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Peer-reviewedCC BY-NC-NDInternal Medicine

Atypical Presentation of Microscopic Polyangiitis with Pericarditis Manifesting as Right-Sided Heart Failure

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.

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BibTeX
@article{hirata2026atypical,
  title = {{Atypical Presentation of Microscopic Polyangiitis with Pericarditis Manifesting as Right-Sided Heart Failure}},
  author = {Kazuhito Hirata and Shuhei Yamasaki and Junya Izena and Tomohiro Arakaki and Kageyuki Ohba and Osamu Arasaki and Masanori Kakazu},
  journal = {Internal Medicine},
  year = {2026},
  month = jul,
  doi = {10.2169/internalmedicine.7613-26},
  url = {https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7613-26/_article},
  note = {CC BY-NC-ND},
}
RIS
TY  - JOUR
TI  - Atypical Presentation of Microscopic Polyangiitis with Pericarditis Manifesting as Right-Sided Heart Failure
AU  - Kazuhito Hirata
AU  - Shuhei Yamasaki
AU  - Junya Izena
AU  - Tomohiro Arakaki
AU  - Kageyuki Ohba
AU  - Osamu Arasaki
AU  - Masanori Kakazu
PY  - 2026
DA  - 2026/07/11
JO  - Internal Medicine
DO  - 10.2169/internalmedicine.7613-26
UR  - https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7613-26/_article
AB  - 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.
SN  - 1349-7235
SN  - 0918-2918
ER  -