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Polyarteritis nodosa in case of familial Mediterranean fever

İbrahim Gökçe1, Ülger Altuntaş1, Deniz Filinte2

  • 1Division of Pediatric Nephrology, Department of Pediatrics, Marmara University Faculty of Medicine, İstanbul, Turkey.

The Turkish Journal of Pediatrics
|December 5, 2018
PubMed

Insights

Familial Mediterranean fever (FMF) can manifest as protacted febrile myalgia syndrome. This case study highlights a rare association between FMF and polyarteritis nodosa (PAN) in a pediatric patient.

Area of Science:

  • Pediatric Rheumatology
  • Genetics and Autoimmune Diseases

Background:

  • Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder.
  • FMF presents with recurrent fever, serositis, and arthritis.
  • Protracted febrile myalgia syndrome (PFMS) is a rare, severe manifestation of FMF.

Observation:

  • Polyarteritis nodosa (PAN) is a systemic vasculitis affecting medium-sized arteries.
  • PAN is uncommon in children but more frequent in FMF patients.
  • A 14-year-old child with FMF was diagnosed with associated PAN.

Findings:

  • The case illustrates a rare co-occurrence of FMF and PAN in a pediatric patient.
  • This association underscores the complex vasculitic manifestations possible in FMF.
  • Early recognition of PAN is crucial in children with FMF.

Implications:

  • Physicians must maintain a high index of suspicion for PAN in pediatric FMF patients.
  • Awareness of this association can lead to timely diagnosis and management.
  • Understanding FMF-associated vasculitis may improve patient outcomes.
Keywords:
familial Mediterranean feverpolyarteritis nodosaprotracted febrile myalgia syndrome

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