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 doses≦150 mg/kg), while Pred. was gradually tapered to
0.5~0.8 mg/kg per day. Then CTX was repeated every 3 months (total doses≦50 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liver functions turned to normal when the drug was withdrawn.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.