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Polyarteritis nodosa in a case of familial Mediterranean fever

Sevcan A Bakkaloğlu1, Sule Muzaç, Sergin Akpek

  • 1Department of Pediatric Nephrology, Faculty of Medicine, Gazi University, Besevler, Ankara, Turkey. sevcan@gazi.edu.tr

Pediatric Nephrology (Berlin, Germany)
|February 14, 2004
PubMed

Insights

Familial Mediterranean fever (FMF) rarely complicates with polyarteritis nodosa (PAN). This case highlights distinct angiographic findings in a child with FMF and PAN, showing a successful response to treatment.

Area of Science:

  • Rheumatology
  • Pediatrics
  • Vascular Medicine

Background:

  • Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
  • Polyarteritis nodosa (PAN) is a systemic vasculitis affecting medium-sized arteries.

Observation:

  • A 7-year-old boy with FMF presented with abdominal pain, arthralgia, and fibromyalgia.
  • He developed maculopapular eruptions, hypertension, gastrointestinal bleeding, and constitutional symptoms suggestive of vasculitis.
  • Renal angiography revealed a perfusion defect indicative of renal infarction due to vasculitis.

Findings:

  • The patient demonstrated a rare co-occurrence of FMF and PAN.
  • Angiography showed non-aneurysmal signs characteristic of PAN.
  • The patient responded favorably to pulse methylprednisolone and colchicine therapy.

Implications:

  • This case underscores the rare association between FMF and PAN.
  • Distinct angiographic findings in PAN, even in the absence of aneurysms, are crucial for diagnosis.
  • Combined therapy with corticosteroids and colchicine can be effective in managing this rare complication.

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