Pediatric Rheumatology Online Journal → June 2003 → Systemic Onset Arthritis → Abstract #15

FAVORABLE OUTCOME IN PATIENTS WITH RENAL INVOLVEMENT COMPLICATING MACROPHAGE ACTIVATION SYNDROME (MAS) IN SYSTEMIC-ONSET JRA (SoJRA)

A. V. Ramanan,¹ N. D. Rosenblum,¹ B. M. Feldman,¹ R. M. Laxer,¹ R. Schneider.¹

¹Division of Rheumatology, Hospital for Sick Children, Toronto, ON, Canada

Background: MAS, a serious and potentially fatal complication of SoJRA, causes death in 10-20% of cases. Renal involvement with MAS has been reported to be associated with a particularly poor outcome. We report a favorable outcome in 3 SoJRA patients with MAS and renal involvement.
Patients: All patients had typical features of MAS including fever, splenomegaly, lymphadenopathy, cytopenia (involving at least 2 cell lines), hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia, raised LDH, raised transaminases and coagulopathy. Bone marrow biopsy was done in all 3 patients; hemophagocytosis was seen in only 1.
The mean age was 12.4 yrs with 2F:1M. The episodes were seen at 10yrs and 5yrs after onset in 2 patients and at onset in one. Proteinuria and hematuria were present in all 3. 1 pt. had WBC casts and the other 2 had RBC casts. 24 hr urinary protein was 0.65 and 28.20g/24hrs in the 2 patients in whom it was done. The peak creatinine was 348, 449 and 64 umol/l in the 3 patients respectively. One patient had CNS, cardiac and lung involvement. 2 patients needed ICU admission. All 3 patients developed renal involvement within 48 hours of diagnosis. None required dialysis. None had renal biopsy performed. All 3 were treated with steroids. In addition cyclosporin and etoposide was used in one patient. All 3 had full recovery with normal renal function.
Conclusions: Significant renal involvement, which may be associated with renal failure, can occur in patients with SoJRA and MAS. Prompt and intensive treatment may result in recovery with normal renal function.