All issues > Volume 41(1); 1998

Case Report

J Korean Pediatr Soc. 1998;41(1):129-134. Published online January 15, 1998.

A Fatal Case of Systemic Juvenile Rheumatoid Arthritis with Disseminated Intravascular Coagulation

Eun Young EY Park¹, Sung Hee SH Oh¹

¹Department of Pediatrics, College of Medicine, Hanyang University, Seoul, Korea

Correspondence Sung Hee SH Oh ,Email: 1

Abstract

Disseminated intravascular coagulation(DIC) occurs rarely but can cause death in patients with juvenile rheumatoid arthritis(JRA), especially in the systemic type. The reports of DIC complicating JRA have increased recently. We experienced a 13 - year - old female patient with systemic JRA who died of DIC. The patient had been managed with aspirin, steroid, nonsteroidal anti-inflammatory drug for 7 years, during which time several episodes of aggravation had occured. The last episode of clinical aggravation had been treated with increased doses of naproxen and the addition of sulfasalazine with some improvement, but she was readmitted a few days later due to the development of persistent fever with chills, nausea, abdominal pain, and rash. The clinical features had been aggravated further with lymphadenopathy, hepatosplenomegaly, jaundice, mental change, gastrointestinal and vaginal bleeding, and renal and respiratory failure. The laboratory findings revealed pancytopenia, low ESR, increased hepatic enzymes, and features of DIC, and renal and respiratory failure. The patient, died despite vigorous treatments including discontinuation of naproxen, and sulfasalazine, and the administration of high doses of prednisolone and intravenous immunoglobulin.

Keywords :Disseminated intravascular coagulation, Systemic juvenile rheumatoid arthritis