Clinician views concerning the prevalence and impact of granulomas on the diagnosis, management, and outcomes of ANCA-associated vasculitis

OBJECTIVES: It is unclear whether clinicians agree which manifestations of AAV are associated with necrotizing granulomas or if their presence affects clinical decision-making. METHODS: We surveyed physicians experienced in caring for individuals with AAV, querying: experience with AAV; beliefs concerning how granulomas affect the diagnosis, treatments, and outcomes of AAV; beliefs concerning the frequency with which granulomas are found in 36 manifestations of AAV; and degree to which granulomas change choice of induction therapy for specific manifestations of AAV. We analyzed responses using descriptive statistics and multivariable linear regression. RESULTS: We received 142 responses from 35 countries. Responses had a median Likert response ≥5 on a 7-point scale (equal to 'partially agree') that granulomatous manifestations respond differently to therapy, increase risk of relapse, and increase organ damage. Four of 36 manifestations were believed to be caused by granulomas in a median of ≥ 75% of cases (on a scale of 0 = never to 100 = always caused by granuloma), 19 in a median of ≤ 25% of cases, and 13 in intermediate medians. The perceived degree to which granulomas caused manifestations was not associated with changes in therapy to induce remission in severe AAV (p-values 0.26-0.93 across scenarios). CONCLUSIONS: Physicians experienced in vasculitis generally agree on which manifestations of AAV are and are not caused by granulomas and that granulomatous inflammation alters the natural history and treatment of AAV. However, the presence of granulomatous manifestations did not alter treatment choices to induce remission in severe AAV.