No abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disease characterized by necrotizing, granulomatous inflammation of small to medium-sized blood vessels. The classification of vasculitis is performed according to the International Chapel Hill Consensus Conference, which was held in 2012; a system that classifies the disease according to the vessel size. According to this classification, ANCA-AAV is classified as granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis polyangiitis (EGPA). Although AAV is a rare disease with an incidence of about 20 per million population per year in Europe and North America. There is a slightly male preponderance. The aim of our study aimed to reveal the clinical features of ANCA-AAVs and to compare the effects of related organ involvement on prognosis Material and Methods:Material and Methods: Forty-four patients who were followed up with the diagnosis of ANCA-AAV between 2006 and 2020 at the rheumatology-immunology unit were included in our study. The data were analyzed retrospectively. Results: Results:In this retrospective study, 44 patients were included who were followed up with the diagnosis of AAV. The patients had 38 (86%) GPA, 4 (9%) MPA, 2 (4.5%) EGPA diagnoses. Forty-two patients were positive for ANCA (35 cytoplasmic-ANCA and 7 perinuclear-ANCA). ANCA test of two patients were negative. Ten of the patients with GPA had limited and 28 of them had severe disease. Forty-two patients were followed up for an average of 36 (3-168 months) months. The initial mean Birmingham vasculitis activity score of the patients was calculated as 19 (±7.512). The number of patients in clinical remission was 31 (71%), and the mean time to remission was 6 months. During the follow-up, 21 patients' disease relapsed, 2 patients quit followed up, and 3 patients died. Conclusion: Conclusion:The variety of clinical symptoms of this curable disease may result as a delay for diagnose and treatment. The disease had a heterogeneous clinical presentation. Therefore, it is appropriate to make a patient-based decisions for management. In this study, we demonstrated the clinical diversity and the efficacy of cyclophosphamide and rituximab during induction therapy.

No abstract