To clarify the risk factors related to prognosis in patients with Henoch-Schoenlein purpura nephritis (HSPN), we investigated the cases with HSPN on long-term observation. We enrolled 114 patients who had been diagnosed with HSPN from 1974-1997. These patients were divided into 2 groups based upon features at last follow-up. One group, designated "favorable", consisted of 69 patients with normal urine and 25 patients with minor urinary abnormalities, and the second group, designated "unfavorable", consisted of 15 patients with active renal disease and 5 patients with renal failure. The clinical features, laboratory data and pathological findings were investigated in 2 groups. Nephrotic syndrome, decreased factor XIII activity, hypertension and renal failure at onset were more frequent in "unfavorable" than in "favorable". The rate of glomeruli with crescents, macrophage infiltrations, tubulointerstitial changes and acute exacerbation in "unfavorable" were higher than those in "favorable". There were 5 cases with renal insufficiency, and renal survival rate was 95.6% for over 15 years. These results suggest that the above mentioned risk factors play an important role in prognosis of the patients with active renal disease and renal failure.

Mizoribine (MZB) is an imidazole nucleoside and an immunosuppressive agent. The immunosuppressive effect of MZB has been reported to be due to the inhibition of DNA synthesis in the S phase of the cell cycle. Because of its relative lack of toxicity, during the past decade MZB has been frequently used instead of azathioprine as a component of immunosuppressive drug regimens. MZB is being used to treat renal transplantation patients, IgA nephropathy, lupus erythematosus, and childhood nephrotic syndrome (NS), and some recent studies have assessed the efficacy of oral MZB pulse therapy for severe lupus nephritis, steroid-resistant NS, and frequently relapsing-steroid-dependent NS. This review summarizes the published findings on the efficacy of MZB for renal disease including IgA nephropathy, lupus nephritis, and NS, as well as of oral MZB pulse therapy for severe lupus nephritis and NS, and also the mechanism of the effect of oral MZB pulse therapy on the lymphocyte cell cycle.

Background. There have been few controlled studies of combined therapy with multiple drugs, including immunosuppressives, for severe Henoch-Schoenlein nephritis (HSPN). We evaluated the efficacy of methylprednisolone and urokinase pulse therapy combined with cyclophosphamide for patients with HSPN of at least grade IVb. Methods. We studied 37 patients who had been diagnosed with HSPN of at least grade IVb. Of them, 20 (Group A) were treated with methylprednisolone and urokinase pulse therapy, and 17 (Group B) were treated with methylprednisolone and urokinase pulse therapy combined with cyclophophamide. We analysed the clinical features, laboratory and pathological findings of the two groups retrospectively. Results. After 6 months of treatment, mean urinary protein excretion in Group B had significantly decreased compared with Group A, and the activity index of both groups at the second biopsy was lower than that at the first. Furthermore, at the second biopsy, the chronicity index of Group B was lower than that of Group A. Four patients of Group A but none of Group B had persistent nephropathy (P<0.05). Conclusions. Our study suggests that methylprednisolone and urokinase pulse therapy combined with cyclophosphamide is useful for patients with severe HSPN.

Objective. To evaluate the efficacy of methylprednisolone and urokinase pulse therapy (MUPT) for severe Henoch-Schönlein nephritis, we examined the clinical manifestation and prognosis of patients with MUPT on long-term observation. Methods. We enrolled 56 patients with Henoch-schönlein nephritis who had been diagnosed with at least type IIIb from 1980 to 1998 on long-term observation and had been treated with MUPT. The clinical features, laboratory data, and pathologic findings between “pre-MUPT” and “post-MUPT,” and the prognosis of these patients on long-term observation were retrospectively investigated. Results. The mean urinary protein excretion after 6 months of treatment had decreased significantly compared with “pre-MUPT.” Hypercoagulant state in “after the completion of urokinase pulse therapy” improved compared with “pre-MUPT.” First renal biopsies were performed in all patients and second biopsies were performed in 27 patients. The activity index decreased significantly from 4.1 ± 1.9 at first biopsy to 2.5 ± 1.7 at second biopsy, while the chronicity index did not differ between first and second biopsy. None had renal insufficiency and renal survival rate was 100% for the decade. Conclusions. Although uncontrolled, our study suggested that MUPT is effective for those patients with the risk of progression of their nephropathy, especially if started early during the course of the disease before the crescents become fibrous.