Key result
Immunosuppression improves right heart failure in microscopic polyangiitis presenting as acute pericarditis and borderline tamponade.
Why the study?
Microscopic polyangiitis is an ANCA-associated vasculitis affecting multiple organs, but cardiac involvement is rare.
Case Report (n=1)
No
This case highlights that acute pericarditis with pericardial compressive syndrome can be a rare initial manifestation of microscopic polyangiitis, necessitating consideration of ANCA-associated vasculitis in the differential diagnosis of pericarditis.
May prompt ANCA testing in unexplained pericarditis with renal involvement; leaves open the prevalence and optimal management of cardiac MPA.
Microscopic polyangiitis (MPA) is an ANCA-associated vasculitis that affects multiple organs. However, cardiac involvement is rare in these patients. We herein report a rare case of MPA presenting with marked peripheral edema and weight gain due to acute pericarditis with borderline tamponade physiology and possible effusive–constrictive features, resulting in right-sided heart failure. Myeloperoxidase-ANCA was strongly positive, and the patient subsequently developed rapidly progressive renal dysfunction. A renal biopsy revealed pauci-immune crescentic glomerulonephritis, confirming the diagnosis of MPA. This case highlights pericarditis with pericardial compressive syndrome as an atypical initial manifestation of MPA.
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Hirata et al. (2026) conducted a case report in Microscopic polyangiitis (n=1). Immunosuppressive therapy (corticosteroids and cyclophosphamide) was evaluated. Microscopic polyangiitis can atypically present with acute pericarditis and borderline tamponade physiology leading to right-sided heart failure, which improved following immunosuppressive therapy.
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