Fukuda M, Morozumi K, Oikawa T, Motokawa M, Usami T, Yoshida A, Kimura G
Department of Internal Medicine and Pathophysiology, Nagoya City University Graduate School of Medical Sciences, Japan.
Clin Nephrol. 2005 May;63(5):368-74. doi: 10.5414/cnp63368.
We present the case of a 69-year-old man with nephrotic syndrome and renal insufficiency, who developed lobular glomerulonephritis. An electron microscopy examination of a renal biopsy showed microtubular structures of 24 nm in diameter in the subendothelial space and the paramesangial area. These deposits were PAS-positive and Congo red-negative, and revealed predominantly positive staining for kappa light chain. There was no evidence of diseases with highly organized glomerular deposits, such as amyloidosis, cryoglobulinemia, systemic lupus erythematosus or paraproteinemia. Therefore, the patient was diagnosed to have immunotactoid glomerulopathy (ITG). During a seven-year course he has not developed any disease known to be associated with organized glomerular immune deposits. Hence, we believe ITG occurred as a primary glomerular disease in this case. We also highlight cases of ITG with microtubular deposits that have been reported in Japan, compare these cases to previous reports, and show that the characteristics of the Japanese cases are male predominance; a high incidence of membranoproliferative glomerulonephritis (MPGN); a low incidence of monoclonal gammopathy and hematological malignancies and a higher incidence of hypocomplementemia.
我们报告一例69岁患有肾病综合征和肾功能不全的男性患者，其发生了小叶性肾小球肾炎。肾活检的电子显微镜检查显示，在内皮下间隙和系膜旁区有直径为24nm的微管结构。这些沉积物PAS染色阳性、刚果红染色阴性，且主要显示κ轻链染色阳性。没有证据表明存在具有高度组织化肾小球沉积物的疾病，如淀粉样变性、冷球蛋白血症、系统性红斑狼疮或副蛋白血症。因此，该患者被诊断为免疫触须样肾小球病（ITG）。在七年的病程中，他未发生任何已知与有组织化肾小球免疫沉积物相关的疾病。因此，我们认为在该病例中ITG是作为一种原发性肾小球疾病发生的。我们还重点介绍了日本报道的伴有微管沉积物的ITG病例，将这些病例与既往报道进行比较，并表明日本病例的特点是男性居多；膜增生性肾小球肾炎（MPGN）发病率高；单克隆丙种球蛋白病和血液系统恶性肿瘤发病率低以及低补体血症发病率高。
