, and histopathological features between both diseases, and hence, they usually mimic each other complicating the differential diagnosis. Furthermore, reports of patients with the coexistence of both conditions (overlap syndrome) have been reported. We herein report a patient with an unequivocal diagnosis of ANCA-associated vasculitis, specifically granulomatosis with polyangiitis (posterior uveitis, polyneuropathy, pauci-immune glomerulonephritis with crescent formation and granulomas, and MPO-ANCA positivity) and IgG4-related disease (thoracic aortitis, tubulointerstitial nephritis with prominent IgG4+ plasma cell infiltration, fibrosis, and obliterative arteritis, high levels of serum IgG4, and eosinophilia) overlap syndrome.
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Affiliation(s)
- David Faz-Muñoz
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, Mexico City, Mexico, 14080
| | - Andrea Hinojosa-Azaola
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, Mexico City, Mexico, 14080
| | - Juan M Mejía-Vilet
- Department of Nephrology and Mineral Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Norma O Uribe-Uribe
- Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Marina Rull-Gabayet
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, Mexico City, Mexico, 14080
| | - Wallace Rafael Muñoz-Castañeda
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, Mexico City, Mexico, 14080
| | - Nancy Janeth Vargas-Parra
- Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Eduardo Martín-Nares
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, Mexico City, Mexico, 14080.
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He JW, Zou QM, Pan J, Wang SS, Xiang ST. Immunoglobulin G4-related kidney disease involving the renal pelvis and perirenal fat: A case report. World J Clin Cases 2022; 10:2510-2515. [PMID: 35434058 PMCID: PMC8968594 DOI: 10.12998/wjcc.v10.i8.2510] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/22/2021] [Revised: 10/30/2021] [Accepted: 02/10/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Immunoglobulin (Ig) G4-related disease (IgG4-RD) is an autoimmune disease associated with chronic and progressive inflammation and fibrosis. It is difficult to differentiate IgG4-RD involving the kidney from infectious diseases and malignancy on imaging.
CASE SUMMARY We report the case of a 51-year-old Chinese man whose abdominal computed tomography scan showed diffuse bilateral enlargement of the kidneys and perirenal fat, thickening of the renal pelvic walls, and hydronephrosis of the right kidney. Relevant laboratory test results showed a serum creatinine level of 464 μmol/L. The patient was diagnosed with acute renal failure and was started on intermittent hemodialysis. Further tests revealed high serum IgG4 levels (20.8 g/L) and an enlarged right submaxillary lymph node. Biopsy and histopathological examination of the enlarged node led to the diagnosis of IgG4-RD. After corticosteroid therapy, his serum creatinine level quickly decreased to near normal levels.
CONCLUSION IgG4-RD affecting the renal pelvis or perirenal fat is rare, with atypical imaging features. Multidisciplinary consultation is critical for accurate diagnosis and treatment of this disease. Suspected cases should undergo biopsy to avoid misdiagnosis.
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Affiliation(s)
- Jun-Wei He
- Department of Urology, the Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510000, Guangdong Province, China
| | - Qian-Ming Zou
- Department of Urology, the Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510000, Guangdong Province, China
| | - Jun Pan
- Department of Urology, the Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510000, Guangdong Province, China
| | - Shu-Sheng Wang
- Department of Urology, the Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510000, Guangdong Province, China
| | - Song-Tao Xiang
- Department of Urology, the Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510000, Guangdong Province, China
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Tawhari M, Al Oudah N, Al Zahrani Y, Radwi M. IgG4-Related Kidney Disease Associated With End-Stage Kidney Disease, Renal Pseudotumor, and Renal Vein Thrombosis. Cureus 2022; 14:e22837. [PMID: 35281583 PMCID: PMC8906446 DOI: 10.7759/cureus.22837] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/04/2022] [Indexed: 11/05/2022] Open
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A rare cause of chronic tubulointerstitial nephritis in childhood: Answers. Pediatr Nephrol 2022; 37:571-575. [PMID: 34734329 DOI: 10.1007/s00467-021-05326-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2021] [Revised: 09/29/2021] [Accepted: 09/30/2021] [Indexed: 10/19/2022]
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Ishibuchi K, Iwakura T, Ema C, Nakagami D, Uchiyama Y, Kaneko M, Fukasawa H, Matsuyama T, Yasuda H, Furuya R. A Case of M-Type Phospholipase A2 Receptor-Associated Membranous Nephropathy With IgG4-Positive Cells Infiltration in the Interstitium. CLINICAL MEDICINE INSIGHTS-CASE REPORTS 2022; 15:11795476221078635. [PMID: 35221739 PMCID: PMC8874188 DOI: 10.1177/11795476221078635] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2021] [Accepted: 12/30/2021] [Indexed: 11/16/2022]
Abstract
A 70-year-old man was referred to our department for evaluation of nephrotic syndrome. Renal biopsy revealed membranous nephropathy (MN). Immunohistochemical analysis demonstrated IgG4-positive staining in the glomeruli and interstitial cells. The presence of serum anti-phospholipase A2 receptor (PLA2R) antibody and enhanced staining of PLA2R in the glomeruli was noted. Computed tomography unidentified the extrarenal lesions of IgG4-related disease. He was diagnosed with PLA2R-associated MN possibly complicated with IgG4 related kidney disease (IgG4-RKD). Storiform fibrosis, a typical manifestation of IgG4-RKD, was not apparent. We herein describe a case of serologically and histologically confirmed PLA2R-associated MN with IgG4+ cell infiltration into the interstitium without any signs of IgG4-RD.
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Affiliation(s)
- Kento Ishibuchi
- Renal Division, Department of Internal Medicine, Iwata City Hospital, Iwata, Japan
| | - Takamasa Iwakura
- Renal Division, Department of Internal Medicine, Iwata City Hospital, Iwata, Japan
- First Department of Internal medicine, Division of Nephrology, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Chiemi Ema
- First Department of Internal medicine, Division of Nephrology, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Daisuke Nakagami
- Renal Division, Department of Internal Medicine, Iwata City Hospital, Iwata, Japan
| | - Yuri Uchiyama
- Renal Division, Department of Internal Medicine, Iwata City Hospital, Iwata, Japan
| | - Mai Kaneko
- Renal Division, Department of Internal Medicine, Iwata City Hospital, Iwata, Japan
| | - Hirotaka Fukasawa
- Renal Division, Department of Internal Medicine, Iwata City Hospital, Iwata, Japan
| | - Takashi Matsuyama
- First Department of Internal medicine, Division of Nephrology, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Hideo Yasuda
- First Department of Internal medicine, Division of Nephrology, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Ryuichi Furuya
- Renal Division, Department of Internal Medicine, Iwata City Hospital, Iwata, Japan
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Kamisawa T. Immunoglobulin G4-related Disease: A New Systemic Disease Emerging in Japan. JMA J 2022; 5:23-35. [PMID: 35224257 PMCID: PMC8826784 DOI: 10.31662/jmaj.2021-0113] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2021] [Accepted: 10/05/2021] [Indexed: 11/16/2022] Open
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a fibro-inflammatory disease characterized by organ enlargement and elevated serum IgG4 levels. In 2003, IgG4-RD was proposed as a distinct form of IgG4-related systemic disease based on a histopathological study involving patients with autoimmune pancreatitis. IgG4-RD occurs mainly in older men and can affect almost any organ simultaneously or metachronously. Pathophysiologically, IgG4-RD occurs when an autoantigen triggers an immune response characterized by Th2 predominance with increased production of cytokines, such as interleukin 4 (IL-4), IL-5, IL-10, IL-13, and tumor growth factor-β (TGF-β), in the affected organ. IL-10 and TGF-β produced by the increased number of regulatory T cells induce a switch from B cells to IgG4-producing plasma cells and fibrosis, respectively. The characteristic histological features consist of dense infiltration of lymphocytes and IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. IgG4-RD is diagnosed based on a combination of clinical, serological, radiological, and histopathological findings. Differentiating IgG4-RD from malignant tumors or similar inflammatory diseases in the affected organs is important. The 2019 America College of Rheumatology/European League against Rheumatism classification criteria for IgG4-RD have high diagnostic sensitivity and specificity. IgG4-RD generally responds well to treatment with steroids, and a swift response is reassuring and provides further diagnostic confirmation. However, relapses are common during tapering or after cessation of steroids. In Japan, low-dose steroid maintenance therapy is usually given to prevent a relapse. B-cell depletion with rituximab is effective in patients resistant to or dependent on steroids. Most patients with IgG4-RD who receive steroid therapy show good short-term clinical, morphological, and functional outcomes. However, long-term outcomes, such as relapse, fibrosis development, and associated malignancies, have not been clearly defined. Therefore, novel treatment strategies, including rituximab, need to be tested in international randomized controlled clinical trials.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan
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Update on classification, diagnosis, and management of immunoglobulin G4-related disease. Chin Med J (Engl) 2022; 135:381-392. [PMID: 34985023 PMCID: PMC8869566 DOI: 10.1097/cm9.0000000000001891] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized chronic fibro-inflammatory autoimmune disease, and its recognition has been constantly increasing worldwide over the last few years. A correct and timely recognition, as well as appropriate intervention, is crucial for the treatment of IgG4-RD. For certain subtypes of IgG4-RD, organ-specific criteria are formulated to make the diagnosis more accurate. New biomarkers have emerged in the recent years to aid the disease diagnosis, its prognosis prediction, as well as therapy response monitoring. Although recurrence is very common in IgG4-RD, glucocorticoid is still the first-line treatment for the majority of patients. The factors that affect the likelihood of disease relapse are multifaceted. The selection strategy of various steroid-sparing agents is still being explored. Besides, when patients have special sites involvement leading to severe clinical conditions, surgical operation or interventional therapy should also be considered. An update on classification, diagnosis, and management of IgG4-RD is provided in the current study to fully elucidate the recommended clinical practice of this mysterious disease.
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Amendment of the Japanese consensus guidelines for autoimmune pancreatitis, 2020. J Gastroenterol 2022; 57:225-245. [PMID: 35192048 PMCID: PMC8938398 DOI: 10.1007/s00535-022-01857-9] [Citation(s) in RCA: 47] [Impact Index Per Article: 15.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/03/2021] [Accepted: 01/28/2022] [Indexed: 02/04/2023]
Abstract
In response to the latest knowledge and the amendment of the Japanese diagnostic criteria for autoimmune pancreatitis (AIP) in 2018, the Japanese consensus guidelines for managing AIP in 2013 were required to be revised. Three committees [the professional committee for developing clinical questions (CQs) and statements by Japanese specialists; the expert panelist committee for rating statements by the modified Delphi method; and the evaluating committee of moderators] were organized. Twenty specialists in AIP extracted the specific clinical statements from a total of 5218 articles (1963-2019) from a search in PubMed and the Cochrane Library. The professional committee made 14, 9, 5, and 11 CQs and statements for the current concept and diagnosis, extra-pancreatic lesions, differential diagnosis, and treatment, respectively. The expert panelists regarded the statements as valid after a two-round modified Delphi approach with individually rating these clinical statements, in which a clinical statement receiving a median score greater than 7 on a 9-point scale from the panel was regarded as valid. After evaluation by the moderators, the amendment of the Japanese consensus guidelines for AIP has been proposed in 2020.
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Nakano K, Sugita J, Mafune N, Seimiya M, Yasuda K, Watanabe C, Teshima T. IgG4-IgE complex in a patient with IgG4-related disease. Clin Chim Acta 2022; 528:52-55. [DOI: 10.1016/j.cca.2022.01.013] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/28/2021] [Revised: 12/29/2021] [Accepted: 01/18/2022] [Indexed: 01/13/2023]
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Yoshida H, Takahashi N, Horiguchi T, Yasuhara H, Tanaka T, Chen Y, Takasaki T, Tsukao H, Yoshida M, Kawakami S, Ohta M, Naiki H, Konishi S, Ito I, Iwano M. Development of Myeloperoxidase Anti-neutrophil Cytoplasmic Antibody-positive Necrotizing Crescentic Glomerulonephritis in an Elderly Patient with Immunological Kidney Disease. Intern Med 2021; 60:3927-3935. [PMID: 34148959 PMCID: PMC8758455 DOI: 10.2169/internalmedicine.7252-21] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 78-year-old man presented with hypercalcemia and renal disease with high serum IgG4 and positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), exhibiting sarcoidosis-like chest findings. A renal biopsy revealed tubulointerstitial nephritis, membranous nephropathy (MN), and sub-capsular lymphoid aggregates without fulfilling the diagnostic criteria of IgG4-related disease or sarcoidosis. Steroid therapy ameliorated the serological and renal abnormalities. After 5 years, following gradual increases in the neutrophil count and upper respiratory infection (URI), necrotizing crescentic glomerulonephritis (NCGN) developed with an increased serum MPO-ANCA level. These results suggest that in the presence of MPO-ANCA in immune senescence, the persistent neutrophil increase with URI may lead to the development of NCGN.
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Affiliation(s)
- Haruyoshi Yoshida
- Department of Internal Medicine, Sugita Genpaku Memorial Obama Municipal Hospital, Japan
| | | | - Takayasu Horiguchi
- Department of Internal Medicine, Sugita Genpaku Memorial Obama Municipal Hospital, Japan
| | - Hiroki Yasuhara
- Department of Internal Medicine, Sugita Genpaku Memorial Obama Municipal Hospital, Japan
| | - Tokuharu Tanaka
- Department of Internal Medicine, Sugita Genpaku Memorial Obama Municipal Hospital, Japan
| | - Yuhao Chen
- Department of Internal Medicine, Sugita Genpaku Memorial Obama Municipal Hospital, Japan
| | - Toshikazu Takasaki
- Department of Internal Medicine, Sugita Genpaku Memorial Obama Municipal Hospital, Japan
| | - Hitokazu Tsukao
- Department of Internal Medicine, Sugita Genpaku Memorial Obama Municipal Hospital, Japan
| | - Michiko Yoshida
- Department of Internal Medicine, Sugita Genpaku Memorial Obama Municipal Hospital, Japan
| | - Satoshi Kawakami
- Department of Diagnostic Radiology, Sugita Genpaku Memorial Obama Municipal Hospital, Japan
| | - Makoto Ohta
- Department of Pathology, Hikone Municipal Hospital, Japan
| | - Hironobu Naiki
- Department of Molecular Pathology, University of Fukui, Japan
| | - Satoshi Konishi
- Department of Respiratory Medicine, Kyoto University Hospital, Japan
| | - Isao Ito
- Department of Respiratory Medicine, Kyoto University Hospital, Japan
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Watanabe K, Kamisawa T, Chiba K, Kikuyama M, Nakahodo J, Igarashi Y. Gallbladder wall thickening in patients with IgG4-related diseases, with special emphasis on IgG4-related cholecystitis. Scand J Gastroenterol 2021; 56:1456-1461. [PMID: 34486468 DOI: 10.1080/00365521.2021.1971758] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
OBJECTIVES Gallbladder (GB) wall thickening sometimes occurs in patients with autoimmune pancreatitis (AIP), a condition for which the name, IgG4-related cholecystitis, was proposed. We examined the radiological findings of the GB in patients with IgG4-related diseases and clinical features of patients with GB wall thickening and presented a hypothesis of its pathogenesis. MATERIALS AND METHODS GB wall thickening was defined by thickness ≥ 4 mm. GB wall thickness was examined in 258 patients with IgG4-related disease. Clinical and imaging findings of 200 patients with AIP with and without GB wall thickening were then compared. RESULTS GB wall thickening was detected in 58 patients (29%) with AIP and two patients with isolated IgG4-related sclerosing cholangitis. In the 60 GBs examined, wall thickening was diffuse, with the walls possessing a smooth inner surface. No GB wall thickening was detected among the 56 patients with IgG4-related disease without AIP or IgG4-related sclerosing cholangitis. Bile duct stenosis was detected in 56 patients (97%) with AIP with GB wall thickening. Intraductal ultrasonography indicated cystic duct wall thickening connected to bile duct wall thickening in 11 of 14 (79%) patients with AIP or IgG4-related sclerosing cholangitis with GB wall thickening. Forty-eight patients in whom IgG4-related cholecystitis was diagnosed experienced resolution of the GB wall thickening after receiving steroid therapy. CONCLUSIONS Most cases of GB wall thickening in IgG4-related diseases are closely associated with IgG4-related sclerosing cholangitis and may be a manifestation of IgG4-related disease throughout the biliary tract, including the bile duct, cystic duct, and GB.
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Affiliation(s)
- Koji Watanabe
- Department of Internal Medicine, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, Japan.,Division of Gastroenterology and Hepatology, Department of Internal Medicine, Toho University Omori Medical Center, Tokyo, Japan
| | - Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, Japan
| | - Kazuro Chiba
- Department of Internal Medicine, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, Japan
| | - Masataka Kikuyama
- Department of Internal Medicine, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, Japan
| | - Jun Nakahodo
- Department of Internal Medicine, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, Japan
| | - Yoshinori Igarashi
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Toho University Omori Medical Center, Tokyo, Japan
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Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory disease. Since its discovery nearly two decades ago, our understanding of its pathophysiology and clinical manifestations has grown substantially. Early diagnosis and treatment of this elusive disease can prevent substantial organ damage from end-stage fibrosis, emphasizing the need for prompt recognition and accurate characterization of IgG4-RD. The classification criteria endorsed by the American College of Rheumatology and the European Alliance of Associations for Rheumatology in 2019 provide a framework for establishing the diagnosis in the clinical setting. This process involves recognizing the typical manifestations of the disease and incorporating clinical, radiological, serological, and histopathological information as well as excluding disease mimickers. Glucocorticoids and rituximab are effective at inducing remission in IgG4-RD in most patients, but the optimal approach to long-term management of IgG4-RD remains an area of active clinical research. Expected final online publication date for the Annual Review of Medicine, Volume 73 is January 2022. Please see http://www.annualreviews.org/page/journal/pubdates for revised estimates.
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Affiliation(s)
- Guy Katz
- Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston, Massachusetts 02114, USA;
| | - John H Stone
- Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston, Massachusetts 02114, USA;
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Liu J, Yin W, Westerberg LS, Lee P, Gong Q, Chen Y, Dong L, Liu C. Immune Dysregulation in IgG 4-Related Disease. Front Immunol 2021; 12:738540. [PMID: 34539675 PMCID: PMC8440903 DOI: 10.3389/fimmu.2021.738540] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2021] [Accepted: 08/09/2021] [Indexed: 12/26/2022] Open
Abstract
Immunoglobin G4-related disease (IgG4-RD) is one of the newly discovered autoimmune diseases characterized by elevated serum IgG4 concentrations and multi-organ fibrosis. Despite considerable research and recent advances in the identification of underlying immunological processes, the etiology of this disease is still not clear. Adaptive immune cells, including different types of T and B cells, and cytokines secreted by these cells play a vital role in the pathogenesis of IgG4-RD. Antigen-presenting cells are stimulated by pathogens and, thus, contribute to the activation of naïve T cells and differentiation of different T cell subtypes, including helper T cells (Th1 and Th2), regulatory T cells, and T follicular helper cells. B cells are activated and transformed to plasma cells by T cell-secreted cytokines. Moreover, macrophages, and some important factors (TGF-β, etc.) promote target organ fibrosis. Understanding the role of these cells and cytokines implicated in the pathogenesis of IgG4-RD will aid in developing strategies for future disease treatment and drug development. Here, we review the most recent insights on IgG4-RD, focusing on immune dysregulation involved in the pathogenesis of this autoimmune condition.
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Affiliation(s)
- Jiachen Liu
- Department of Pathogen Biology, School of Basic Medicine, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Wei Yin
- Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Lisa S Westerberg
- Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm, Sweden
| | - Pamela Lee
- Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, China
| | - Quan Gong
- Department of Immunology, School of Medicine, Yangtze University, Jingzhou, China
| | - Yan Chen
- The Second Department of Pediatrics, Affiliated Hospital of Zunyi Medical University, Zunyi, China
| | - Lingli Dong
- Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Chaohong Liu
- Department of Pathogen Biology, School of Basic Medicine, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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Osuorji C, Master K, Osuorji I. IgG4-Related Disease With Renal and Pulmonary Involvement. Cureus 2021; 13:e17071. [PMID: 34522549 PMCID: PMC8428951 DOI: 10.7759/cureus.17071] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/10/2021] [Indexed: 12/04/2022] Open
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated disease affecting multiple organs and tissues. There is often the presence of elevated serum Ig4 subtype with histological evidence of lymphoplasmacytic infiltration, fibrosis, and phlebitis. The mainstay of treatment is steroids therapy. We report the case of a 66-year-old man presenting with acute on chronic renal failure and pulmonary nodules seen on PET-CT scan. He also had elevated serum IgG4 subclass and histological features in keeping with IgG4-RD. He failed steroid therapy but responded subsequently to rituximab with complete resolution of his symptoms.
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Affiliation(s)
- Chinenye Osuorji
- Internal Medicine, Burrell College of Osteopathic Medicine, Las Cruces, USA
| | - Kiron Master
- Radiology, Hospitals of Providence Sierra Campus, El Paso, USA
| | - Ikenna Osuorji
- Hematology and Oncology, Burrell College of Osteopathic Medicine, Las Cruces, USA
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49
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Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition of unknown etiology, with presumed autoimmune mechanisms. It is characterized by high levels of IgG4 and variable clinical manifestations. It can involve one or multiple organs. Herein, we reported the case of a 62-year-old man with three organs involvement. He initially presented with recurrent jaundice. Laboratory analysis revealed cholestasis, high gamma-globulin levels, renal failure, and proteinuria. Abdominal Magnetic Resonance Imaging (MRI) showed segmental strictures of the left intrahepatic bile ducts and the wirsung duct with an increased volume of the pancreas and diffuse bilateral enlargement of the kidneys. Laboratory tests revealed high IgG4 levels (770 mg/dL). Based on the biological and radiological findings, we have suggested the diagnosis of systemic IgG4-related disease involving bile ducts, the pancreas, and probably the kidneys. Renal biopsy revealed lymphoplasmacytic infiltrate and fibrosis, but no IgG4-positive cell. The patient received corticosteroid therapy with a complete resolution of all symptoms and a rapid normalization of all blood tests. The present case underlines the complexity of IgG4-RD because of its variable clinical presentation. The diagnosis is challenging and should be carefully assessed for possible multi-organ involvement.
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Tian M, Luan J, Jiao C, Chang Q, Kopp JB, Zhou H. Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report. BMC Nephrol 2021; 22:279. [PMID: 34384379 PMCID: PMC8358553 DOI: 10.1186/s12882-021-02477-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2021] [Accepted: 07/19/2021] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Cases of concurrent immunoglobulin A nephropathy (IgAN) and IgG4-related tubulointerstitial nephritis (IgG4-TIN) are rare and previous case reports have lacked important data. KDIGO suggests a treatment with systemic glucocorticoids in IgAN patients. Glucocorticoids are recommended as the first-line therapy for IgG4-TIN. The use of tacrolimus as a long-term maintenance treatment has not been described. We report the case of a man who developed IgAN and IgG4-TIN without abnormalities in extra-renal tissue, without renal function abnormalities or impairment as well, and was treated by tacrolimus as a long-term maintenance during 45 months follow-up. CASE PRESENTATION A 56-year-old Chinese man first presented to our hospital with the chief complaint of foamy urine for 1 year and hematuria for 3 months, with a medical history of hypertension. Testing revealed a notable increase in serum IgG4 level without abnormalities in renal function or imaging, or in dysfunction other organs. Renal biopsy showed mesangial extracellular matrix proliferation, increased mesangial cell numbers and infiltration of plasma cells. Immunofluorescence showed mesangial positivity for IgA and C3. Immunohistochemistry staining showed widespread IgG4 and increased CD38 and CD138 expression. Electron microscopy showed immune complexes located on the tubular basement membrane. He was diagnosed with IgAN and IgG4-TIN. He received glucocorticoids, leflunomide and tacrolimus to induce remission. He was given tacrolimus as long-term maintenance treatment. When tacrolimus was temporarily withdrawn, proteinuria recurred. After resuming tacrolimus therapy, he again entered complete remission. After 45 months of therapy, he remains in complete remission and the serum IgG4 level is normal. CONCLUSIONS The finding of concurrent IgAN and IgG4-TIN without abnormalities in renal function, imaging or extra-renal tissue is rare and their coexistence may be coincidental. Long-term treatment with tacrolimus proved effective and he has remained in remission during 45 months follow-up.
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Affiliation(s)
- Mi Tian
- Department of Nephrology, Shengjing Hospital of China Medical University, 36 Sanhao St, Shenyang, 110004, Liaoning, China
| | - Junjun Luan
- Department of Nephrology, Shengjing Hospital of China Medical University, 36 Sanhao St, Shenyang, 110004, Liaoning, China
| | - Congcong Jiao
- Department of Nephrology, Shengjing Hospital of China Medical University, 36 Sanhao St, Shenyang, 110004, Liaoning, China
| | - Qing Chang
- Clinical Epidemiology, Shengjing Hospital of China Medical University, Shenyang, China
| | - Jeffrey B Kopp
- Renal Diagnostics and Therapeutics Unit, NIDDK/NIH, Bethesda, MD, USA
| | - Hua Zhou
- Department of Nephrology, Shengjing Hospital of China Medical University, 36 Sanhao St, Shenyang, 110004, Liaoning, China.
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