Immunotactoid glomerulopathy: clinicopathologic and proteomic study
Immunotactoid glomerulopathy: clinicopathologic and proteomic study
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DOI：
10.1093/ndt/gfs348
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发表时间：
2012-11-01
影响因子：
6.1
通讯作者：
Sethi, Sanjeev
中科院分区：
文献类型：
--
作者：
Nasr, Samih H.;Fidler, Mary E.;Sethi, Sanjeev
免疫类肾小球病（ITG）是一种罕见的肾小球疾病。在这里，我们报告了ITG最大的临床病理系列，并定义了其蛋白质组学特征。1993年至2011年期间，从我们的病理档案中确定的16例ITG患者的特征。3例患者行激光显微切割和质谱（LMD/MS）检查，表现为蛋白尿100例，肾病综合征69例，肾功能不全50例，微量血尿80例。低补体血症46例，血清M峰63例。血液系统恶性肿瘤38例，其中慢性淋巴细胞白血病19例，淋巴浆细胞性淋巴瘤13例，骨髓瘤13例。肾小球损伤类型为膜增生性（56例）、膜性（31例）或增生性（13例）肾小球肾炎。微管沉积物是免疫球蛋白轻链限制性的，平均直径为31 nm（范围1752）。在12例患者平均48个月的随访中，50例缓解，33例持续肾功能不全，17例进展为终末期肾病。LMD/MS蛋白质组学分析显示存在免疫球蛋白、单型轻链、经典途径和终末途径的补体因子以及少量血清淀粉样蛋白P组分。ITG似乎比其他副蛋白相关性肾脏病变有更好的预后，预计有一半的患者可以通过免疫抑制治疗或化疗恢复肾功能。ITG的蛋白质组学特征与单型免疫球蛋白的沉积和补体经典和末端途径的激活一致。
Immunotactoid glomerulopathy (ITG) is a rare glomerular disease. Here, we report the largest clinicopathologic series of ITG and define its proteomic profile.The characteristics of 16 ITG patients who were identified from our pathology archives are provided between 1993 and 2011. We also performed laser microdissection and mass spectrometry (LMD/MS) in three cases.Presentation included proteinuria (100), nephrotic syndrome (69), renal insufficiency (50) and microhematuria (80). Hypocomplementemia was present in 46 and a serum M-spike in 63. Hematologic malignancy was present in 38, including chronic lymphocytic leukemia in 19, lymphoplasmacytic lymphoma in 13 and myeloma in 13. The pattern of glomerular injury was membranoproliferative (56), membranous (31) or proliferative (13) glomerulonephritis. The microtubular deposits were immunoglobulin light chain restricted in 69 and had a mean diameter of 31 nm (range 1752). During an average of 48 months of follow-up for 12 patients, 50 had remission, 33 had persistent renal dysfunction and 17 progressed to end-stage renal disease. Proteomic analysis by LMD/MS revealed the presence of immunoglobulins, monotypic light chains, complement factors of the classical and terminal pathway and small amount of serum amyloid P-component.Hematologic malignancy, particularly lymphoma, is not uncommon in ITG. ITG appears to have a better prognosis than other paraprotein-related renal lesions, with a half of patients expected to recover kidney function with immunosuppressive therapy or chemotherapy. The proteomic profile of ITG is consistent with deposition of monotypic immunoglobulins and activation of the classical and terminal pathway of complement.