Characteristics of patients with adult-onset familial Mediterranean fever in Turkey: analysis of 401 cases

Abstract

It has been generally accepted that the clinical onset of familial Mediterranean fever (FMF) begins before 20 years of age in most patients. In this study, we aimed to investigate the demographic and clinical characteristics of our FMF patients with an age of onset > or =20. Records of 401 patients (female/male: 204/197) that followed up between 1990 and 1999 were reviewed according to a pre-defined protocol. All patients fulfilled the diagnostic criteria of Livneh et al. The demographic and clinical features of adult-onset FMF patients were compared to those of patients with a disease onset before 20 years of age. There were 57 patients (14%) who experienced symptoms of FMF at > or =20 years of age; 34 of them (8.5%) reported their first attack between 20 and 29 years of age; 18 of them (4.5%) between 30 and 39 years of age and five patients (1.25%) had their first attack after 40 years of age. Arthritis (42 vs. 65%, p = 0.001) and erysipelas-like erythema (7 vs. 17%, p = 0.047) were significantly less frequent in patients with adult-onset FMF compared to patients with disease onset before 20 years of age. Arthritis and erysipelas-like erythema were less frequent in adult-onset patients compared to those with an earlier disease onset. Adult-onset FMF may be a form of disease with distinct clinical, demographic and molecular characteristics. Prospective clinical studies and investigation of genotypic features are needed to identify the characteristics of this phenotypic variant.

The Effect of M694V Mutation on Clinical Presentation and Acute Phase Response in Children with Familial Mediterranean Fever: Single Center Experience in Western Turkey

“…Alt ho ugh a ma le pre do mi nan ce has be en re por ted in Arab, Ar me ni an, and Je wish and Tur kish pa ti ents, we ob ser ved a slight fe ma le su peri o rity (ma le:fe ma le ra ti o of 1:1.2). 2,[6][7][8][9][10] In addition, the age of the di se a se on set and the di ag no sis we re fo und to be smaller compared to lar ge stu di es and si mi lar to a study from Tur key. 2,9,11 In our se ri es, abdo mi nal pa in was the most com mon fe a tu re (97.7%), fol lo wed by fe ver (72.4%) and jo int in volve ment (58.6%).…”

Familial Mediterranean Fever Patient with Onset of Attacks at the Age of Seventies

“…Our patient also has a mild disease without any amyloidosis and responded well to colchicine treatment. In another study from Turkey, Sayarlioglu et al reported that among 401 FMF patients, 57 (14 %) were adult onset FMF (disease onset over 20 years of age) and 5 of them (2.25 %) had the first attack after 40 years of age [8]. In the study of Ureten et al, among 260 patients, 77 (30 %) were adult onset FMF but none of the patients were late onset FMF [9].…”

Idiopathic chronic polyserositis with massive ascites responding to colchicine in an adult: A case report

“…However, a late onset of the disease has also been reported for both entities, with 14%-30% of patients manifesting FMF in late adulthood up to the age of 65 [5,22,23] , and some cases of TRAPS appearing up to the age of 63 years [17] . Late onset cases present as a milder disease and they are frequently related to low-penetrance mutations [22] . Adult-onset FMF and TRAPS patients may develop atypical manifestations, including recurrent serosal involvement as the only clinical feature [18,24,25] .…”