Division of Renal electrolyte and hypertension, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Nephron. 2021;145(1):85-90. doi: 10.1159/000510759. Epub 2020 Oct 15.
Collapsing glomerulopathy has multiple associations, including viral infections, medications like bisphosphonates and interferon, autoimmune diseases, and genetic predisposition. We report a case of collapsing focal segmental glomerulosclerosis associated with persistently high levels of interferon gamma produced by T-cell receptor αβ (+), CD4- CD8- (double negative) T lymphocytes that progressed despite treatment and improvement of other cytokine levels. Double negative T cells are elevated and activated in autoimmune lymphoproliferative syndrome (ALPS). Production of elevated interferon gamma levels from double negative T cells in ALPS despite treatment provides insight to the pathophysiology of collapsing glomerulopathy, guiding future research for collapsing glomerulopathy.
局灶节段性肾小球硬化症与多种疾病相关，包括病毒感染、双膦酸盐和干扰素等药物、自身免疫性疾病和遗传易感性等。我们报告了一例与 T 细胞受体 αβ（+）、CD4-CD8-（双阴性）T 淋巴细胞持续产生高水平干扰素γ相关的局灶节段性肾小球硬化症，尽管进行了治疗，但其他细胞因子水平有所改善，病情仍在进展。双阴性 T 细胞在自身免疫性淋巴增生综合征（ALPS）中升高并被激活。尽管进行了治疗，ALPS 中的双阴性 T 细胞仍会产生高水平的干扰素γ，这为局灶节段性肾小球硬化症的病理生理学提供了新的见解，并为局灶节段性肾小球硬化症的未来研究提供了指导。
