FRI0455 IS THERE AN INCREASE IN THE FREQUENCY OF INFLAMMATORY DISEASES IN THE FAMILIES OF PATIENTS WITH FMF?. (13th June 2020)

• Record Type: Journal Article
• Authors: Yildirim, S.; Yildiz, M.; Aliyeva, A.; Haslak, F.; Koker, O.; Adrovic, A.; Sahin, S.; Barut, K.; Kasapcopur, O.
• Is Part Of: Annals of the rheumatic diseases. Volume 79(2020)Supplement 1
• Page Start: 824
• Page End: 825
• Publication Date: 2020-06-13
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2020-eular.6263
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: Familial Mediterranean Fever (FMF) is the most common periodic fever syndrome in childhood with an autosomal recessive inheritance pattern and is characterized by unprovoked fever attacks, serositis episodes. The causative gene of the disease is MEFV that encodes pyrin protein. The pyrin protein takes a role in pathways related to inflammation, and mutations of it lead to increased inflammation. It is already shown that frequencies of some certain diseases like PAN, HSP increase in patients with FMF. Nevertheless, frequencies of inflammatory diseases in families of patient with FMF haven't been investigated. Objectives: In this study, we have aimed to evaluate the comorbid disorders in a large cohort of families of patients with FMF. Methods: Four hundred and ninety-eight children with FMF, one hundred and forty patients with JIA and ninety-two healthy children were interviewed between December 2019 and January 2020. In JIA group and healthy control group, patients who have family history for FMF were excluded from the study. Patients were asked about characteristics of their disease attacks and if there is a relative with any inflammatory diseases who does not have FMF in patient's 1 st and 2th degree relatives. Results: Demographic features of study group have shown in Table 1 . The most common MEFV mutations in patients with FMF were: M694V homozygotes (13.2 %), M694V heterozygotes (12 %), M694V homozygotes and R202Q homozygotes (6, 8 %). Type II diabetes, asthma and hypothyroidism were the most commonly detected diseases in all cohorts. Frequency of Behçet's disease, allergic rhinitis and type II diabetes were significantly higher in families of patients with FMF than other groups (p<0.05) (Table 2 ). Conclusion: In this study, we have reported increased frequencies of Behçet's disease, allergic rhinitis and type II diabetes in families of patients with FMF. Our results suggest that possible increased mutation load among families of patients with FMF may cause increased inflammatory diseases. References: [1]Yildiz M, Adrovic A, Tasdemir E, et al. Evaluation of co-existing diseases in children with familial Mediterranean fever. Rheumatol Int . 2020;40(1):57–64. doi:10.1007/s00296-019-04391-9 Disclosure of Interests: None declared