Related Experiment Video
Updated: May 4, 2026
Determining Immune System Suppression versus CNS Protection for Pharmacological Interventions in Autoimmune Demyelination
Published on: September 12, 2016
Comparison of phenotype and outcome in microscopic polyangiitis between Europe and Japan
Shunsuke Furuta1, Afzal N Chaudhry, Yoshitomo Hamano
1From the Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology, Tokyo Metropolitan Geriatric Hospital, Tokyo; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki; Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Science, Okayama; Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya; Division of Nephrology and Dialysis, Department of Medicine, Kitano Hospital, the Tazuke Kofukai Medical Research Institute, Osaka; Division of Nephrology, Department of Medicine, Jichi Medical University, Tochigi; Department of Allergy and Clinical Immunology, Chiba University Hospital; Department of Allergy and Clinical Immunology, Asahi General Hospital, Chiba, Japan; Department of Nephrology and Transplantation, Skane University Hospital, Malmö, Sweden.
Objective:
There are differences between Europe and Japan in the incidence and antineutrophil cytoplasmic antibody (ANCA) serotype of patients with microscopic polyangiitis (MPA). However, differences in phenotype or outcome have not been explored. We aimed to identify differences in phenotype and outcome of MPA between Europe and Japan.
Methods:
Sequential cohorts of patients with MPA and renal limited vasculitis were collected from European and Japanese centers (n = 147 and n = 312, respectively). Trial databases from the European Vasculitis Society and the Japanese patients with Myeloperoxidase (MPO)-ANCA-Associated Vasculitis (JMAAV) trial were studied (n = 254 and n = 48, respectively). We evaluated baseline characteristics including ANCA status and organ involvement, treatment, survival, and renal survival. Differences in survival and renal survival were studied using multivariate analysis.
Results:
The non-trial cohorts showed patients with MPA in Japan had a higher age at onset, more frequent MPO-ANCA positivity, lower serum creatinine, and more frequent interstitial pneumonitis than those in Europe (all p < 0.01). Comparisons between the trial databases demonstrated similar results. Cumulative patient survival and renal survival rates were not different between Europe and Japan (p = 0.71 and p = 0.38, respectively). Multivariate analysis identified age at onset, serum creatinine, gastrointestinal, and respiratory involvement as factors with higher risk of death. For endstage renal failure, serum creatinine and use of plasma exchange were identified as factors with higher risk, and immunosuppressant use as lower risk factors.
Conclusion:
Phenotypes in patients with MPA were different between Europe and Japan. However, the outcomes of patient survival and renal survival were similar.
More Related Videos
09:16Supervised Machine Learning for Semi-Quantification of Extracellular DNA in Glomerulonephritis
Published on: June 18, 2020
10:05Simultaneous Distinction of Monospecific and Mixed DFS70 Patterns During ANA Screening with a Novel HEp-2 ELITE/DFS70 Knockout Substrate
Published on: January 17, 2018
