The Clinical Characters and Outcome of Antineutrophil Cytoplasmic Antibody-associated Vasculitis: A Retrospective Single-Center Study

Abstract

Objective: To retrospectively review the classification, clinical course, treatment response and investigated prognostic factors. of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods: Patients with positive ANCA that received treatment at the National Taiwan University Hospital (NTUH) between January 1, 2007 and March 31, 2013 were enrolled. The range of involved organs was based on medical records, images and laboratory data. The Birmingham vasculitis activity score (BVAS) was evaluated for each patient. The treatments, clinical outcome and disease recurrences were recorded for statistical analyses. Results: Nineteen myeloperoxidase (MPO)-ANCA-positive patients and seven proteinase 3 (PR3)-ANCApositive patients had newly diagnosed AAV during the study period. Microscopic polyangiitis (MPA) was the most common classification (42% overall). Kidneys and lungs were the most frequently involved organs, with an incidence rate of 73% and 69%, respectively. Moreover, 53% of patients with MPO-AAV had chronic kidney disease before diagnosis. In addition, the all-cause mortality rate in this study was 26% and infection was the most common cause of death. Of the six expired patients, 5 died due to infectious complications. The higher BVAS, Japanese vasculitis activity score (JVAS) for patients with renal vasculitis, or involvement of both lung and kidney, has the higher all-cause mortality. Conclusions: AAV is not common, but does cause considerable morbidity and mortality. MPO-AAV was more common than PR3-AAV in this study, and the majority of the diagnoses were MPA. Both BVAS and JVAS predicted survival outcome. Early diagnosis combined with adequate treatment, and measures to prevent, reduce or monitor infection, may help with overall survival.