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Published on: March 17, 2022
Atypical Presentation of Microscopic Polyangiitis with Pericarditis Manifesting as Right-Sided Heart Failure
Kazuhito Hirata1, Shuhei Yamasaki1, Junya Izena2
1Division of Cardiology, Yuuai Medical Center, Japan.
Abstract:
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.
Insights
Microscopic polyangiitis (MPA), a rare vasculitis, can manifest unusually with acute pericarditis causing heart failure. This case highlights cardiac symptoms as an atypical initial presentation of MPA.
Area of Science:
- Nephrology
- Cardiology
- Rheumatology
Background:
- Microscopic polyangiitis (MPA) is a systemic vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA).
- Cardiac involvement is an uncommon complication of MPA.
- MPA typically affects the kidneys and lungs.
Purpose of the Study:
- To report a rare case of MPA presenting with cardiac complications.
- To highlight atypical initial manifestations of MPA.
Main Methods:
- Case report of a patient with MPA.
- Clinical presentation analysis including peripheral edema, weight gain, and echocardiography findings.
- Laboratory tests including myeloperoxidase-ANCA (MPO-ANCA) assessment.
- Renal biopsy for histopathological examination.
Main Results:
- The patient presented with acute pericarditis, borderline cardiac tamponade, and right-sided heart failure.
- Strongly positive MPO-ANCA confirmed MPA.
- Renal biopsy showed pauci-immune crescentic glomerulonephritis, consistent with MPA.
- Pericarditis with compressive syndrome was the initial manifestation.
Conclusions:
- MPA can present atypically with pericarditis and cardiac compromise.
- Early recognition of cardiac involvement is crucial in MPA diagnosis.
- This case expands the spectrum of MPA initial presentations.
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