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

Abstract

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. Propylthiouracil-induced 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 therapy. Besides, we describe predictors of different outcomes in Chinese patients, including mortality, relapse, treatment resistance and end-stage renal disease. Key Messages: AAV is not rare in the Chinese population. The disease spectrum and subtypes of ANCA are different between patients with AAV in China and Western countries. The treatment strategy for AAV in China is in consistency with that in Western countries. Predictors of different clinical outcomes are provided. Facts from East and West: Treatment options for AAV are shared between the East and West, with corticosteroid combined with cyclophosphamide being the standard regimen for inductive therapy and switching to azathioprine after remission. The major cause of death in treated patients is infection related to immunosuppressive therapy within the first year after diagnosis, and this rate might be higher in China than in Western countries. Western studies demonstrated the efficacy and safety of rituximab for induction of remission in cases with relatively mild disease and maintenance therapy, but this agent is rarely used in China.

Risk factors for infectious complications of ANCA-associated vasculitis: a cohort study

“…Immunosuppressive therapy is performed with consideration of the disease activity, which is comprehensively evaluated based on the BVAS score [ 20 ]. Nonetheless, even in patients with severe ANCA-associated vasculitis, secondary infection, rather than active AAV, is the leading cause of death [ 21 ]. There still remains no firm conclusion about the burden and characteristics of major infections in patients with AAV.…”

Pituitary Involvement in Granulomatosis with Polyangiitis: A Retrospective Analysis in a Single Chinese Hospital and a Literature Review

“…The use of IIF and ELISA in ANCA testing gives a 96% sensitivity and 98.5% specificity for AAV [47], with 80% of patients with GPA being seropositive for PR3-ANCA and 10% for MPO-ANCA in Western countries [48]. However, in Chinese patients with systemic GPA, 60%–70% of them were p-ANCA specificity of MPO [49, 50]. In the literature review, almost all patients testing for ANCA were PR3-positive except for three; however, our four patients were all positive for MPO-ANCA.…”

Risk HLA class II alleles and amino acid residues in myeloperoxidase–ANCA-associated vasculitis

“…The prevalence (83.1%) of MPO-ANCA is much higher in Chinese patients with AAV 7 and other Asian populations 8,9 compared with the more common finding of PR3-ANCA among white populations. 5,10,11 High prevalence of HLA-DPB1*04 has been revealed with close association to PR3-AAV. [12][13][14][15] However, few studies investigated the associations between human leukocyte antigen (HLA) alleles and MPO-AAV.…”