Pleural and pericardial involvement are well recognized in eosinophilic granulomatosis with polyangiitis (EGPA) but considered rare manifestations of the other ANCA-associated vasculitides (AAV).What are the frequency and clinical characteristics of pleuritis and pericarditis in AAV?Using an institutional database of 1,830 AAV patients, clinical notes and diagnosis codes were analyzed for key words related to pleuritis and pericarditis. Chart review to confirm these findings was performed.Eighty-eight of 1058 (8.3%) patients with GPA, 27 of 267 (10.1%) with MPA, and 35 of 201 (17.4%) with EGPA had a manifestation of pleuritis and/or pericarditis attributable to vasculitis. There was a higher frequency of pericarditis in EGPA compared to the other AAV (p<0.0001). There was no difference in the frequency of pleuritis in GPA, MPA, or EGPA. In the 156 AAV patients with pleuritis and/or pericarditis, this was a presenting feature in 127 (81.4%). Overall, it was a presenting feature in 6.9% of all AAV patients including 6.5% GPA, 8.6% MPA, and 15.9% EGPA.Pleuritis and pericarditis occur across all the AAV and, when present, are commonly presenting features of these diseases. Patients with EGPA have a higher proportion of pericardial involvement compared to pleural involvement, whereas this distribution is more equal in GPA/MPA. Pleuritis and pericarditis represent under-recognized features of AAV. All forms of AAV should be considered in the differential diagnosis when evaluating a patient with pleuritis or pericarditis.
Gwen E Thompson, Michael Bourne, Marta Casal Moura, Misbah Baqir, Rodrigo Cartin-Ceba, Ashima Makol, Matthew J Koster, Kenneth J Warrington, Bijan J Borah, Ulrich Specks