Nationwide Experience With Off-Label Use of Interleukin-1 Targeting Treatment in Familial Mediterranean Fever Patients.

OBJECTIVE: Approximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. METHODS: In all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. RESULTS: In total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. CONCLUSION: Anti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.

The process from symptom onset to rheumatology clinic in polymyalgia rheumatica.

Polymyalgia rheumatica (PMR) is an inflammatory disease of individuals aged over 50 years. Because of the concomitant malignancy possibility and the high prevalence of constitutional symptoms seen in this condition, patients with classical clinical picture often experience delay in diagnosis and treatment and are exposed to a wide list of laboratory and imaging procedures. In this study, we aimed to explore the adventure these patients experience from symptom onset to rheumatology clinic. A total of 106 PMR patients (84 women, 22 men) mean age 70.1 ± 8 were analyzed retrospectively. The time period from the onset of symptoms and referral to rheumatology specialists was explored. Diagnostic methods applied to these patients, antibiotic use and hospitalization during this period were recorded. The interval between the onset of the symptoms and admission to rheumatology unit was 13 ± 13 months. In this period, abdominal computed tomography (29.2 %), chest computed tomography (21.7 %), cranial magnetic resonance imaging (18.9 %) and whole-body scintigraphy (3.8 %) were applied to the patients. About 30 % of the patients were hospitalized for a mean period of 7 ± 3 days before referral to rheumatology unit, and 30 % of the patients were given antibiotics. In order to reduce the delay in the diagnosis of PMR and prevent unnecessary and expensive diagnostic methods, education of clinicians about the diagnosis of PMR may be beneficial.