Nagashima T, Hirata D, Yamamoto H, Okazaki H, Minota S
Division of Rheumatology and Clinical Immunology, Jichi Medical School, Tochigi, Japan.
Am J Kidney Dis. 2001 May;37(5):E38. doi: 10.1016/s0272-6386(05)90002-4.
A 17-year-old girl had been placed with ventriculoperitoneal, then ventriculoatrial shunts for congenital hydrocephalus since birth. The patient originally was diagnosed as having a lupus-like disease, but later turned out to have shunt nephritis, presenting with fever, proteinuria, pancytopenia, and hypocomplementemia. Antineutrophil cytoplasmic autoantibody specific for proteinase 3 (PR3-ANCA) was detected in her serum. The patient received oral prednisolone and repeated methylprednisolone pulses, with essentially no beneficial effects. A gram-positive coccus, Gemella morbillorum, was recovered from her blood as well as cerebrospinal fluid, and the culture of the shunt catheter established the diagnosis of shunt nephritis. Removal of the shunt catheter improved symptoms dramatically and decreased PR3-ANCA in serum to an undetectable level. Because steroids had no effects and the control of bacterial infection lowered PR3-ANCA levels, the antibody would have been induced by continuous infection with G morbillorum.
一名17岁女孩自出生起就因先天性脑积水接受了脑室腹腔分流术，之后又进行了脑室心房分流术。该患者最初被诊断为患有狼疮样疾病，但后来被证实患有分流性肾炎，表现为发热、蛋白尿、全血细胞减少和补体血症。在她的血清中检测到了针对蛋白酶3的抗中性粒细胞胞浆自身抗体（PR3-ANCA）。患者接受了口服泼尼松龙和多次甲泼尼龙冲击治疗，但基本没有效果。从她的血液和脑脊液中分离出一种革兰氏阳性球菌——麻疹孪生球菌，对分流导管进行培养后确诊为分流性肾炎。移除分流导管后症状显著改善，血清中的PR3-ANCA降至无法检测的水平。由于类固醇治疗无效，而控制细菌感染可降低PR3-ANCA水平，因此该抗体可能是由麻疹孪生球菌的持续感染诱导产生的。
