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Aortic aneurysm due to microscopic polyangiitis

Masaaki Ryomoto1, Masataka Mitsuno, Hiroyuki Nishi

  • 1Department of Cardiovascular Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan.

The Annals of Thoracic Surgery
|November 26, 2009
PubMed

Insights

Microscopic polyangiitis, a condition usually affecting small vessels, rarely causes aortic aneurysms. This case highlights a unique instance of ascending aortic aneurysm in a patient with this autoimmune vasculitis.

Area of Science:

  • Cardiovascular Medicine
  • Rheumatology
  • Immunology

Background:

  • Microscopic polyangiitis (MPA) is a rare systemic vasculitis typically affecting small-caliber vessels.
  • Aortic aneurysms are an uncommon manifestation of MPA, posing diagnostic and therapeutic challenges.

Observation:

  • A 36-year-old woman with a 10-year history of MPA, treated with oral hydrocortisone, presented with an ascending aortic aneurysm and severe aortic insufficiency.
  • The aneurysm occurred without dilatation of the aortic root (sinus of Valsalva).
  • High titers of serum perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were detected.

Findings:

  • Surgical replacement of the ascending aorta and aortic valve was performed.
  • Pathologic examination of the resected aorta revealed significant adventitial and intimal thickening.
  • The aortic media showed severe destruction and atrophy, indicative of inflammatory damage.

Implications:

  • This case represents the first reported surgical instance of an aortic aneurysm associated with microscopic polyangiitis.
  • It underscores the potential for MPA to affect large vessels, including the aorta.
  • Further research is warranted to understand the pathogenesis and optimal management of MPA-associated aortic aneurysms.

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