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- Title
Comorbidities in familial Mediterranean fever: analysis of 2000 genetically confirmed patients.
- Authors
Balcı-Peynircioğlu, Banu; Kaya-Akça, Ümmüşen; Arıcı, Zehra Serap; Avcı, Edibe; Akkaya-Ulum, Z Yeliz; Karadağ, Ömer; Kalyoncu, Umut; Bilginer, Yelda; Yılmaz, Engin; Özen, Seza
- Abstract
Objectives FMF is the most common periodic fever syndrome, characterized by recurrent episodes of fever and serosal inflammation accompanied with high acute phase reactants. The analysis of possible comorbidities is important to understand the impact of these conditions on clinical care and whether they share a common aetiological pathway. In this study, we aimed to evaluate the comorbidities associated with FMF patients in a large genetically diagnosed cohort. Methods We retrospectively evaluated the medical and genetic records of FMF patients who were followed up by rheumatologists in Hacettepe University for 15 years. The FMF patients who had homozygous or compound heterozygous mutations were included in the study. Comorbidities associated with FMF were divided into three groups: (i) comorbidities directly related to FMF, (ii) comorbidities due to increased innate inflammation, and (iii) comorbidities that were regarded as being incidental. Results A total of 2000 patients with a diagnosis of FMF were enrolled in the study. Among them 636 were children (31.8%) and M694V was the most common mutation in patients with associated inflammatory conditions. The frequency of AS, Iga Vasculitis (Henoch–Schönlein purpura), juvenile idiopathic arthritis, polyarteritis nodosa, multiple sclerosis and Behçet's disease were increased in patients with FMF when compared with those in the literature. Conclusion This study represents the largest genetically confirmed cohort and compares the frequencies with existing national and international figures for each disease. The increased innate immune system inflammation seen in FMF may be considered as a susceptibility factor since it predisposes to certain inflammatory conditions.
- Subjects
TURKEY; GENETIC disorder diagnosis; GENETIC disorders; INFLAMMATION; MEDICAL records; GENETIC mutation; COMORBIDITY; GENETIC testing; RETROSPECTIVE studies; DESCRIPTIVE statistics; ACQUISITION of data methodology; DISEASE complications
- Publication
Rheumatology, 2020, Vol 59, Issue 6, p1372
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/kez410