Division of Nephrology, Icahn School of Medicine at Mount Sinai, New York, NY.
Memorial Sloan Kettering Cancer Center, Renal Service, New York, NY; Department of Medicine, Weill Cornell Medical College, New York, NY.
Adv Kidney Dis Health. 2024 Jul;31(4):326-333. doi: 10.1053/j.akdh.2024.03.003.
Immunotactoid glomerulopathy (ITG) is a rare glomerular disease that typically presents with proteinuria, hematuria, and kidney dysfunction. A kidney biopsy is essential to establish the diagnosis of ITG. ITG is characterized by glomerular electron-dense immunoglobulin deposits with hollow-cored microtubules. ITG is classified as either monoclonal or polyclonal based on immunofluorescence staining of the immunoglobulin deposits. Monoclonal ITG is associated with an underlying hematologic disorder in two-thirds of the cases, lymphoma and plasma cell dyscrasias being the most common. Polyclonal ITG is associated with autoimmune diseases but can be seen with hematologic disorders and chronic infections. Due to the preponderance of hematologic disorders in both monoclonal and polyclonal ITG, a thorough hematologic workup must be performed in all cases of ITG. In monoclonal ITG with a detectable clone, clone-directed therapy is administered to achieve hematologic remission, as the renal response is highly dependent on the hematologic response. In clone-negative monoclonal ITG, anti-B cell therapy is often used as a first-line therapy. Management of polyclonal ITG without an underlying hematologic disorder is poorly defined. Compared to monoclonal ITG, patients with polyclonal ITG have a higher risk of progression to end-stage kidney disease. Recurrence of ITG following kidney transplantation is common and is often associated with hematologic relapse.
免疫触须样肾小球病 (ITG) 是一种罕见的肾小球疾病，通常表现为蛋白尿、血尿和肾功能障碍。肾脏活检对于确立 ITG 的诊断至关重要。ITG 的特征是肾小球电子致密免疫球蛋白沉积伴空心微管。根据免疫球蛋白沉积的免疫荧光染色，ITG 可分为单克隆或多克隆。三分之二的单克隆 ITG 与潜在的血液系统疾病相关，其中淋巴瘤和浆细胞异常增生症最为常见。多克隆 ITG 与自身免疫性疾病相关，但也可见于血液系统疾病和慢性感染。由于单克隆和多克隆 ITG 中血液系统疾病的优势，所有 ITG 病例均必须进行彻底的血液学检查。在可检测到克隆的单克隆 ITG 中，给予针对克隆的治疗以实现血液学缓解，因为肾脏反应高度依赖于血液学反应。在克隆阴性的单克隆 ITG 中，通常使用抗 B 细胞治疗作为一线治疗。无潜在血液系统疾病的多克隆 ITG 的管理尚未明确。与单克隆 ITG 相比，多克隆 ITG 患者进展为终末期肾病的风险更高。肾移植后 ITG 的复发很常见，常伴有血液学复发。
