Evidence for intravascular coagulation in systemic onset, but not polyarticular, juvenile rheumatoid arthritis

• Published in: Arthritis and rheumatism Vol. 28; no. 3; p. 256
• Main Authors: Scott, J P, Gerber, P, Maryjowski, M C, Pachman, L M
• Format: Journal Article
• Language: English
• Published: United States 01.03.1985
• Subjects: Adolescent; Arthritis, Juvenile - complications; Arthritis, Juvenile - diagnosis; Child; Child, Preschool; Diagnosis, Differential; Disseminated Intravascular Coagulation - complications; Disseminated Intravascular Coagulation - diagnosis; Female; Fibrinogen - analysis; Humans; Male; Thrombocytosis - physiopathology
• ISSN: 0004-3591
• DOI: 10.1002/art.1780280304

After observing a child with systemic onset juvenile rheumatoid arthritis (S-JRA) who developed purpura fulminans in association with disseminated intravascular coagulation, with subsequent gangrene and autoamputation, we undertook a prospective study of coagulation parameters in children with JRA. Ten consecutive children with S-JRA, 10 children with rheumatoid factor-negative, polyarticular juvenile rheumatoid arthritis (P-JRA), and 10 age- and sex-matched controls were studied. Routine coagulation screening tests were performed, as were tests for plasma fibrinopeptide A (a sensitive measure of intravascular thrombin generation), factor VIII-related antigen (an endothelial cell protein), and platelet factor 4 (a platelet-secreted protein). Our studies suggest that activation of intravascular coagulation is common in systemic onset JRA, but not in rheumatoid factor-negative, polyarticular disease. The coagulopathy may cause severe morbidity. In addition, marked elevations of plasma factor VIII-related antigen suggest perturbation of endothelial cells and vascular involvement in S-JRA, but not in P-JRA. Normal ranges of platelet factor 4 indicate that intravascular platelet consumption does not occur in either type of JRA, despite the thrombocytosis common in both.