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A case of aortoduodenal fistula caused by IgG4-related periaortitis

Hideki Oka1,2, Shuji Sumitomo1, Hayato Shimizu1,2

  • 1Department of Rheumatology, Kobe City Medical Center General Hospital, Kobe, Hyogo, Japan.

Modern Rheumatology Case Reports
|June 6, 2022
PubMed

Insights

A rare complication of IgG4-related periaortitis, aortoduodenal fistula, can occur after endovascular aortic repair. Careful patient monitoring post-procedure is crucial for inflammatory abdominal aortic aneurysms.

Area of Science:

  • Vascular Surgery
  • Immunology
  • Gastroenterology

Background:

  • Endovascular aortic repair (EVAR) is a common treatment for abdominal aortic aneurysms (AAAs).
  • Inflammatory AAAs, including IgG4-related periaortitis, can present unique challenges post-EVAR.
  • Aortoduodenal fistula is a rare but life-threatening complication.

Observation:

  • An 86-year-old male presented with fatigue and melena one year after EVAR for a AAA.
  • Diagnostic imaging revealed gastrografin in the abdominal aorta, confirming an aortoduodenal fistula.
  • Blood cultures showed multiple bacterial species.

Findings:

  • Pathological examination of the aorta revealed extensive immunoglobulin G4 (IgG4)-positive plasma cell infiltration and adventitial thickening.
  • Serum IgG4 levels were elevated at 241 mg/dL.
  • The patient was diagnosed with IgG4-related periaortitis.

Implications:

  • Aortoduodenal fistula is a critical complication of IgG4-related periaortitis.
  • This case highlights the importance of vigilant long-term surveillance for patients undergoing EVAR, particularly those with inflammatory AAAs.
  • Early detection and management are vital for improving patient outcomes in such rare but fatal conditions.
Keywords:
IgG4-related diseaseIgG4-related periaortitisaortoduodenal fistulaendovascular aortic repairinflammatory abdominal aortic aneurysm

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