PMC3995009_01
Clinical features of chronic infantile neurologic, cutaneous, articular syndrome in the patient. A, Persistent urticaria-like skin rash despite canakinumab treatment (4 mg/kg every 8 weeks). At the time this photograph was taken (when the patient was 7 years old), the cryopyrin-associated periodic syndromes Disease Activity Score (DAS) was 11 of 20, and the serum amyloid A (SAA) level was elevated at 154 mg/liter (normal <10). B, Improvement of the skin rash 8 weeks after the dosage of canakinumab was increased to 8 mg/kg every 8 weeks. At the time this photograph was taken, the DAS had declined to 1 of 20, but the SAA level was still elevated at 118 mg/liter. C, Patellar and tibial bony overgrowth, seen on a radiograph obtained when the patient was 7 years old.
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During his first year of life he was frequently hospitalized due to fever, urticarial skin rash (Figure 1A), and elevated acute-phase reactant levels, without an infectious cause. At age 18 months he was diagnosed as having CINCA syndrome based on the above-mentioned clinical features and the additional presence of synovitis of both knees with fixed flexion deformity and bony overgrowth of the left patella and tibia (Figure 1C). The CAPS DAS remained unchanged until the canakinumab dosage reached 8 mg/kg every 8 weeks, at which point the DAS declined to 1 of 20 (with notable improvement of the skin rash, fevers, arthralgia, myalgia, headaches, and energy levels) (Figure 1B), and the prednisolone was tapered and then discontinued completely.