PRECORDIAL PAIN, LEUKOCYTOSIS AND BICYTOPENIA IN A TEENAGER WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS UNDER IMMUNOSUPPRESSIVE THERAPY

Revista paulista de pediatria : orgão oficial da Sociedade de Pediatria de São Paulo
Marina de Sousa VieiraJosé Roberto Provenza

Abstract

To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Sys...Continue Reading

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BETA
ESR
biopsy

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