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A case of aortic dissection in familial Mediterranean fever

Asmara Malik1, Jahanzeb Malik2, Muhammad Javaid2

  • 1Public Health, National University of Medical Sciences, Rawalpindi, Pakistan.

Journal of Cardiology Cases
|December 17, 2021
PubMed

Insights

Familial Mediterranean fever (FMF), an autoinflammatory disease, can rarely cause Stanford type A aortic dissection. This case highlights an unusual presentation of FMF in a young Iranian man.

Area of Science:

  • Rheumatology and Genetics
  • Cardiovascular Medicine

Background:

  • Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder common in Arab populations.
  • FMF typically presents with recurrent fever and serositis, but can also manifest as amyloidosis, vasculitis, or pericarditis.
  • The Mediterranean fever (MEFV) gene mutations are causative for FMF.

Observation:

  • A 23-year-old man of Iranian descent presented with periodic fever.
  • He was diagnosed with Stanford type A aortic dissection during an acute FMF attack.

Findings:

  • This case represents the first reported instance of Stanford type A aortic dissection as a complication of FMF.
  • While FMF commonly affects small- and medium-sized arteries, this presentation involves the aorta, an unusual manifestation.

Implications:

  • Physicians should consider FMF in the differential diagnosis of aortic involvement, especially in susceptible ethnic groups.
  • Increased awareness of FMF prevalence, particularly in regions like Pakistan due to migration, is crucial for timely diagnosis and management.
  • This case underscores the importance of recognizing atypical presentations of FMF to prevent severe complications.
Keywords:
Aortic dissectionFamilial mediterranean feverGenetic markers

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