Fujinaga Shuichiro, Ohtomo Yoshiyuki, Umino Daisuke, Mochizuki Hiroshi, Murakami Hitohiko, Shimizu Toshiaki, Yamashiro Yuichiro, Kaneko Kazunari
Division of Nephrology, Saitama Children's Medical Center, 2100 Magome, Iwatsuki-ku, Saitama-city Saitama, 339 8551, Japan.
Pediatr Nephrol. 2007 Jun;22(6):899-902. doi: 10.1007/s00467-006-0428-4. Epub 2007 Feb 7.
Although diffuse crescentic formation in immunoglobulin A (IgA) nephropathy, histologically characterized by extensive extracapillary proliferation, is assumed to have a poor prognosis, there has still been no established treatment because of the low prevalence of the condition, especially in pediatric patients. This paper reports on a 5-year-old boy with rapidly progressive IgA nephropathy requiring dialysis for 1 month. He had been treated with plasma exchange (PE) combined with immunosuppressive treatment, including steroids and mizoribine, because renal function deteriorated rapidly despite initial treatment with intravenous methylprednisolone pulse. The histological findings at that time revealed IgA nephropathy, with large circumferential cellular crescent formation in approximately 80% of the glomeruli. Three weeks after PE initiation, serum levels of creatinine and IgA-containing immune complexes returned to normal, and urinary protein excretion gradually decreased. The second renal biopsy taken 7 months later demonstrated mild IgA nephropathy with small fibrocellular crescents. This case report indicates that PE combined with immunosuppressive treatment may benefit children with rapidly progressive IgA nephropathy, even when extensive crescent formations are present.
尽管免疫球蛋白A（IgA）肾病中的弥漫性新月体形成，其组织学特征为广泛的毛细血管外增生，被认为预后不良，但由于该病症的发病率较低，尤其是在儿科患者中，目前仍没有既定的治疗方法。本文报道了一名5岁男孩，患有快速进展性IgA肾病，需要透析1个月。尽管最初采用静脉注射甲泼尼龙脉冲治疗，但他的肾功能仍迅速恶化，因此接受了血浆置换（PE）联合免疫抑制治疗，包括使用类固醇和咪唑立宾。当时的组织学检查结果显示为IgA肾病，约80%的肾小球出现大的环状细胞新月体形成。开始血浆置换三周后，血清肌酐水平和含IgA的免疫复合物恢复正常，尿蛋白排泄逐渐减少。7个月后进行的第二次肾活检显示为轻度IgA肾病，伴有小的纤维细胞新月体。本病例报告表明，即使存在广泛的新月体形成，血浆置换联合免疫抑制治疗可能对患有快速进展性IgA肾病的儿童有益。
