The Prevalence and Management of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in China

Background: Anti-neutrophil cytoplasmic antibody (ANCA)associated vasculitis (AAV) comprises microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Myeloperoxidase (MPO) and proteinase 3 (PR3) are the main antigens for ANCA. AAV is a common multisystem autoimmune disease and most of the studies on AAV have been conducted in Western countries. Nowadays in China many efforts are made to investigate this disease. Summary: This review highlights the progress in the prevalence, management and outcomes of AAV in Chinese patients. With respect to the prevalence of AAV, though there are no precise data, AAV is not rare in the Chinese population. In Chinese patients with AAV there is a striking preponderance of MPA, and MPO-ANCA is much more common than PR3-ANCA. Even in patients with GPA there is a predominance of MPO-ANCA over PR3-ANCA. Propylthiouracilinduced AAV and ANCA-negative pauci-immune glomerulonephritis are stated in this review as well. With respect to the management of AAV, glucocorticoids in combination with cyclophosphamide remain the mainstay of induction theraReceived: September 23, 2015 Accepted: October 18, 2015 Published online: December 9, 2015 Min Chen, MD, PhD Renal Division, Peking University First Hospital 8 Xishiku Street, Xicheng District Beijing 100034 (China) E-Mail chenmin74 @ sina.com © 2015 S. Karger AG, Basel 2296–9381/15/0014–0216$39.50/0 www.karger.com/kdd Z.-Y. Li and T.-T. Ma contributed equally to this paper. For the prevalence and management of pauci-immune glomerulonephritis and vasculitis in Western countries, see Lionaki and Boletis, Kidney Dis 2015;1:224–234.