Otsuka Tomoyuki, Sakai Yukinao, Ohno Dai, Tsuruoka Shuichi
Department of Nephrology, Nippon Medical School Musashi Kosugi Hospital.
J Nippon Med Sch. 2015;82(4):193-201. doi: 10.1272/jnms.82.193.
A 61-year-old man with bilateral purpura of the lower limbs and subsequent edema, was hospitalization after renal dysfunction developed. The presence of hepatitis C virus (HCV) RNA and cryoglobulin and the finding of membranoproliferative glomerulonephritis on renal biopsy led to a diagnosis of HCV-related glomerulonephritis due to cryoglobulinemia. Because of the pre-existence of nephrotic syndrome and the continuously increasing serum level of creatinine, treatment with cryofiltration, interferon, and steroids was started. After 5 cryofiltration sessions, the cryocrit level had decreased to 1% and the levels of serum creatinine and proteinuria had also decreased. However, 3 weeks after the start of treatment, nephrotic syndrome developed again and was accompanied by lower-extremity mononeuropathy and renal dysfunction. Thereafter, the patient showed disorientation, an affective disorder, and delirium, and his condition gradually deteriorated. Radiological examination of the head and examination of the cerebrospinal fluid showed no abnormalities. Despite the withdrawal of the interferon therapy and the reduction of the steroid dose, the patient's conditions remained unchanged, and the level of consciousness deteriorated. Although cryofiltration had beneficial effects and plasma exchange was continuously performed, the patient died on the 74th hospital day. Because of the significant changes due to ventilatory support and hemorrhage associated with disseminated intravascular coagulation, the autopsy findings did not allow us to definitively determine whether the symptoms had been caused by the HCV-related membranoproliferative glomerulonephritis or the interferon therapy or both. We have reported this case to provide insight into whether interferon therapy should be administered for HCV-related membranoproliferative glomerulonephritis with marked neurological symptoms due to cryoglobulinemia.
一名61岁男性，双下肢出现紫癜并继发水肿，在出现肾功能不全后住院治疗。肾活检发现丙型肝炎病毒（HCV）RNA、冷球蛋白以及膜增生性肾小球肾炎，诊断为冷球蛋白血症所致的HCV相关性肾小球肾炎。由于患者先前已存在肾病综合征且血清肌酐水平持续升高，遂开始进行冷滤过、干扰素及类固醇治疗。经过5次冷滤过治疗后，冷球蛋白水平降至1%，血清肌酐和蛋白尿水平也有所下降。然而，治疗开始3周后，肾病综合征再次出现，并伴有下肢单神经病和肾功能不全。此后，患者出现定向障碍、情感障碍和谵妄，病情逐渐恶化。头颅影像学检查和脑脊液检查均未发现异常。尽管停用了干扰素治疗并减少了类固醇剂量，但患者病情仍无改善，意识水平恶化。尽管冷滤过治疗有一定效果且持续进行了血浆置换，但患者在住院第74天死亡。由于通气支持及弥散性血管内凝血相关出血导致的显著变化，尸检结果无法明确确定这些症状是由HCV相关性膜增生性肾小球肾炎、干扰素治疗还是两者共同引起的。我们报告此病例，旨在探讨对于因冷球蛋白血症导致明显神经症状的HCV相关性膜增生性肾小球肾炎，是否应给予干扰素治疗。
