Effectiveness and safety of ruxolitinib for the treatment of refractory systemic idiopathic juvenile arthritis like associated with interstitial lung disease : a case report

We read with interest the report of a series of 61 patients with systemic juvenile idiopathic arthritis (s-JIA) or s-JIA-like associated with high-fatality lung diseases.1 Lung disease was associated with digital clubbing, a high frequency of anaphylactic reactions to tocilizumab, and macrophage activation syndrome (MAS). Because of the low 5-year survival probability of 42%, there is an urgent need to identify efficient drugs to treat such patients. Herein we report the effectiveness and safety of ruxolitinib in one patient demonstrating clinical and radiological characteristics consistent to those reported by Saper et al A 2-year-old girl born to non-consanguineous parents presented with s-JIA-like since the age of 12 months, including recurring episodes of unexplained fever, urticaria, arthralgia, poor general health status, leukocytosis and elevated serum C-reactive protein (CRP). The use of corticosteroids resulted in a complete remission but the patient relapsed when prednisone was tapered below 0.5 mg/kg/day. Owing to this corticosteroid dependence, the patient received several lines of biological agents from the …