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

Yazılıtaş, Fatma; Çakıcı, Evrim Kargın; Şükür, Eda Didem Kurt; Özdel, Semanur; Arda, Nilüfer; Güngör, Tülin; Bülbül, Mehmet

doi:10.1159/000506289

Cited by 4 publications

(3 citation statements)

References 20 publications

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“…Twelve of these patients had amyloidosis in biopsy, two had FSGS, and one had IGA nephropathy and another had MPGN. Similar results were obtained with a small number of studies investigating non-amyloid kidney diseases (10,11,22) Early diagnosis and presence of arthritis were determined as factors associated with the development of CKD. The role of genetic mutations was not found.…”

Section: Discussionsupporting

confidence: 82%

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

Büberci

Duranay

2021

Preprint

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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.

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Section: Discussionsupporting

confidence: 82%

“…The clinic of two amyloidosis patients (15.3%) was found to be phenotype II. This rate was found to be 13.3% in the study by Yazilitas et al and 17.1% in the study by Huzmeli et al (10,11).…”

Section: Discussionmentioning

confidence: 82%

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

Büberci

Duranay

2021

Preprint

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“…Many types of glomerulonephritis (GN) have also been reported. IgA nephropathy, focal GN, and mesangioproliferative GN were the most prevalent non-amyloid glomerular lesions [ 26 ].…”

Section: Renal Involvement In the Four Canonical Monogenic Aidsmentioning

confidence: 99%

Autoinflammatory diseases and the kidney

et al. 2023

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The kidney represents an important target of systemic inflammation. Its involvement in monogenic and multifactorial autoinflammatory diseases (AIDs) vary from peculiar and relatively frequent manifestations to some rare but severe features that may end up requiring transplantation. The pathogenetic background is also very heterogeneous ranging from amyloidosis to non-amyloid related damage rooted in inflammasome activation. Kidney involvement in monogenic and polygenic AIDs may present as renal amyloidosis, IgA nephropathy, and more rarely as various forms of glomerulonephritis (GN), namely segmental glomerulosclerosis, collapsing glomerulopathy, fibrillar, or membranoproliferative GN. Vascular disorders such as thrombosis or renal aneurysms and pseudoaneurysms may be encountered in patients with Behcet’s disease. Patients with AIDs should be routinely assessed for renal involvement. Screening with urinalysis, serum creatinine, 24-h urinary protein, microhematuria, and imaging studies should be carried out for early diagnosis. Awareness of drug-induced nephrotoxicity, drug-drug interactions as well as addressing the issue of proper renal adjustment of drug doses deserve a special mention and should always be considered when dealing with patients affected by AIDs. Finally, we will explore the role of IL-1 inhibitors in AIDs patients with renal involvement. Targeting IL-1 may indeed have the potential to successfully manage kidney disease and improve long-term prognosis of AIDs patients.

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How to handle the main drugs to treat autoinflammatory disorders and how we treat common autoinflammatory diseases

Koné‐Paut

Dusser

2020

G Ital Dermatol Venereol

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

Cited by 4 publications

References 20 publications

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

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

Autoinflammatory diseases and the kidney

How to handle the main drugs to treat autoinflammatory disorders and how we treat common autoinflammatory diseases

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