Full Text: Case Report

Favorable Outcome in Patients with Renal Involvement Complicating Macrophage Activation Syndrome in Systemic Onset Juvenile Rheumatoid Arthritis

ATHIMALAIPET V. RAMANAN, NORMAN D. ROSENBLUM, BRIAN M. FELDMAN, RONALD M. LAXER, and RAYFEL SCHNEIDER

ABSTRACT.

Systemic-onset juvenile rheumatoid arthritis (SoJRA) constitutes about 10-20% of all JRA. However more than two-thirds of the mortality seen in JRA patients is accounted for by SoJRA. Macrophage activation syndrome (MAS), which can also be considered as a form of secondary hemophagocytic lymphohistiocytosis, is a major cause of morbidity and mortality in children with SoJRA. MAS is characterized by persistent high fever, pancytopenia, mild to serious derangements of liver cell function, encephalopathy, and disseminated intravascular coagulation. Renal involvement in MAS is a rarely recognized feature. In 2 recently reported case series of MAS in SoJRA, renal involvement appeared to be associated with poor prognosis. We describe 3 children with SoJRA who had renal involvement complicating MAS and had a favorable outcome. (J Rheumatol 2004;31:2068-70)

Key Indexing Terms:

MACROPHAGE ACTIVATION SYNDROME
SYSTEMIC ONSET JRA
HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

From the Divisions of Pediatric Rheumatology and Pediatric Nephrology, and the Department of Pediatrics, Health Policy Management and Evaluation, and Public Health Sciences, Department of Pediatrics, Hospital for Sick Children, and University of Toronto, Toronto, Ontario, Canada.

A.V. Ramanan, MD, Division of Pediatric Rheumatology; N.D. Rosenblum, MD, Division of Pediatric Nephrology; B.M. Feldman, MD, Division of Pediatric Rheumatology and Department of Pediatrics, Health Policy Management and Evaluation, and Public Health Sciences, Department of Pediatrics; R.M. Laxer, MD; R. Schneider, MD, Division of Pediatric Rheumatology.

Address reprint requests to Dr. R. Schneider, Division of Pediatric Rheumatology, Hospital for Sick Children, Toronto, ON, Canada, M5G 1X8.

Submitted October 23, 2003; revision accepted April 7, 2004.