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BackgroundFibrodysplasia ossificans progressiva (FOP) is the most catastrophic form of heterotopic ossification, due to ongoing intracellular signaling through the bone morphogenic protein pathway. The paroxysmal appearance of inflammatory lumps and elevated inflammatory markers during flares, suggest that FOP is an auto-inflammatory disease. Based on evidence, demonstrating a role for interleukin-1β (IL-1β) in other forms of heterotopic ossification, we hypothesized that treating FOP patients with anti-IL-1 agents could help lower the rate of FOP paroxysms and/or limit the symptoms and residual lesions.Case presentationA 13.5-year-old Arab boy was diagnosed with FOP. Treatment with anti-inflammatory drugs did not change the disease course. New lumps appeared in a rate of approximately one every 8 days. Treatment with the anti-IL-1 agents anakinra and canakinumab resulted in significantly lower rate of paroxysms (every 22–25 days, of which almost all involved only 2 existing lumps), as well as shorter duration. High levels of IL-1β were found in the patient’s plasma samples, collected during a paroxysm that appeared 8 weeks after the last canakinumab dose. In contrast, IL-1β plasma levels were undetectable in the previous three plasma samples, obtained while he was treated with anti-IL-1 agents.ConclusionsOur data demonstrate the efficacy of anti-IL-1 agents in the treatment of a patient with FOP. Results showing the marked increase in IL-1β plasma levels during a paroxysm support a role for IL-1β in the pathogenesis of FOP and further provide the rationale for the use of anti-IL-1 agents in FOP treatment.

BackgroundJuvenile idiopathic arthritis (JIA) might lead to decreased physical activity and quality of life. Physical activity is an important part of children's social development and is important in the treatment of JIA.ObjectivesTo characterize clinical aspects, physical activity rates, obesity, and screen time in a group of JIA patientsMethodsOver a 6-month period, a cohort of consecutive JIA patients in the Pediatric Rheumatology Clinic at Meir Medical Center were evaluated and compared to healthy children. Information on disease activity, type and amount of physical activity (using the Modified Godin Leisure-Time Exercise Questionnaire), and daily screen time hours were collected.Results97 patients and 98 matched controls completed the questionnaires. Among the patients, 56% had oligoarthritis, 22% polyarthritis and 17% systemic disease. Disease activity among all JIA sub-groups was low (average of 1.7 out of 10), two thirds of patients had a disease activity lower than 3, only 4% over 5, and 56% were in clinical remission. Obesity rates in the patient group and control groups were 21.5% and 19.4%, respectively. Physical activity levels were similar in both groups. There was a statistically significant reverse correlation between physical activity and age. The “total weekly leisure activity” of the control group was higher (46.9 vs. 38.4 hours, respectively). Daily screen time was similar (3.2 vs. 2.9 hours).ConclusionsPhysical activity, screen time hours and obesity rates were similar between JIA patients and controls. This lack of difference could be attributed to the clinical remission following early, aggressive, treat-to-target therapy.Disclosure of InterestNone declared

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