Early Disease Onset and Arthritis Are Predictors of Chronic Kidney Disease Development in FMF Patients

Abstract

Background: Familial Mediterranean fever (FMF) is an autosomal recessive genetic disease characterized by fever and serositis attacks. The most important complication is amyloidosis. In FMF patients, chronic kidney disease (CKD) can develop without amyloid development. The aim of the study is to evaluate the development of CKD in FMF patients and to determine the factors that are involved in this development.Method: One hundred seventy eight FMF patients who were followed up between 2000 and 2020 were included in the study. FMF diagnosis was made according to the Tel-Hashomer criteria. Genetic tests were studied in cases which there was suspicion of diagnosis. Clinical and demographic characteristics of patients and all laboratory data including urea, creatinine, estimated glomerular filtration rate (eGFR), and proteinuria in 24-hour urine at the time of first and last admission were evaluated.Results: The mean age of the patients was 34.53 ± 10.72, the follow-up period was 6.12 ± 3.94, and the diagnosis age was 21.7 ± 11.5 years. The number of patients with late disease onset and the percentage of kidney biopsy performed were higher in the genetic test group. There was no difference in the inflammatory parameters. The risk factors associated with the development of CKD were early disease onset and arthritis attacks.Conclusion: The role of genotype characteristics in the development of CKD has not been determined. Patients diagnosed with FMF disease at an early age and especially with arthritis attacks should be closely monitored in terms of the risk of developing CKD.

The spectrum of urological disease in familial Mediterranean fever: amyloidosis and beyond

“…They are mainly related to the uncontrolled inflammatory status in some FMF patients rather than being a genotype-phenotype variety. 30 Over-expressed pro-inflammatory cytokines in FMF proved to stimulate endothelial and leukocyte cells in the kidney, leading to excessive evolution of reactive oxygen species. This in turn causes disruption to the glomerular endothelial structure and activates the coagulation system.…”

Serum Uric Acid Level Among Children with Familial Mediterranean Fever

“…The non-amyloid kidney disease in FMF patients includes mesangial IgA nephropathy, crescentic, rapidly progressing glomerulonephritis, diffuse proliferative glomerulonephritis, IgM nephropathy, and vasculitis. They are mainly related to the uncontrolled inflammation (8). Inflammation also plays an important role in the pathogenesis of CKD.…”