[The clinical analysis of 46 cases with antineutrophil cytoplasmic antibody-associated vasculitis].

OBJECTIVE: To investigate the clinical features of patients with antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and to explore the disease activity of AAV patients and the relationship with prognosis.

METHODS: The clinical data of 46 cases of AAV patients in the First Affiliated Hospital of PLA General Hospital were analyzed retrospectively.The clinical and laboratory features of each clinical subtype were compared.The disease activity of AAV and the relationship between disease activity and prognosis were evaluated.

RESULTS: Among the 46 patients with AAV, 24 were male, and 22 were female, with the average age of 56±18.Among the subtypes of AAV, the number of granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) were 22, 17 and 4 respectively, while the positive rate of ANCA are 72.7%, 88.2% and 50.0% respectively.For GPA, the results of ANCA were mainly C-ANCA or ANCA directed toward proteinase-3 (PR3), and for MPA, the results of ANCA were mainly P-ANCA or ANCA directed toward myeloperoxidase (MPO). Upper and lower respiratory disease, renal involvement and non-deformity arthropathy were the common clinical manifestations of all primary AAV subtypes.Epistaxis, nasal blood scab, saddle nose, pulmonary nodule and intrapulmonary cavities were the characteristic manifestations of GPA, while rapid progress of renal failure was prominent in MPA.Whatever their ANCA results, there were no significant differences between each other as to system-organ involvements and laboratory indexes.Seven patients (15.2%) died during hospitalization or in follow-up visits.Serious involvements of heart, lung, kidney, and complicated with infections were the main risk factors of death in AAV patients.

CONCLUSIONS: Upper and lower respiratory involvements and kidney diseases are the primary manifestations of AAV patients.ANCA results are irrelevant with disease activity and system-organ involvements.Serious involvements of heart, lung, kidney, and complicated with infections are the main risk factors of death in AAV patients.