间断环磷酰胺冲击治疗难治性紫癜性肾炎的疗效观察

CYCLOPHOSPHAMIDE PULSE THERAPY (IV CTX) IN PATIENTS WITH STEROID RESISTANT HENOCH SCHNLEIN NEPHRITIS (HSN)

目的：观察间断环磷酰胺冲击（ＩＶ－ＣＴＸ）治疗顽固性紫癜性肾炎（ＨＳＮ）的近期疗效。方法：对经过激素治疗无效，仍有明显蛋白尿（＞２ｇ／２４ｈ）及血尿，肾组织有明显活动性病变（袢坏死或（和）新月体形成）的９例ＨＳＮ患者进行ＩＶ－ＣＴＸ治疗。ＣＴＸ剂量按０．５～１．０ｇ／ｍ２。最初每月给药（＜６次），尿蛋白转阴后每３个月给药一次。随访１２～５６个月。结果：治疗后８例尿蛋白转阴，７例血尿消失，１例患者在肾功能不全者治疗后肾功能恢复正常。１例患者在治疗１６个月后重复肾活检，肾小球病变及ＣＤ４＋，ＣＤ８＋及ＣＤ６８＋细胞浸润明显减轻。结论：ＩＶ－ＣＴＸ治疗顽固性ＨＳＮ的近期疗效令人满意，但远期疗效有待进一步临床验证。

In this study, we prospectively observed the effect of IV CTX in patients with HSN who were resistant to steroid therapy. METHODOLOGY Nine HSN patients (average age 13±7.5y) with haematuria and proteinuria greater than 2.0 g/d were enrolled into this study. Renal biopsies revealed active lesions in the glomeruli and tubulo interstitium in all cases. IV CTX (0.5～1.0 g/m 2) was given every month (m) for the first3～6 m, then changed to every 3 m, combined with small doses of prednisone ( 0.5 mg/kg·d -1 ). All patients were treated for 12～16 m and followed up for at least 12 m (12～56 m, average 18.6 m). RESULTS It was found that all patients responded well to IV CTX at the total dosage of 3.0～7.2 g. Proteinuria disappeared in 8 patients within 6 m after the treatment. Gross haematuria, which was present persistently in 4 patients, disappeared quickly after IV CTX. At the end of observation, proteinuria was present in one case and microscopic haematuria found in only 3 patients. Re biopsy was done in one case 16 m after treatment. Initially, there were segmental glomerular necrosis, glomerulo sclerosis (69.1 %) and marked cellular interstitial infiltration during first biopsy. These histological lesions improved dramatically in the second biopsy. CD4 +, CD8 + and CD68 + cell infiltration decreased from 108, 132, 124/mm 2[KG*2〗to 16, 16, 86/mm 2 respectively in the second biopsy. CONCLUSION IV CTX is effective in the treatment of refractory HSN.