Renal involvement in familial Mediterranean fever in an Algerian population

Clin Nephrol. 2023 Apr;99(4):172-179. doi: 10.5414/CN110930.

Abstract

The objectives of this study were to investigate the clinical biological and histological renal involvement secondary to familial Mediterranean fever (FMF), the epidemiological data, genetics of our patients and their evolution under treatment. We prospectively studied 58 Algerian patients admitted in our nephrology department from January 2012 to January 2021. The diagnosis of nephropathy was suspected clinically and biologically and confirmed histologically. All our patients were tested for MEFV mutations. Results: 58 patients, 30 males and 28 females, mean age 31.68 ± 12.71; 3 (5.17%) chronic dialysis patients and 55 (94.82%) referred to the nephrology department for renal biopsy with renal symptomatology consisting of nephrotic syndrome in 50 (94. 73%), associated with renal failure 27 (47.36%), mainly primary in 23 (34.5%), secondary to seronegative lupus 13 (22.4%), Crohn's disease 9 (14.5%), sarcoidosis 3 (5.26%), and lymphoma 1 (1.7%); 29 (50%) were from consangineous marriages, the histological study found AA amyloidosis in 52 (89.6%); the genetic study confirmed the diagnosis of FMF in 58 (100%). The evolution of the patients: 20 (34.48%) followed in consultation, 25 (43.10%) in hemodialysis and 13 (22.41%) deceased. Conclusion: Renal involvement was the revealing complication in the diagnosis of FMF which exists in our country, and is still underdiagnosed.

MeSH terms

  • Adolescent
  • Adult
  • Amyloidosis* / pathology
  • Familial Mediterranean Fever* / complications
  • Familial Mediterranean Fever* / diagnosis
  • Familial Mediterranean Fever* / epidemiology
  • Female
  • Humans
  • Male
  • Mutation
  • Nephrotic Syndrome* / complications
  • Pyrin / genetics
  • Young Adult

Substances

  • Pyrin
  • MEFV protein, human