FDA Approves Treatment for Still's Disease

July 14, 2020
Rheumatology Network Editorial Staff

The U.S. FDA has approved the first treatment for adult-onset Still's disease, a rare form of systemic juvenile idiopathic arthritis associated with high fever, arthritis and rash.

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Canakinumab (Ilaris, Novartis) has been approved by the U.S. Food and Drug Administration to treat adult-onset Still’s disease (AOSD), a rare and severe form of juvenile rheumatoid arthritis that affects adults.

It is the first FDA-approved treatment for the condition. “Prior to today’s approval, patients had no FDA-approved treatments for their disease, which can include symptoms such as painful arthritis, fevers and rash,” said Nikolay Nikolov, M.D., acting director of the FDA’s rheumatology and transplant medicine center within the Center for Drug Evaluation and Research.

Still’s is associated with high spiking fevers, joint inflammation, inflammation of internal organs and skin rashes. It is a rare condition and, according to an analysis conducted in Europe, there are 0.1 to 0.4 cases per 100,000 cases.

Canakinumab is an IL-1 inhibitor that is well-established in JIA. It is currently approved to treat Periodic Fever Syndromes , including Familial Mediterranean Fever, Cryopyrin-Associated Periodic Syndromes (CAPS), Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS), and Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD).

The safety and efficacy of canakinumab was demonstrated in a study of 216 children, 56 young adolescents and 29 older adolescents and young adults with systemic JIA. Canakinumab was administered at 4 mg/kg every four weeks. By day 15 of treatment, at least 50 percent of patients across all age groups achieved ACR 70.