미만성 증식성 낭창성 신염의 임상상 및 예후인자

Abstract

Lupus nephritis is a major cause of morbidity and mortality arising from systemic lupus erythematous. It is generally acknowledged that the presence of diffuse proliferative lupus nephritis(DPLN) is highly predictive of a poor prognosis in terms of renal and patient outcome on survival. The objective of this study was to evaluate the clinicopathologic characteristics, renal outcome according to therapeutic regimen. and prognostic factors of biopsy-proven diffuse proliferative lupus nephritis. Among the biopsy-proven lupus nephritis patients who were admitted to Yonsei University Medical Center from January 1986 to June 1997, 36 patients treated for at least 6 months and regularly followed-up for at least 12 months were included. We retrospectively reviewed the medical recorders. Patients were treated with steroid regimen with or without cyclophosphamide. According to the therapeutic response, patients were divided into two groups : a therapeutic response group(n=24), and a therapeutic non-response group(n=12). The mean age of the patients was 27.4 years and the mean follow-up duration was 51 months. Lupus nephritis developed at a mean 9.7 months after SLE diagnosis and mean duration of nephritis was 39.2 months. Mean serum creatinine was 1.6㎎/dL, 24 hour proteinuria was 4,873㎎, and anti-DNA antibody was positive in 81% of patients at the time of renal biopsy. Activity index and chronicity index were 10.4 and 2.8, respectively. Overall 5 year renal survival rate was 75% and no difference between steroid single therapy and cyclophosphamide combination therapy was observed. Factors affecting therapeutic response included delayed development of nephritis(3.1 vs 13.8 months, p<0.05) and elevated serum creatinine level(0.9 vs 1.9㎎/dL, p<0.05), which were associated with poor therapeutic response. Other clinicopathologic, biochemical and immunologic parameters were not different between the therapeutic response group and the therapeutic non-response group. In conclusion, delayed development of lupus nephritis and elevated serum creatinine at nephritis presentation are poor prognostic factors of DPLN, but further randomized prospective study(including divided cytoxan intravenous pulse therapy and oral therapy, with long-term follow-up) is necessary.