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An unusual association between familial mediterranean fever and IgM nephropathy

Harun Peru1, Ahmet Midhat Elmaci, Fatih Akin

  • 1Department of Pediatric Nephrology, Faculty of Meram Medicine, Selcuk University, Konya, Turkey. harunperu@gmail.com

Medical Principles and Practice : International Journal of the Kuwait University, Health Science Centre
|April 15, 2008
PubMed
Abstract

Insights

This case highlights the rare co-occurrence of Familial Mediterranean Fever (FMF) and IgM nephropathy in a child. Early colchicine treatment resolved proteinuria, suggesting glomerulopathy can be an FMF manifestation.

Area of Science:

  • Nephrology
  • Genetics
  • Rheumatology

Background:

  • Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder characterized by recurrent fever and serositis.
  • While FMF-associated amyloidosis is well-documented, non-amyloid glomerulopathies are less common.
  • This case explores an unusual presentation of FMF.

Observation:

  • A 9-year-old boy presented with recurrent abdominal pain and laboratory findings consistent with active FMF, including elevated inflammatory markers.
  • Genetic testing revealed a homozygous M694V mutation, confirming FMF.
  • Renal biopsy showed IgM nephropathy without amyloid deposition.

Findings:

  • The patient exhibited significant proteinuria and mesangial proliferation on biopsy, indicative of IgM nephropathy.
  • Immunofluorescence revealed IgM and C1q deposits in the glomeruli.
  • Treatment with colchicine led to complete resolution of proteinuria and symptoms.

Implications:

  • This case suggests that IgM nephropathy can be a non-amyloid manifestation of FMF.
  • It underscores the importance of considering glomerulopathies in FMF patients presenting with renal abnormalities.
  • Early diagnosis and treatment of FMF-related glomerulopathy with colchicine can prevent disease progression.

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