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- Title
The Characteristics of Pediatric Patients with Familial Mediterranean Fever Treated with Anti-Interleukin-1 Treatment.
- Authors
Avar Aydın, Pınar Özge; Özçakar, Zeynep Birsin; Aydın, Fatma; Karakaş, Hatice Dilara; Çakar, Nilgün; Yalçınkaya, Fatoş
- Abstract
Objective: Interleukin-1 inhibitors are effective agents used in colchicine resistance or intolerance during the treatment of familial Mediterranean fever. This study aims to review the characteristics of patients treated with interleukin-1 inhibitors and their long-term follow-up in a large pediatric cohort of familial Mediterranean fever patients. Materials and Methods: The study was conducted in a pediatric rheumatology reference center. The patients treated with interleukin-1 inhibitors for at least 6 months were included and compared to other patients with familial Mediterranean fever. Clinical and laboratory characteristics of the cohort were recorded. Results: Among 542 patients with familial Mediterranean fever, 6.1% (n = 33) were treated with interleukin-1 inhibitors. Colchicine resistance was the reason in 82.8% and renal amyloidosis in 17.2% of the patients. Patients with interleukin-1 inhibitors had earlier disease onset and higher frequencies of acute arthritis, chest pain, and erysipelas-like erythema with pathogenic exon 10 mutations of the MEFV gene (all P < .04). All patients diagnosed with renal amyloidosis also received interleukin-1 inhibitors. Six patients were switched from anakinra to canakinumab or vice versa to control ongoing disease activity. Attack frequency was reduced in all patients. Conclusion: Interleukin-1 inhibitors are used in a relatively small number of pediatric patients with familial Mediterranean fever. Patients presenting with earlier disease onset, acute arthritis, chest pain, and erysipelas-like erythema and carrying pathogenic exon 10 mutations of the MEFV gene may show a higher need for interleukin-1 inhibitors. In pediatric familial Mediterranean fever patients who are resistant to colchicine, interleukin-1 inhibitors seem to be highly effective agents.
- Subjects
THERAPEUTIC use of monoclonal antibodies; THERAPEUTIC use of proteins; INTERLEUKINS; AMYLOIDOSIS; PATIENTS; DRUG resistance; PEDIATRICS; TREATMENT effectiveness; COMPARATIVE studies; DEMOGRAPHIC characteristics; COLCHICINE; AUTOINFLAMMATORY diseases; LONGITUDINAL method; CHEMICAL inhibitors; CHILDREN
- Publication
Turkish Archives of Pediatrics, 2022, Vol 57, Issue 4, p448
- ISSN
2757-6256
- Publication type
Article
- DOI
10.5152/TurkArchPediatr.2022.22039