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Chen Z, Tian X, Qu J, Chen J, Yang Y, Li J. Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis. RHEUMATOLOGY AND IMMUNOLOGY RESEARCH 2023; 4:30-39. [PMID: 37138647 PMCID: PMC10150875 DOI: 10.2478/rir-2023-0005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/25/2023] [Accepted: 03/14/2023] [Indexed: 05/05/2023]
Abstract
Objectives Risk stratification and prognosis prediction are critical for appropriate management of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Herein, we aim to develop and internally validate a prediction model specifically for long-term survival of patients with AAV. Methods We thoroughly reviewed the medical charts of patients with AAV admitted to Peking Union Medical College Hospital from January 1999 to July 2019. The Least Absolute Shrinkage and Selection Operator method and the COX proportional hazard regression was used to develop the prediction model. The Harrell's concordance index (C-index), calibration curves and Brier scores were calculated to evaluate the model performance. The model was internally validated by bootstrap resampling methods. Results A total of 653 patients were included in the study, including 303 patients with microscopic polyangiitis, 245 patients with granulomatosis with polyangiitis and 105 patients with eosinophilic granulomatosis with polyangiitis, respectively. During a median follow-up of 33 months (interquartile range 15-60 months), 120 deaths occurred. Age at admission, chest and cardiovascular involvement, serum creatinine grade, hemoglobin levels at baseline and AAV sub-types were selected as predictive parameters in the final model. The optimism-corrected C-index and integrated Brier score of our prediction model were 0.728 and 0.109. The calibration plots showed fine agreement between observed and predicted probability of all-cause death. The decision curve analysis (DCA) showed that in a wide range of threshold probabilities, our prediction model had higher net benefits compared with the revised five factor score (rFFSand) and the birmingham vasculitis activity score (BVAS) system. Conclusion Our model performs well in predicting outcomes of AAV patients. Patients with moderate-to-high probability of death should be followed closely and personalized monitoring plan should be scheduled.
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Affiliation(s)
- Zhe Chen
- Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing100730, China
- Department of Rheumatology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing100730, China
| | - Xinping Tian
- Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing100730, China
| | - Jingge Qu
- Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing100730, China
- Department of Respiratory and Critical Care Medicine, Peking University Third Hospital, Research Center for Chronic Airway Diseases, Peking University Health Science Center, Beijing100191, China
| | - Jing Chen
- Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing100730, China
- Department of Rheumatology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan250021, Shandong Province, China
| | - Yunjiao Yang
- Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing100730, China
| | - Jing Li
- Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing100730, China
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Grygiel-Górniak B, Limphaibool N, Perkowska K, Puszczewicz M. Clinical manifestations of granulomatosis with polyangiitis: key considerations and major features. Postgrad Med 2018; 130:581-596. [PMID: 30071173 DOI: 10.1080/00325481.2018.1503920] [Citation(s) in RCA: 36] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
BACKGROUND Granulomatosis with polyangiitis (GPA) presents a wide spectrum of manifestations from the common respiratory symptoms to infrequent neurological and cardiac complications. The challenge in diagnosis and management makes the rapidly progressive disorder one of the most challenging dilemmas in clinical medicine. METHOD An extensive electronic literature search was done using multiple databases (e.g. PubMed) from January 2007 to August 2017 to gather information on the etiopathogenesis, clinical presentation, and current treatment management plan of GPA. The language used was English. Search items included 'Granulomatosis with Polyangiitis symptoms,' 'Wegener's Granulomatosis,' and 'Treatment of Granulomatosis with Polyangiitis' for the databases. Inclusion criteria consisted of published articles in the English language which reported on the clinical manifestations of GPA, as well as on the treatment of the disease. Exclusion criteria included articles that: (1) did not specifically report on GPA vasculitis but also focused on discussion of other vasculitis such as MPA or Churg-Strauss; (2) reported on treatment/symptoms of GPA prior to 2007. RESULT This review provides an overview of work undertaken in key areas of GPA research, including its complex multifactorial etiology, clinical manifestations, treatment strategies, and treatment-related adverse effects. CONCLUSION Major advances in the understanding and treatment of GPA over recent decades have contributed to the notable decline in morbidity and mortality of patients. The ultimate goal is an improved prognosis through outcome measures which assesses the disease control with minimal adverse effects of intensive immunosuppressive regimens, an integral part of the clinical approach to improve the quality of life of GPA patients.
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Affiliation(s)
- Bogna Grygiel-Górniak
- a Department of Rheumatology and Internal Medicine , Poznan University of Medical Sciences , Poznan , Poland
| | - Nattakarn Limphaibool
- a Department of Rheumatology and Internal Medicine , Poznan University of Medical Sciences , Poznan , Poland
| | - Katarzyna Perkowska
- a Department of Rheumatology and Internal Medicine , Poznan University of Medical Sciences , Poznan , Poland
| | - Mariusz Puszczewicz
- a Department of Rheumatology and Internal Medicine , Poznan University of Medical Sciences , Poznan , Poland
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Banerjee S, Grayson PC. Vasculitis Around the World: Epidemiologic Insights into Causality and a Need for Global Partnerships. J Rheumatol 2018; 44:136-139. [PMID: 28148752 DOI: 10.3899/jrheum.161468] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Affiliation(s)
- Shubhasree Banerjee
- US National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), Vasculitis Translational Research Program, Bethesda, Maryland, USA
| | - Peter C Grayson
- US National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), Vasculitis Translational Research Program, Bethesda, Maryland, USA.
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Sato N, Yokoi H, Imamaki H, Uchino E, Sakai K, Matsubara T, Tsukamoto T, Minamiguchi S, Yanagita M. Renal-limited vasculitis with elevated levels of multiple antibodies. CEN Case Rep 2017; 6:79-84. [PMID: 28509135 DOI: 10.1007/s13730-017-0248-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2016] [Accepted: 01/18/2017] [Indexed: 10/20/2022] Open
Abstract
Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with crescentic glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic glomerulonephritis who were dual positive for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA or ANCA and anti-glomerular basement membrane (GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with pauci-immune type crescentic glomerulonephritis positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody. Renal biopsy led to the definitive diagnosis of RLV with pauci-immune-type crescentic glomerulonephritis. Fluorescence immunostaining showed no linear deposition of IgG on GBM, indicating no involvement of anti-GBM associated diseases. Intensive therapy, including prednisolone, plasma exchange, and intravenous cyclophosphamide, was effective. We report the case of triple-positive patient with crescentic glomerulonephritis, who was successfully treated with glucocorticoid, plasma exchange, and cyclophosphamide, suggesting that treatment for RLV in the patient with serological triple antibodies positivity in the absence of linear IgG deposition could benefit from the combination therapy regimen for plasma exchange and primary induction of remission against microscopic polyangiitis.
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Affiliation(s)
- Noriaki Sato
- Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto, 606-8507, Japan
| | - Hideki Yokoi
- Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto, 606-8507, Japan.
| | - Hirotaka Imamaki
- Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto, 606-8507, Japan.,Department of Nephrology, Hirakata Kohsai Hospital, Federation of National Public Service Personnel Mutual Aid Associations, Hirakata, Japan
| | - Eiichiro Uchino
- Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto, 606-8507, Japan
| | - Kaoru Sakai
- Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto, 606-8507, Japan
| | - Takeshi Matsubara
- Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto, 606-8507, Japan
| | - Tatsuo Tsukamoto
- Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto, 606-8507, Japan.,Department of Nephrology and Dialysis, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan
| | | | - Motoko Yanagita
- Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto, 606-8507, Japan
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Chen D, Xie N, Lin Y, Yang Z, Liu W, Wu S, Chen J, Pan X, Yang S, Cai Y. Diagnostic value of antineutrophil cytoplasmic antibodies in children with bronchiolitis obliterans. J Thorac Dis 2016; 8:1306-1315. [PMID: 27293851 PMCID: PMC4886013 DOI: 10.21037/jtd.2016.05.04] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2016] [Accepted: 05/09/2016] [Indexed: 08/15/2023]
Abstract
BACKGROUND Diagnosis of childhood bronchiolitis obliterans (BO) is difficult owing to non-specific clinical presentations and limited investigational options. There is a lack in established serum biomarkers for BO. While the diagnostic value of antineutrophil cytoplasmic antibodies (ANCAs) has been discussed, little is known about this in BO. We aimed to investigate the serological profiles of ANCAs against myeloperoxidase (MPO-ANCA) and proteinase-3 (PR3-ANCA) in BO and acute pneumonia. METHODS In this study, 42 BO children (BO group) and 43 with mild acute pneumonia (pneumonia group) were included, based on rigorous diagnostic criteria and additional constraints for minimizing selection bias. Serum MPO-ANCA and PR3-ANCA levels were measured on the first (baseline) and the last day of hospitalization (on discharge) by enzyme linked immunosorbent assay. RESULTS Although the BO children had a longer hospital stay, the overall rate of positivity (≥180 AAU/mL) and median serum level of MPO-ANCA were higher in the BO group compared with the pneumonia group, either at baseline (69.1% vs. 9.3%, 292.00 vs. 104.75 AAU/mL, both P<0.001) or on discharge (61.9% vs. 9.3%, 310.50 vs. 95.42 AAU/mL). Similar was found for PR3-ANCA (38.1% vs. 4.7%, 106.66 vs. 54.56 AAU/mL at baseline; 35.7% vs. 2.3%, 97.98 vs. 57.23 AAU/mL on discharge, both P<0.001). There were a higher rate of dual-positivity and a lower rate of dual-negativity to both ANCAs in the BO group than those in the pneumonia group (all P<0.001). CONCLUSIONS Detection of MPO- and PR3-ANCA can help diagnosis of childhood BO.
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Togashi M, Komatsuda A, Nara M, Omokawa A, Okuyama S, Sawada K, Wakui H. Validation of the 2010 histopathological classification of ANCA-associated glomerulonephritis in a Japanese single-center cohort. Mod Rheumatol 2014; 24:300-3. [DOI: 10.3109/14397595.2013.854068] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
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Isa H, Lightman S, Pusey CD, Taylor SRJ. Ocular manifestations of Wegener’s granulomatosis. EXPERT REVIEW OF OPHTHALMOLOGY 2014. [DOI: 10.1586/eop.11.62] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
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Suzuki K. Remarks on the Asia Pacific Meeting of Vasculitis and ANCA Workshop 2012. Clin Exp Nephrol 2013; 17:599-602. [DOI: 10.1007/s10157-013-0805-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2013] [Accepted: 03/22/2013] [Indexed: 11/27/2022]
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Shirai T, Fujii H, Saito S, Ishii T, Yamaya H, Miyagi S, Sekiguchi S, Kawagishi N, Nose M, Harigae H. Polyarteritis nodosa clinically mimicking nonocclusive mesenteric ischemia. World J Gastroenterol 2013; 19:3693-3698. [PMID: 23801874 PMCID: PMC3691024 DOI: 10.3748/wjg.v19.i23.3693] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/09/2013] [Revised: 04/08/2013] [Accepted: 04/16/2013] [Indexed: 02/06/2023] Open
Abstract
Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive for myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA). Computed tomography and surgical findings were compatible with nonocclusive mesenteric ischemia (NOMI). He underwent small intestinal resection by emergency surgery and an intestinal fistula was made. Pathologically, necrotizing vasculitis with fibrinoid necrosis was present in medium to small-sized arteries, which was equivalent to Arkin’s classification II-IV. Most of the arteries had fibrous intimal thickening, which was considered to obstruct the arteries and thus cause segmental intestinal necrosis. A diagnosis of polyarteritis nodosa (PAN) was made, and intravenous cyclophosphamide pulse therapy was added to the therapeutic regimen. This patient was successfully treated with these multidisciplinary therapies and his stoma was finally closed. This is a very rare and indicative case of PAN weakly positive for MPO-ANCA and clinically mimicking NOMI, which occurred even after treatment with pulsed methylprednisolone.
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Kobayashi S, Fujimoto S. Epidemiology of vasculitides: differences between Japan, Europe and North America. Clin Exp Nephrol 2013; 17:611-614. [PMID: 23715862 PMCID: PMC3824199 DOI: 10.1007/s10157-013-0813-9] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/14/2012] [Accepted: 04/27/2013] [Indexed: 11/21/2022]
Abstract
The epidemiology of systemic vasculitides differs between Japan, Europe and North America. Takayasu’s arteritis occurs frequently in Japan, unlike giant cell arteritis. A collaborative international study comparing the epidemiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis between Japan and the United Kingdom (UK) demonstrated that microscopic polyangiitis and myeloperoxidase-ANCA were more common in Japan whereas granulomatosis with polyangiitis and pronase 3-ANCA were more common in the UK. These differences may be attributed to differences in latitude and genetic backgounds. These findings provide useful information on the aetiology and pathogenesis of primary systemic vasculitides in various geographical regions.
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Affiliation(s)
- Shigeto Kobayashi
- Rheumatology, Department of Internal Medicine, Juntendo University Koshigaya Hospital, 560 Fukuroyama, Koshigaya, Saitama, 343-0032, Japan.
| | - Shouichi Fujimoto
- Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan
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Togashi M, Komatsuda A, Nara M, Omokawa A, Okuyama S, Sawada K, Wakui H. Validation of the 2010 histopathological classification of ANCA-associated glomerulonephritis in a Japanese single-center cohort. Mod Rheumatol 2013. [PMID: 23579500 DOI: 10.1007/s10165-013-0877-0] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2012] [Accepted: 03/26/2013] [Indexed: 10/27/2022]
Abstract
OBJECTIVES: To validate the 2010 histopathological classification system of anti-neutrophil cytoplasmic antibodies (ANCA)-associated glomerulonephritis (GN) in a Japanese single-center cohort. METHODS: We retrospectively studied 54 patients (28 renally limited pauci-immune GN, 25 microscopic polyangiitis, and one Churg-Strauss syndrome). RESULTS: There were 17 patients with focal GN, eight patients with crescentic GN, 19 patients with mixed GN, and 10 patients with sclerotic GN. Detailed information regarding treatment was available in 39 patients. All these patients were treated with steroids with or without immunosuppressive agents. Hemodialysis was introduced in two patients with crescentic GN and three patients with sclerotic GN. During the follow-up period, 27 of 54 patients died. The major cause of death was pneumonia. Significant differences were observed in estimated glomerular filtration rate among patients with focal, crescentic, mixed, and sclerotic GN at entry and 1- and 5-year follow-up. Patients with focal GN had preserved renal function and favorable outcome. CONCLUSIONS: Our validation study suggests that the 2010 histopathological classification of ANCA-associated GN might aid in prognostication of patients at the time of diagnosis and in therapy selection.
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Affiliation(s)
- Masaru Togashi
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita City, Akita, 010-8543, Japan
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Current world literature. Curr Opin Ophthalmol 2011; 22:523-9. [PMID: 22005482 DOI: 10.1097/icu.0b013e32834cb7d7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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