CINCA syndrome: a differential diagnosis of the juvenile idiopathic arthritis

CINCA syndrome (chronic-infantile-neurological-cutaneousarticular) is a rare multisystemic inflammatory disease with neonatal onset characterized by fever, skin rash, articular, and central nervous system involvement. This syndrome is known in the North American medical literature as infantile onset multisystem inflammatory disease (NOMID). We describe the case of a 3-yearold child admitted in our service with fever and skin rash since the neonatal period. She presented seizures at 6 months-old and bilateral arthritis of the knees since her 9 months. On admission she looked sick, pale and presented generalized erythematosus maculopapular rash, arthritis of the knees and ankles. She presented growth and mental retardation too. Laboratory Endings included anemia, leukocytosis, thrombocytosis, elevated C-reactive protein levels and the examination Endings from the cerebrospinal fluid showed an aseptic meningitis. Other test results were negative. Radiographs of the knees, hips and ankles revealed abonrmalities. The child received treatment with nonsteroidal anti-inflammatory drugs, corticosteroids and methotrexate, with partial response to treatment, leading to improvement in pain and fever defervescence. The etiology of CINCA syndrome remains unknown and no single treatment has been found to be effective. This disease should be distinguished from systemic onset juvenile idiophatic arthritis (JIA), the main differential diagnosis.

CINCA syndrome; NOMID; multisystemic inflammatory syndrome; juvenile idiophatic arthritis


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