Work

093. Atypical Presentation of Anca-associated Vasculitis: a Case Series

Year: 2019

Type: article

Abstract: Background: ANCA associated vasculitis (AAV) can present in an atypical manner and obscure the clinical picture. We sought to characterize clinical characteristics and renal outcomes in these uncommon presentations. Methods: We conducted a retrospective study of 136 patients in our ANCA database to identify patients with atypical presentation of AAV and subsequently developed renal dysfunction. Patient demographics, serologies, renal indices and treatment regimens were assessed. Results: Of the 136 patients, 8 were identified to have atypical presentations. These patients were usually extremes of age with three being less than 30 years and four being more than 70 years. Six patients were positive for PR3 antibodies. The mean delay in diagnosis from time of symptom development was 9 months. Majority of the patients (n = 7) subsequently developed acute kidney injury, with two of them requiring temporary hemodialysis. Pancreatitis was the most frequent atypical presentation (n = 3), with pulmonary pathologies (cystic lung disease and usual interstitial pneumonia) and splenic infarcts being present in 2 patients each (Table 1). The diagnosis of AAV was established in these patients by positive ANCA serology and renal biopsy evidence of ANCA GN. Six patients had an induction regimen of steroids and rituximab, whilst the remainder received steroids and cyclophosphamide. One patient died of respiratory failure in the first month following diagnosis while the remaining patients achieved disease remission. Four patients did not recover renal function (including two with dialysis dependence). Conclusion: Atypical presentations of AAV afflict extremes of age with a PR3 predominant antibody and would be complicated by subsequent development of AKI. This case series demonstrates that a significant delay in diagnosis can be associated with these presentations. Disclosures: Duvuru Geetha- consultant to Chemocentry and Kyowa Hakko Kirin; Other authors-none

Source: Lara D. Veeken

DOI: 10.1093/rheumatology/kez058.033