Abstract
BACKGROUND & OBJECTIVES: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of rare chronic autoimmune diseases characterized by recurrent systemic inflammation provoking multiple morbidities. AAV patients suffer from various organ manifestations and treatment-related severe adverse effects. This retrospective study investigated the concrete burden of AAV disease on patients in Germany. METHODS: Based on anonymized longitudinal German statutory health insurance (SHI) claims data from the years 2011-2016, a representative cohort of approximately 3 million insured persons was used to identify patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), and selected clinical aspects were systematically assessed. RESULTS: The most frequent concomitant morbidities of GPA and MPA were renal and respiratory disorders. Severe renal involvement occurred in 11.6% of GPA and 24.3% of MPA patients within 15 quarters of diagnosis. Severe infections developed in one third of AAV patients within the first three quarters post-diagnosis. The annual rate of major relapses was 5-8%. AAV patients with renal impairment or infections showed increased annual mortality rates of 14.4 and 5.6%, respectively. CONCLUSION: Based on this analysis of German health care data, disease-specific assumptions regarding the burden on AAV patients were confirmed and concretized for the German context. AAV patients suffer from a high burden of morbidity, including multiple disease manifestations, relapses, and severe complications due to AAV treatment.