|
1
|
Zhang H, Lin J, Lu H, Zhong Y, Deng L, Kuang B, Li Q. Systemic lupus erythematosus with podocyte infolding glomerulopathy: A case report and literature review. Medicine (Baltimore) 2024; 103:e39809. [PMID: 39470480 PMCID: PMC11521074 DOI: 10.1097/md.0000000000039809] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/13/2024] [Accepted: 09/02/2024] [Indexed: 10/30/2024] Open
Abstract
RATIONALE Podocyte infolding glomerulopathy (PIG) is a rare glomerular disease, its diagnosis mainly depends on pathological manifestations of the kidney. Few clinical cases of PIG have been reported, but it is sometimes associated with connective tissue diseases. Here we describe a case of systemic lupus erythematosus (SLE) with PIG and undertake a review of the literature. PATIENT CONCERNS A 34-year-old female patient was admitted to our hospital in August 2019 with repeated facial erythema and proteinuria for more than 10 years. The patient was previously diagnosed with SLE. DIAGNOSIS Systemic lupus erythematosus. INTERVENTIONS Renal biopsy was performed to investigate ongoing proteinuria and the results were consistent with PIG. Treatment with methylprednisolone, hydroxychloroquine sulfate, mycophenolate mofetil, and candesartan ester. OUTCOMES Improved the patient's condition and resolved the proteinuria. LESSONS This study reported a case of PIG and SLE. The patient was diagnosed according to biopsy, and the disease remain stable after immunosuppressive therapy. It is recommended to carefully study renal biopsies from patients with proteinuria and underlying autoimmune diseases to identify additional cases.
Collapse
Affiliation(s)
- Huiqing Zhang
- Guangzhou University of Chinese Medicine, Dongguan Hospital of Traditional Chinese Medicine, Dongguan, Guangdong 523000, China
| | - Jie Lin
- Guangzhou University of Chinese Medicine, Dongguan Hospital of Traditional Chinese Medicine, Dongguan, Guangdong 523000, China
| | - Hanqi Lu
- Guangzhou University of Chinese Medicine, Dongguan Hospital of Traditional Chinese Medicine, Dongguan, Guangdong 523000, China
| | - Yunliang Zhong
- Guangzhou University of Chinese Medicine, Dongguan Hospital of Traditional Chinese Medicine, Dongguan, Guangdong 523000, China
| | - Lie Deng
- Guangzhou University of Chinese Medicine, Dongguan Hospital of Traditional Chinese Medicine, Dongguan, Guangdong 523000, China
| | - Bin Kuang
- Guangzhou University of Chinese Medicine, Dongguan Hospital of Traditional Chinese Medicine, Dongguan, Guangdong 523000, China
| | - Qiang Li
- Guangzhou University of Chinese Medicine, Dongguan Hospital of Traditional Chinese Medicine, Dongguan, Guangdong 523000, China
| |
Collapse
|
|
2
|
Hong L, Wang L, Wang H, Wang Q, Yang S, Tian T, Cui T, Yue S, Hou X, Zheng Z, Chen W. Podocyte Infolding Glomerulopathy: A Special Morphology of Podocyte Injury Caused by Heterogeneous Diseases. Kidney Int Rep 2023; 8:2742-2753. [PMID: 38106587 PMCID: PMC10719646 DOI: 10.1016/j.ekir.2023.09.014] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2023] [Revised: 09/07/2023] [Accepted: 09/11/2023] [Indexed: 12/19/2023] Open
Abstract
Introduction Podocyte infolding glomerulopathy (PIG) is a newly recognized rare glomerular injury. The clinical significance and mechanism of this injury pattern remains unclear. Methods We conducted a retrospective study of renal biopsies from January 2018 to December 2020 in Kingmed Diagnostics. The renal biopsy features and clinical data were reviewed. Laser scanning microdissection and mass spectrometry (LMD/MS) was conducted to analyze the potential mechanism. Results A total of 116 (0.092%) out of 126,086 biopsies were diagnosed as PIG during the period. Of these, 89 (76.7%) cases were found to have PIG coexisting with immune-complex associated glomerulonephritis (IC-PIG) whereas 27 (23.3%) were identified as isolated PIG without immunoglobulin or complement deposition. Systemic lupus erythematosus (SLE), especially with membranous lupus nephritis (LN), was diagnosed in most (70.8%) IC-PIG cases. Of the isolated PIG cases, 51.9% had no known underlying conditions; however, a relatively high positive rate (42.1%) of antinuclear antibody (ANA) was detected. Nearly half (47.5%) of the patients presented with nephrotic syndrome (NS). PIG grade was associated with proteinuria in isolated PIG (P = 0.035). LMD/MS revealed dysregulated cytoskeletal protein α-actinin4 (ACTN4) and tubulin beta-4 chain in PIG compared with normal donor kidney and minimal change disease (MCD). The displacement of ACTN4 into the glomerular basement membrane (GBM) was confirmed by the confocal microscope. Conclusion PIG is a rare podocyte injury that can exist alone without underlying disease or be concurrent with various diseases, especially SLE. Podocyte cytoskeletal protein ACTN4 and tubulin beta-4 chain were dysregulated, which may be involved in the mechanism of PIG.
Collapse
Affiliation(s)
- Ling Hong
- Department of Nephrology, Center of Kidney and Urology, The Seventh Affiliated Hospital, Sun Yat-sen University, Shenzhen, China
- Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Lin Wang
- Department of Renal Pathology, Guangzhou Kingmed Diagnostic Laboratory Ltd, Guangzhou International Biological Island, Guangzhou, China
| | - Honglei Wang
- Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Qihua Wang
- Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Shicong Yang
- Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Tian Tian
- Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Tianjiao Cui
- Department of Nephrology, Center of Kidney and Urology, The Seventh Affiliated Hospital, Sun Yat-sen University, Shenzhen, China
| | - Shuling Yue
- Department of Renal Pathology, Guangzhou Kingmed Diagnostic Laboratory Ltd, Guangzhou International Biological Island, Guangzhou, China
| | - Xiaotao Hou
- Department of Renal Pathology, Guangzhou Kingmed Diagnostic Laboratory Ltd, Guangzhou International Biological Island, Guangzhou, China
| | - Zhihua Zheng
- Department of Nephrology, Center of Kidney and Urology, The Seventh Affiliated Hospital, Sun Yat-sen University, Shenzhen, China
| | - Wenfang Chen
- Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| |
Collapse
|
|
3
|
Chang MY, Zhang Y, Li MX, Xuan F. Integrated Chinese and Western medicine in the treatment of a patient with podocyte infolding glomerulopathy: A case report. World J Clin Cases 2023; 11:4684-4691. [PMID: 37469726 PMCID: PMC10353505 DOI: 10.12998/wjcc.v11.i19.4684] [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: 02/28/2023] [Revised: 05/16/2023] [Accepted: 05/31/2023] [Indexed: 06/30/2023] Open
Abstract
BACKGROUND Podocyte infolding glomerulopathy (PIG) is a newly described and rare glomerular disease. To date, only approximately 40 cases have been reported globally.
CASE SUMMARY A 26-year-old female patient presented to our hospital with a complaint of intermittent edema of both lower limbs over the past 2 years. The patient was diagnosed with PIG. She was prescribed corticosteroid therapy in other hospitals during the initial stage, to which she had responded poorly and had developed femoral head necrosis. Therefore, we administered immunosuppressants, renin-angiotensin system inhibitors, combined with traditional Chinese medicine. The patient was followed for 1 year, during which her clinical condition improved.
CONCLUSION Integrated Chinese and Western medicine may be effective for PIG treatment, which requires active intervention to improve prognosis.
Collapse
Affiliation(s)
- Mei-Ying Chang
- Department of Nephrology, Xiyuan Hospital, China Academy of Chinese Medical Sciences, Beijing 100091, China
| | - Yu Zhang
- Department of Nephrology, Xiyuan Hospital, China Academy of Chinese Medical Sciences, Beijing 100091, China
| | - Ming-Xu Li
- Department of Nephrology, First Medical Center of Chinese PLA General Hospital, Nephrology Institute of the Chinese People’s Liberation Army, State Key Laboratory of Kidney Diseases, National Clinical Research Center for Kidney Diseases, Beijing Key Laboratory of Kidney Disease Research, Beijing 100853, China
| | - Fang Xuan
- Department of Nephrology, First Medical Center of Chinese PLA General Hospital, Nephrology Institute of the Chinese People’s Liberation Army, State Key Laboratory of Kidney Diseases, National Clinical Research Center for Kidney Diseases, Beijing Key Laboratory of Kidney Disease Research, Beijing 100853, China
| |
Collapse
|
|
4
|
Podocyte Infolding Glomerulopathy: A Case Series Report and Literature Review. J Clin Med 2023; 12:jcm12031088. [PMID: 36769735 PMCID: PMC9918010 DOI: 10.3390/jcm12031088] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/03/2023] [Revised: 01/19/2023] [Accepted: 01/28/2023] [Indexed: 02/01/2023] Open
Abstract
BACKGROUND Podocyte infolding glomerulopathy (PIG) is a peculiar and very rare manifestation in renal pathology. Its underlying pathogenesis mechanism and clinical characteristics remain unclear due to sparse reports. OBJECTIVE To further elucidate the clinical profile of PIG by carefully reporting our four cases and a comprehensive review of cases in the literature. METHODS This study retrospectively reviewed four cases of PIG from 2010 to 2022 in our centre. Clinical and pathological profiles were reported. PIG cases in the literature were searched in the MEDLINE database and analysed together with our cases. RESULTS Four cases of PIG identified from our centre and 40 cases from the current literature were reported. The pooled analysis of these 44 cases indicated 79.5% (35/44) were females, 93.2% (41/44) were East Asians, and 63.6% (28/44) were reported in Japan. The average age was 42.0 ± 12.5 years old. The average amount of proteinuria at the time of renal biopsy was 3.06 ± 3.2 g/day. The most reported comorbidities were connective tissue diseases, mainly systemic lupus erythematosus, and 20.5% (9/44) of the cases did not have any contaminant disease. Most of the cases (81.8%, 36/44) had been treated with immunosuppressants, of which a combination of corticosteroids and one other type of immunosuppressant was most commonly reported. In addition, 45.4% (20/44) and 34.1% (15/44) of the cases had achieved complete response and partial response, respectively, after treatment. Whole exosome sequencing indicated mutations in the INF2 gene. CONCLUSIONS PIG is a rare condition and seen in relatively younger populations, often associated with connective tissue diseases clinically and one or two other glomerulopathies histologically. The outcomes following immunosuppressive treatment are relatively good. Mutations in INF2 might be involved in the development of PIG; however, the implications of these results need to be investigated.
Collapse
|
|
5
|
Choung HYG, Jean-Gilles J, Goldman B. Subepithelial deposits with microspherular structures in membranous glomerulonephritis. Ultrastruct Pathol 2022; 46:377-387. [PMID: 35709324 DOI: 10.1080/01913123.2022.2090646] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
Rare cases of membranous glomerulopathy (MGN) with subepithelial deposits consisting of microspherular structures identified by electron microscopy have been described in the literature as either MGN with spherules or podocyte infolding glomerulopathy (PIG). The paucity of available studies shows a strong association with underlying autoimmune disease. To further understand the significance of subepithelial microspherular deposits, we retrospectively identified native kidney biopsies from 10 patients diagnosed as MGN with subepithelial microspherular structures identified by ultrastructural examination at the University of Rochester Medical Center (URMC) during an 11-year period. The majority were Caucasian (80%) with a mean age of 51.3 (±12.9) years. 50% had an autoimmune disorder, of which 80% were SLE. Two SLE cases had concomitant rheumatoid arthritis and Sjogren's syndrome. One additional case had antiphospholipid syndrome and showed lupus-like features on biopsy. 40% were idiopathic and negative for PLA2R, NELL1, and THSD7A. MGN with subepithelial microspherular structures is frequently associated with an underlying autoimmune disease. The majority are negative for markers of primary MGN (PLA2R, THSD7A, and NELL1) and show features suggestive of secondary MGN.
Collapse
Affiliation(s)
- Hae Yoon Grace Choung
- Department of Pathology and Laboratory Medicine, Division of Renal Pathology and Electron Microscopy, University of Rochester Medical Center, Rochester, NY, USA
| | - Jerome Jean-Gilles
- Department of Pathology and Laboratory Medicine, Division of Renal Pathology and Electron Microscopy, University of Rochester Medical Center, Rochester, NY, USA
| | - Bruce Goldman
- Department of Pathology and Laboratory Medicine, Division of Renal Pathology and Electron Microscopy, University of Rochester Medical Center, Rochester, NY, USA
| |
Collapse
|
|
6
|
Ting JA, Hung W, McRae SA, Barbour SJ, Copland M, Riazy M. Podocyte Infolding Glomerulopathy, First Case Report From North America. Can J Kidney Health Dis 2021; 8:20543581211048357. [PMID: 34659774 PMCID: PMC8516371 DOI: 10.1177/20543581211048357] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2021] [Accepted: 09/04/2021] [Indexed: 11/16/2022] Open
Abstract
Rationale Podocyte infolding glomerulopathy (PIG) is a newly described condition with only 37 cases reported worldwide. Due to its rarity, the pathogenesis and evolution of this disease is unclear. This case report contributes to our collective knowledge about the clinical and histological progression of this disease. Presenting concerns of the patient Over the course of a year, a 52-year-old Malaysian woman with no known prior medical history developed progressively worsening edema and other findings consistent with nephrotic syndrome. Diagnosis Unlike most patients with PIG, this patient did not have any autoimmune disease. She was Hepatitis B core antibody positive with a Hepatitis B surface antibody >1000, suggesting prior Hepatitis B infection with immunity. A renal biopsy was performed which was consistent with PIG. A second renal biopsy was done 2 years later which again showed characteristic findings of PIG with worsened podocyte effacement but no interval change in chronicity. Interventions The patient was treated with blood pressure control and renin-angiotensin-aldosterone system (RAAS) blockade with irbesartan and spironolactone. She was also treated with prednisone at 1 mg/kg for 2 months followed by a taper for a total of 7 months of prednisone treatment. Outcomes The patient had a partial response to a course of prednisone. However, since stopping steroids, her proteinuria and renal function has been gradually worsening. Teaching points PIG is mostly found in patients of East Asian descent. It presents as proteinuria and is often associated with autoimmune disease but can be idiopathic. It is characterized on renal biopsy by infolding or protrusion of podocyte cytoplasm into glomerular basement membrane, as well as intramembranous cytoplasmic microspherules or microtubules. Atypical membranous nephropathy should be ruled out prior to diagnosis. Unlike membranous nephropathy, PIG usually responds at least partially to steroid monotherapy. To our knowledge, this is the first reported case of PIG from North America. Furthermore, it is the first case of PIG with repeat biopsy showing interval worsening of PIG rather than either resolution of PIG or transformation of PIG to a different diagnosis.
Collapse
Affiliation(s)
- Julie Anne Ting
- Division of Nephrology, The University of British Columbia, Vancouver, Canada
| | - Wayne Hung
- Division of Nephrology, The University of British Columbia, Vancouver, Canada
| | - Susanna A McRae
- Division of Nephrology, The University of British Columbia, Vancouver, Canada.,Department of Pathology, The University of British Columbia, Vancouver, Canada
| | - Sean J Barbour
- Division of Nephrology, The University of British Columbia, Vancouver, Canada
| | - Michael Copland
- Division of Nephrology, The University of British Columbia, Vancouver, Canada
| | - Maziar Riazy
- Division of Nephrology, The University of British Columbia, Vancouver, Canada.,Department of Pathology, The University of British Columbia, Vancouver, Canada.,Department of Pathology, St. Paul's Hospital, Vancouver, BC, Canada
| |
Collapse
|
|
7
|
Shi J, Zheng R, Gao H, Zhao Z, Wu H, Zhang Z. Podocyte infolding glomerulopathy with undifferentiated connective tissue disease: a case report. Ultrastruct Pathol 2020; 44:245-248. [PMID: 32172632 DOI: 10.1080/01913123.2020.1739791] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
Abstract
Podocyte infolding glomerulopathy (PIG) is a special type of glomerular disease that has been proposed in recent years and has attracted considerable attention. PIG is characterized by the formation of microspheres and microtubules in thickened glomerular basement membrane (GBM) on electron microscopy (EM), which is recognized as podocyte cytoplasmic infolding to the GBM. However, to date, only a few cases of PIG have been reported. Herein, we report a case of a 33-year-old female with PIG with undifferentiated connective tissue disease (UCTD) in China and review the literature.
Collapse
Affiliation(s)
- Jiaoyu Shi
- Department of Pathology, School of Basic Medical Science, Shanghai Medical College, Fudan University, Shanghai, China
| | - Rong Zheng
- Department of Nephrology, Longhua Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai, China
| | - Hongyang Gao
- Department of Electron Microscope, School of Basic Medical Science, Shanghai Medical College, Fudan University, Shanghai, China
| | - Zhonghua Zhao
- Department of Electron Microscope, School of Basic Medical Science, Shanghai Medical College, Fudan University, Shanghai, China
| | - Huijuan Wu
- Department of Pathology, School of Basic Medical Science, Shanghai Medical College, Fudan University, Shanghai, China
| | - Zhigang Zhang
- Department of Pathology, School of Basic Medical Science, Shanghai Medical College, Fudan University, Shanghai, China
| |
Collapse
|
|
8
|
Malvar A, Davila P, Ferrari M, Delgado P, Iscoff P, Lococo B, Alberton V. Podocyte infolding glomerulopathy; report of the first case in Latin America and review of the literature. Nefrologia 2020; 40:469-473. [PMID: 31952852 DOI: 10.1016/j.nefro.2019.09.010] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2019] [Accepted: 09/07/2019] [Indexed: 10/25/2022] Open
Abstract
BACKGROUND Podocyte infolding glomerulopathy (PIG) is a condition of uncertain origin, frequently associated with autoimmune diseases. Its specific treatment and clinical course are unknown. It is characterised by thickening of the capillary walls due to the presence of non-argyrophilic intramembranous bubbles similar to those found in membranous glomerulopathy, but without electron-dense deposits of immune complexes in the ultrastructure, where translucent microspheres generated by invagination of the podocyte cytoplasm into the basement membranes are observed. OBJECTIVES Generally reported in young females patients. To date, few cases in Asian patients have been reported. Our case is the first to be reported in a Latin American Caucasian patient. METHODS A 38-year-old woman with SLE. In 2014 she presented with nephrotic syndrome empirically treated with corticosteroids (CO) and intravenous cyclophosphamide with good response. She had a relapse in April 2015 with normal renal function and no extrarenal lupus activity, so she was referred to our hospital to be biopsied. RESULTS The biopsy reported focal segmental glomerular sclerosis without deposits of immune complexes in the immunofluorescence. However, methenamine silver staining revealed clear spaces in the capillary walls accompanied by marked podocyte alterations. On electron microscope study, numerous aggregates of microvesicular and cylindrical ultrastructures bound to the membranes were observed, without evidence of dense deposits, and diffuse effacement of pedicel foot processes, confirming the suspected diagnosis. CONCLUSIONS This is the first reported case of what can be considered a new pathological glomerular entity in a Latin American Caucasian patient, whose clinical course and therapy are still unknown.
Collapse
Affiliation(s)
- Ana Malvar
- Unidad de Nefrología, Hospital Juan A. Fernández, Buenos Aires, Argentina.
| | - Pedro Davila
- Unidad de Nefrología, Hospital Juan A. Fernández, Buenos Aires, Argentina
| | - Matías Ferrari
- Unidad de Nefrología, Hospital Juan A. Fernández, Buenos Aires, Argentina
| | - Pamela Delgado
- Unidad de Nefrología, Hospital Juan A. Fernández, Buenos Aires, Argentina
| | - Paula Iscoff
- Unidad de Nefrología, Hospital Juan A. Fernández, Buenos Aires, Argentina
| | - Bruno Lococo
- Unidad de Nefrología, Hospital Juan A. Fernández, Buenos Aires, Argentina
| | - Valeria Alberton
- División de Patología, Hospital Juan A. Fernández, Buenos Aires, Argentina
| |
Collapse
|
|
9
|
Wöstmann F, Müller RU, Göbel H, Benzing T, Becker JU, Bartram MP. Case report: a peculiar glomerulopathy in a patient suffering from nephrotic syndrome. BMC Nephrol 2019; 20:326. [PMID: 31438874 PMCID: PMC6704495 DOI: 10.1186/s12882-019-1478-8] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2019] [Accepted: 07/19/2019] [Indexed: 11/29/2022] Open
Abstract
Background Podocyte infolding glomerulopathy (PIG) is a rare histopathologic finding with global infolding of the podocytes into the glomerular basement membrane (GBM), accompanied by microstructures underneath. Described in 2002 for the first time, PIG was proposed as a new pathological entity in 2008 based on the largest case series so far. Yet all of the described cases derive from Asian countries. We report a case from Germany fulfilling the diagnostic criteria of PIG. Considering the scarcity of data on this entity especially in Western countries, collecting cases like ours and multicentric meta-analyses will be crucial to obtain a better understanding of PIG, its causes, clinical course and potential treatment options. Case presentation A 56-year-old Caucasian woman with a history of rheumatoid arthritis (RA), no other comorbidities and no known renal disease was admitted to the hospital with acute kidney injury (AKI) and nephrotic syndrome. Physical examination was unremarkable except for anasarca. Renal ultrasound revealed no abnormalities. Laboratory and urine analyses were consistent with the nephrotic syndrome and renal failure. Serological studies regarding ANA, ANCA, anti-PLA2R autoantibodies, complement, virus infections, immunofixation and quantitative light chain analysis were unremarkable. A renal biopsy was performed. Light microscopic examination showed flattened tubular epithelium consistent with acute tubular damage, no infiltrates and unremarkable glomeruli except diffuse and global holes in the GBM (Fig. 1a) and negative staining for immunoglobulin heavy-chains, light-chains and complement split products. Electron microscopy revealed a rare correlate for these holes: global peculiar infolding of podocyte cytoplasm into the GBM. Most of these infoldings were accompanied by condensation of the GBM underneath. No such condensation or electron dense deposits were found without these infoldings or outside the GBM. Conclusion Here we report the first case of PIG outside of Asia. Since there are only few reports about this specific finding, we feel there is a need to share information in an attempt to accumulate knowledge about this possible new entity and potential treatment options.
Collapse
Affiliation(s)
- Fabian Wöstmann
- Department II of Internal Medicine and Center for Molecular Medicine Cologne, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany
| | - Roman-Ulrich Müller
- Department II of Internal Medicine and Center for Molecular Medicine Cologne, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany.,CECAD, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany
| | - Heike Göbel
- Institute of Pathology, University Hospital of Cologne, Cologne, Germany
| | - Thomas Benzing
- Department II of Internal Medicine and Center for Molecular Medicine Cologne, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany.,CECAD, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany
| | - Jan U Becker
- Institute of Pathology, University Hospital of Cologne, Cologne, Germany.
| | - Malte P Bartram
- Department II of Internal Medicine and Center for Molecular Medicine Cologne, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany.
| |
Collapse
|
|
10
|
Podocytic infolding glomerulopathy: two new cases with connective tissue disease and literature review. Clin Rheumatol 2019; 38:1521-1528. [PMID: 30879204 DOI: 10.1007/s10067-019-04504-6] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2018] [Revised: 02/17/2019] [Accepted: 03/05/2019] [Indexed: 10/27/2022]
Abstract
Podocytic infolding glomerulopathy (PIG) is a newly proposed disease entity, and only 29 cases have been reported worldwide so far, characterized by microspheres or microtubular structures or both associated with podocytic infolding into the glomerular basement membrane (GBM) on electron microscopy. We present two new cases of PIG with connective tissue disease (CTD), one with primary Sjögren's syndrome and the other with systemic lupus erythematosus (SLE), and make a systemic review of the literature. In the entire 31 patients of PIG, 24 (77.42%) were women and seven (22.58%) were men, with an average age of 41.2 ± 15.2 (ranging from 14 to 79) years old. Almost two-thirds of patients (67.74%) were diagnosed with CTD, in which 76.19% were SLE. All patients presented with proteinuria and six (19.35%) patients were accompanied with hematuria. Serum creatinine was elevated in six (19.35%) patients. Pathological findings of all patients were consistent with PIG characteristics, and four patients with repeated renal biopsies further provided profound insights.
Collapse
|
|
11
|
Matthai SM, Mohapatra A, Mathew AJ, Roy S, Varughese S, Danda D, Tamilarasi V. Podocyte Infolding Glomerulopathy (PIG) in a Patient With Undifferentiated Connective Tissue Disease: A Case Report. Am J Kidney Dis 2018; 72:149-153. [DOI: 10.1053/j.ajkd.2017.11.023] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/08/2017] [Accepted: 11/29/2017] [Indexed: 11/11/2022]
|
|
12
|
Ciocchini M, Arbelbide J, Musso CG. Monoclonal gammopathy of renal significance (MGRS): the characteristics and significance of a new meta-entity. Int Urol Nephrol 2017; 49:2171-2175. [DOI: 10.1007/s11255-017-1594-y] [Citation(s) in RCA: 24] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/04/2016] [Accepted: 04/11/2017] [Indexed: 11/29/2022]
|
|
13
|
|