The most important complication of familial Mediterranean fever (FMF) is secondary amyloidosis. The aim of this study is to investigate the risk of developing FMF-related amyloidosis with macrophage migration inhibitory factor (MIF), interleukin 4 (IL-4), and IL-1 receptor antagonist (IL-1RA) variants. This study included 62 FMF patients with amyloidosis, 110 FMF patients without amyloidosis, and 120 controls. The clinical information of the patient groups was compared. , variant number tandem repeat (VNTR), and VNTR variants were analyzed for all participants. The use of colchicine, pleurisy, and appendectomy was more common in FMF patients with amyloidosis than in FMF patients without amyloidosis. C/C genotype and C allele were higher in both patient groups compared to controls. VNTR A1/A2 and A1/A4 genotypes and A1-A4 alleles were more common in both patient groups than controls. The VNTR P1 allele was more common in FMF patients with amyloidosis compared to controls. The allele and the VNTR A1-A4 allele are associated with FMF in the Turkish population but not with amyloidosis risk in FMF patients. The VNTR P1 allele is more common in FMF patients with amyloidosis than in healthy individuals.
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http://dx.doi.org/10.1177/10815589231207789 | DOI Listing |
Turk Arch Pediatr
January 2025
Department of Pediatric Rheumatology, Ankara Etlik City Hospital, Ankara, Türkiye.
Objective: Familial Mediterranean fever (FMF) is a hereditary, autoinflammatory disease. The characteristics of siblings with FMF have not been described in large cohorts up to now. This study aimed to examine the features of siblings with FMF.
View Article and Find Full Text PDFClin J Gastroenterol
January 2025
Department of Gastroenterology and Hepatology, Tokyo Medical University Hospital, 6-7-1, Nishi-shinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
We describe a case of familial Mediterranean fever (FMF) with sigmoid colon stricture. The patient, a woman in her 30 s, had a 12-year history of ileocolitis-type Crohn's disease. The colonoscope could not pass because of the sigmoid colon stricture, and the patient was referred to our hospital with complaints of abdominal pain and fever.
View Article and Find Full Text PDFLiver Int
February 2025
Sorbonne Université, Service Médecine Interne, Centre de référence des maladies autoinflammatoires et des amyloses (CEREMAIA), Assistance Publique des hôpitaux de Paris, Hôpital Tenon, Paris, France.
Background: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, associated with MEFV mutations. FMF patients can experience liver involvement, potentially leading to cirrhosis.
Objectives: This study aimed to evaluate liver involvement in FMF patients at a French tertiary centre for adult FMF.
Allergol Immunopathol (Madr)
January 2025
Faculty of Medicine, Department of Pediatric Allergy and Immunology, Ondokuz Mayıs University, Samsun, Turkey.
Background: Familial Mediterranean Fever is a common genetic autoinflammatory disease prevalent in the Mediterranean region. The clinical course of the disease is characterized by fever and serositis attacks. While defects in the innate immune system are known to play a role in the pathogenesis of the disease, the impact of the adaptive immune system remains unclear.
View Article and Find Full Text PDFCurr Pain Headache Rep
January 2025
Department of Anesthesiology, Louisiana State University Health Sciences Center at Shreveport, Shreveport, LA, 71103, USA.
Purpose Of Review: Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disorder predominantly affecting individuals of Mediterranean and Middle Eastern descent, including those with certain heritages including Sephardic Jewish, Armenian, Turkish, and Arab. The disorder affects up to 1 in 200 people making it a very common etiology for pain states worldwide, including serositis mediated painful states of the chest, joint, and abdomen.
Recent Findings: Defined by recurrent episodes of fever and inflammation, FMF can lead to not only severe pain, but complications such as renal amyloidosis, if untreated.
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