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Clinicopathological Assessment of Kidney Biopsies in Children with Familial Mediterranean Fever: A Single-Center

Fatma Yazılıtaş1, Evrim Kargın Çakıcı2, Eda Didem Kurt Şükür2

  • 1Department of Pediatric Nephrology, Ankara Dr. Sami Ulus Maternity and Children Hospital, Ankara, Turkey, fmeryemesra@yahoo.com.

Nephron
|March 16, 2020
PubMed
Abstract

Insights

Familial Mediterranean Fever (FMF) rarely causes kidney disease in children. This study found amyloid nephropathy in FMF patients had worse outcomes, including higher proteinuria and lower kidney function.

Area of Science:

  • Nephrology
  • Pediatrics
  • Genetics

Background:

  • Familial Mediterranean Fever (FMF) is a rare monogenic autoinflammatory disease.
  • Glomerulopathy is an uncommon complication of FMF, particularly in pediatric populations.
  • Understanding the characteristics of FMF-associated glomerulopathy is crucial for early diagnosis and management.

Purpose of the Study:

  • To investigate the clinical, demographic, and genetic features of glomerulopathy in pediatric FMF patients.
  • To identify the types of glomerular lesions present in these patients.
  • To compare outcomes between amyloid nephropathy and non-amyloid nephropathy in pediatric FMF.

Main Methods:

  • Retrospective review of 30 pediatric FMF patients with biopsy-proven glomerulopathy.
  • Patients classified into amyloid nephropathy (AN) and non-amyloid nephropathy (N-AN) groups.
  • Analysis of clinical data, laboratory results, and genetic mutations (p.M694V).

Main Results:

  • Amyloid nephropathy (AN) group showed higher rates of hypertension, proteinuria, and serum creatinine, with lower eGFR at biopsy.
  • End-stage renal disease (ESRD) was more frequent in the AN group (p = 0.011).
  • Homozygous p.M694V mutations were associated with a higher frequency of amyloidosis (p = 0.039).

Conclusions:

  • Nephropathy is a rare but serious complication in pediatric FMF.
  • Pediatric FMF patients with AN exhibit poorer renal function and higher blood pressure compared to N-AN.
  • This study highlights the importance of kidney biopsy in characterizing glomerulopathy in pediatric FMF.
Keywords:
AmyloidosisFamilial Mediterranean feverGlomerulonephritisNon-amyloid nephropathy

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