ANCA Associated Vasculitis: Clinical course and outcome of 44 patients from a single center in Turkey

INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a group of disease characterized by necrotizing, granulomatous inflammation of small to medium sized blood vessels.ANCA associated vasculitis is classified as Granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis polyangiitis (EGPA).
METHODS: Forty-four patients who were followed up with the diagnosis of ANCA-associated vasculitis between 2006 and 2020 at the Rheumatology-Immunology Unit at Research Hospital of Çukurova University were included in our study. GPA, MPAN and EGPA patients were diagnosed according to the criteria published by the 1990 American College of Rheumatology. The data were analyzed retrospectively.
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. Fourty two patients were positive for ANCA ( 35 c-ANCA and 7 p-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 BVAS score of the patients was calculated as 19 (± 7,512).
DISCUSSION AND CONCLUSION: During follow-up, 21 patients’ disease relapsed, 2 patients quitted followed up and 3 patients died. The variety of clinical symptoms of this curable disease may result as delay for diagnose and treatment. The disease has a heterogeneous clinical presentation. Therefore, it is appropriate to make a patient-based decision for management.