讨论1例肾脏重链沉积病（IgG-γ1重链）。57岁男性，临床表现高血压、肾病综合征、大量蛋白尿、肾功能不全，病情进行性发展，血肌酐升至438µmol/L时，双肾超声体积仍不小。肾外损害不明显。肾活检光镜下肾小球呈“结节样病变”，刚果红染色阴性。免疫荧光见免疫球蛋白G（IgG）和IgG亚型IgG1（γ1重链单抗）沿肾小球毛细血管袢及肾小管基膜（TBM）线样沉积，IgA和IgM阴性。IgG2，IgG3，IgG4阴性。κ轻链和λ轻链染色阴性。电镜下见肾小球基膜内侧及TBM外侧电子致密物沉积。通过临床-实验室-病理结合，光学显微镜-免疫病理-电子显微镜结合，特别是针对性免疫病理检查，确诊为肾脏重链沉积病（IgG-γ1重链）。%A case of renal heavy chain deposition disease (IgG-γ1 heavy chain) was discussed in this article. A 57-year-old male presented with hypertension, nephrotic syndrome, massive proteinuria, and progressive renal dysfunction. When serum creatinine was 438µmol/L, ultrasound examination showed that bilateral kidneys were within normal range in size. In terms of histological examination, nodular glomerulopathy was identified under light microscopy with negative Congo red staining result. Immunofluorescent study showed that IgG and IgG1 were deposited along glomerular tufts and tubular basement membrane (TBM). Meanwhile, IgA, IgM, IgG2, IgG3, IgG4 and light chain staining was absent. Electron microscopy revealed high-density deposits inside glomerular basement membrane and outside TBM. Based on clinical, laboratory, and histological findings, the patient was finally diagnosed as renalγ1-heavy chain deposition disease.
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