Apostolos Kontzias1, Petros Efthimiou. 1. National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Abstract
OBJECTIVES: We describe the successful treatment of adult-onset Still's disease (AOSD) with canakinumab, a novel anti-interleukin (IL)-1β, long-acting, monoclonal antibody, on patients refractory to anakinra and rilonacept. In many cases the expected positive therapeutic effect of short-acting IL-1 inhibitors is transient or completely absent, leading to our hypothesis that their short half-life may be associated with incomplete IL-1 blockade, given the cyclic nature of the disease. METHODS: We report 2 cases of AOSD resistant to short-acting IL-1 blockade, which were subsequently treated with canakinumab. A retrospective chart review was conducted of patients diagnosed with AOSD in our regional referral center. RESULTS: Response to treatment was assessed by its effect on the systemic symptoms (resolution of fever and rash), polyarthritis (using the disease activity score 28--C-reactive protein score), and the levels of serum ferritin. Canakinumab demonstrated sustained efficacy in both patients as evidenced by clinical and laboratory parameters with minimal adverse reactions. CONCLUSIONS: This is the first documented report of successful use of canakinumab, a novel IL-1β inhibitor, in AOSD patients refractory to traditional disease-modifying antirheumatic drugs and short- to moderate-acting IL-1 blockade. Prospective comparative studies are needed to validate canakinumab's efficacy and safety.
OBJECTIVES: We describe the successful treatment of adult-onset Still's disease (AOSD) with canakinumab, a novel anti-interleukin (IL)-1β, long-acting, monoclonal antibody, on patients refractory to anakinra and rilonacept. In many cases the expected positive therapeutic effect of short-acting IL-1 inhibitors is transient or completely absent, leading to our hypothesis that their short half-life may be associated with incomplete IL-1 blockade, given the cyclic nature of the disease. METHODS: We report 2 cases of AOSD resistant to short-acting IL-1 blockade, which were subsequently treated with canakinumab. A retrospective chart review was conducted of patients diagnosed with AOSD in our regional referral center. RESULTS: Response to treatment was assessed by its effect on the systemic symptoms (resolution of fever and rash), polyarthritis (using the disease activity score 28--C-reactive protein score), and the levels of serum ferritin. Canakinumab demonstrated sustained efficacy in both patients as evidenced by clinical and laboratory parameters with minimal adverse reactions. CONCLUSIONS: This is the first documented report of successful use of canakinumab, a novel IL-1β inhibitor, in AOSD patients refractory to traditional disease-modifying antirheumatic drugs and short- to moderate-acting IL-1 blockade. Prospective comparative studies are needed to validate canakinumab's efficacy and safety.