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Aneurysmal rupture in microscopic polyangiitis: a case-based review.

Keita Imanishi1,2, Kazuhiro Yasuo3, Yusuke Shirai4

  • 1Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Bunkyo-Ku, Tokyo, Japan. k.imanishi.jl@juntendo.ac.jp.

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|January 21, 2025
PubMed
Summary

Microscopic polyangiitis (MPA) can cause arterial aneurysms, with ruptured aneurysms leading to significant mortality. Early immunosuppressive treatment may prevent these dangerous ruptures.

Keywords:
AneurysmMedium vessel vasculitisMicroscopic polyangiitisRupture

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Area of Science:

  • Nephrology
  • Rheumatology
  • Vascular Surgery

Background:

  • Microscopic polyangiitis (MPA) is a small and medium vessel vasculitis that can lead to arterial aneurysms.
  • Ruptured aneurysms in MPA are rare, with only 15 reported cases in English literature prior to this study.
  • This study investigates a fatal case of MPA with multiple visceral aneurysms and reviews existing literature to understand aneurysmal rupture in MPA.

Purpose of the Study:

  • To analyze the clinical features, rupture characteristics, and outcomes of arterial aneurysms in patients with microscopic polyangiitis.
  • To identify risk factors and optimal timing for intervention in MPA-associated aneurysms.

Main Methods:

  • A comprehensive review of 16 cases of ruptured aneurysms in MPA, including one fatal case experienced by the authors.
  • Analysis of organ involvement, aneurysm locations, time to rupture, presenting symptoms, pathological findings, and causes of death.
  • Correlation of clinical presentation with pathological findings and treatment outcomes.

Main Results:

  • Common organ involvement included glomerulonephritis (100%), pulmonary (25%), and peripheral neuropathy (25%).
  • Ruptured aneurysms were most frequently found in the left gastric artery (31.25%), renal and hepatic arteries (18.75% each).
  • Rupture occurred median 45 days post-symptom onset and 15 days post-immunosuppressive treatment; mortality rate was 50%.

Conclusions:

  • MPA-associated ruptured aneurysms often present initially as renal-limited vasculitis, with abdominal medium-sized arteries being prone to rupture.
  • Aneurysms typically develop asymptomatically and rupture within weeks of symptom onset or treatment initiation.
  • Early immunosuppressive treatment is crucial for potentially shrinking aneurysms and preventing rupture in MPA patients.