Inflammatory comorbidities ın the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG).

Publisher: Springer Country of Publication: Germany NLM ID: 8211469 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1434-9949 (Electronic) Linking ISSN: 07703198 NLM ISO Abbreviation: Clin Rheumatol Subsets: MEDLINE

Publication: <2008->: Heidelberg : Springer
Original Publication: Brussels : Acta Medica Belgica, [1982-

Familial Mediterranean Fever*/complications ; Familial Mediterranean Fever*/epidemiology ; Familial Mediterranean Fever*/drug therapy ; Rheumatology* ; Arthritis, Juvenile*/drug therapy; Humans ; Child ; Retrospective Studies ; Mutation ; Colchicine/therapeutic use ; Pyrin/genetics

Aim: The aim of this study was to investigate the frequency and type of FMF-associated inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF patients without concomitant inflammatory diseases.
Materials and Methods: Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1). The clinical findings and treatments were compared between the two groups.
Results: The study group comprised 3475 patients with FMF. There were 294 patients (8.5%) in group 1 and 3181 patients (91.5%) in group 2. Juvenile idiopathic arthritis (n = 136) was the most common accompanying inflammatory disease. Arthritis, M694V homozygosity, and the need for biological therapy were more frequently observed in Group 1 (p < 0.05). Fever and abdominal pain were more frequently detected in Group 2 (p < 0.05). FMF patients with concomitant inflammatory diseas more frequently demonstrated colchicine resistance. There were no significant differences in the median attack frequency, chest pain, amyloidosis, erysipelas-like erythema, or family history of FMF between the two patient groups.
Conclusion: To the best of our knowledge, this is the largest pediatric cohort reviewed to date. FMF patients may have different clinical profiles and colchicine responses if they have with concomitant inflammatory diseases. Key points • FMF is associated with some inflammatory comorbidities diseases. • To the best of our knowledge, this is the largest cohort evlauated pediatric FMF associated inflammatory comorbidities diseases reviewed to date.
(© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

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Keywords: Concomitant; Familial; Fever; Inflammatory

SML2Y3J35T (Colchicine)
0 (Pyrin)

Date Created: 20231105 Date Completed: 20240109 Latest Revision: 20240130

20240130

10.1007/s10067-023-06802-6

37926798