Suzuki Toshiaki, Okubo Soichiro, Ikezumi Yohei, Ueno Mitsuhiro, Nishi Shinichi, Saito Kazuhide, Takahashi Kouta, Uchiyama Makoto
Division of Pediatrics, Niigata University Graduate School of Medicine and Dental Sciences, Niigata, Japan.
Nihon Jinzo Gakkai Shi. 2004;46(4):360-4.
Collagenofibrotic glomerulopathy is a recently recognized entity that is characterized by massive accumulation of collagen fibrils in the mesangial and subendothelial areas, and an elevated serum level of procollagen III peptide (PIIIP). We report the first case of a collagenofibrotic glomerulopathy patient who received a kidney transplantation. She received the kidney transplantation at the age of 18 years and the post-operative course was uneventful with good renal function on immunosuppression, which consisted of methylprednisolone, tacrolimus and basiliximab. Although urinary protein was negative, the serum level of PIIIP gradually elevated which suggests new collagen production in the graft. These findings indicate that the recipient had a systemic factor that stimulated collagen production. To investigate the prognosis of collagenofibrotic glomerulopathy, we carried out a questionnaire survey on 14 patients at 9 hospitals. None of these patients had received a kidney transplantation. However, 7 already had end-stage renal failure. Ten years after diagnosis, the renal survival rate was 49%. This rate is lower than cited in previous reports.
胶原纤维性肾小球病是一种最近才被认识的疾病，其特征是系膜和内皮下区域大量胶原纤维堆积，血清Ⅲ型前胶原肽（PIIIP）水平升高。我们报告了首例接受肾移植的胶原纤维性肾小球病患者。她18岁时接受了肾移植，术后过程顺利，在使用甲基强的松龙、他克莫司和巴利昔单抗进行免疫抑制治疗的情况下肾功能良好。尽管尿蛋白为阴性，但血清PIIIP水平逐渐升高，这表明移植肾中有新的胶原生成。这些发现表明受者存在刺激胶原生成的全身因素。为了研究胶原纤维性肾小球病的预后，我们对9家医院的14例患者进行了问卷调查。这些患者均未接受肾移植。然而，其中7例已处于终末期肾衰竭。诊断后10年，肾脏存活率为49%。该比率低于先前报告中的数据。
