A STUDY OF SEQUENTIAL TREATMENT FOR CHILDHOOD SYSTEMIC LUPUS ERYTHEMATOSUS

Hu J, Li G-L, Zhang X, Chen X-Y, Liu Y

Department of Immunology and the Department of Nephrology, Tianjin Children’s Hospital, Tianjin, China

 

Objective: To evaluate the effect of sequential treatment for childhood systemic lupus erythematosus.

Methods: The methods of Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), weighted averages of SLEDAI (WAS) and Systemic Lupus International College of Rheumatology Damage Index (SLICC/ACR-DI) were evaluated at the beginning of the treatment. Five patients (CTX group) with renal and/or nervous system damage determined by SLEDAI and DI were first treated with pulsed doses of methylprednisolone (MP, 15~30mg/kg per day for 3 days intravenously), followed by prednisone (Pred.) therapy (1 mg/kg per day orally). Three to four weeks after the initial administration of Pred., pulse cyclophosphamide (CTX) was given intravenously (8~12 mg/kg per day for 2 consecutive days, repeated every 2 weeks, total doses150 mg/kg), while Pred. was gradually tapered to 0.5~0.8 mg/kg per day. Then CTX was repeated every 3 months (total doses50 mg/kg). The patients received funduscopy every 3 months after the onset of HCQ therapy. Six to ten months after the ending of CTX therapy, tripterygium wilfordii hook. f (TWHf) was added for 3~6 months. The average minimal effective doses of Pred. then was 12.5~17.5 mg/d. For the other 7 patients (non-CTX group), oral Pred. (1.5~2.0 mg/kg) was administrated initially and tapered in 1 month after resolution of clinical symptoms. HCQ (same doses as CTX group) and TWHf (same doses as CTX group for 4~9 months) were given when Pred. was tapered to 1.0 mg/kg per day. The final maintenance dose of Pred. was 12.5~17.5 mg/kg per day in non-CTX group.

Results: The SLEDAI of the patients in CTX group was higher than 25. Severe nephritis or nephritic syndrome occurred in all of the five cases, and nervous system abnormalities (epilepsy and organic brain syndrome) occurred in two. The liver functions turned to normal when the drug was withdrawn. Transient leukopenia occurred in 2 patients of the CTX group 1 week after the initial administration of CTX. The average duration of HCQ therapy of both groups was 14 months (6~48months), None retinopathy or vision damage occurred. Intercurrent infections of herpes-simplex virus and varicella-zoster virus occurred in 2 and 3 children respectively during the overall period of the treatment.

Conclusion: Choosing appropriate drugs on the basis of SLEDAI and DI for sequential treatment can achieve satisfied clinical outcome and improve the life quality of those with SLE.

 
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