Clinical characteristics of patients with vasculitis positive for anti-neutrophil cytoplasmic antibody targeting both pr
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Rheumatology International https://doi.org/10.1007/s00296-019-04447-w
INTERNATIONAL
OBSERVATIONAL RESEARCH
Clinical characteristics of patients with vasculitis positive for anti‑neutrophil cytoplasmic antibody targeting both proteinase 3 and myeloperoxidase: a retrospective study Sun Moon Kim1 · Song‑Yi Choi2 · Seon Young Kim3 · Jinhyun Kim4 Received: 13 June 2019 / Accepted: 16 September 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019
Abstract Anti-neutrophil cytoplasmic antibody (ANCA) may target proteinase 3 (PR3) or myeloperoxidase (MPO). Although a few patients with vasculitis have both MPO- and PR3-ANCA, the details of their clinical characteristics are not known. The objective of this study was to analyze the characteristics of patients with dual MPO- and PR3-ANCA-positive vasculitis. The medical records of patients with ANCA and vasculitis confirmed by biopsy were reviewed. The age at diagnosis, sex, and data on organ involvement of the kidney, lung, upper airways, skin, nervous system, and gastrointestinal tract were collected. Clinical variables were analyzed according to ANCA specificity. Of 85 patients with ANCA and vasculitis included in this study, 67 (78.8%) had MPO-ANCA, 10 (11.8%) had PR3-ANCA, and 8 (9.4%) had both MPO- and PR3-ANCA. Patients with MPO− PR3 + ANCA-associated vasculitis (AAV) were younger at diagnosis (median, 54.4 years; p
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