Mukai K, Kitazawa K, Totsuka D, Saito K, Sugisaki T
Department of Nephrology, Showa University School of Medicine, Tokyo, Japan.
Clin Nephrol. 1998 May;49(5):321-4.
A 57-year-old man with monoclonal gamma-globulinemia was admitted because of edema and proteinuria. A renal biopsy specimen showed lobular glomerulonephritis associated with deposition of material that was positive for IgG, C3, C1q, fibrin, kappa light chain, and lambda light chain but was not stained by Congo red. Glomeruli showed massive electron-dense deposits with two kinds of unusual, highly organized crystalline structures in the mesangial matrix and peripheral capillary loops. Clinically, the patient had nephrotic syndrome, microscopic hematuria, and hypertension. No Bence-Jones protein or cryoglobulin was found in the urine or serum. Immunoelectrophoresis of blood and urine revealed increased IgG-lambda paraprotein, but no free light chains were found. This case was not associated with amyloidosis, systemic lupus erythematosus, light chain deposition disease, cryoglobulinemia, or multiple myeloma. Immunotactoid glomerulopathy was diagnosed. Treatment with oral prednisone was effective for the management of nephrotic syndrome and renal dysfunction. Glomerular deposition of two kinds of microtubular structure in immunotactoid glomerulopathy has rarely been reported.
一名患有单克隆丙种球蛋白血症的57岁男性因水肿和蛋白尿入院。肾活检标本显示为小叶性肾小球肾炎,伴有IgG、C3、C1q、纤维蛋白、κ轻链和λ轻链阳性物质沉积,但刚果红染色阴性。肾小球显示大量电子致密沉积物,在系膜基质和外周毛细血管袢中有两种异常的、高度有组织的晶体结构。临床上,患者有肾病综合征、镜下血尿和高血压。尿液和血清中未发现本周氏蛋白或冷球蛋白。血液和尿液的免疫电泳显示IgG-λ副蛋白增加,但未发现游离轻链。该病例与淀粉样变性、系统性红斑狼疮、轻链沉积病、冷球蛋白血症或多发性骨髓瘤无关。诊断为免疫触须样肾小球病。口服泼尼松治疗对肾病综合征和肾功能不全的管理有效。免疫触须样肾小球病中两种微管结构的肾小球沉积鲜有报道。