Pathology Department, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France; Sorbonne Universités, Université Pierre et Marie Curie, Paris, France; INSERM, UMR S 1155, Paris, France.
Nephrology Department, Hôpital Claude Huriez, Centre Hospitalier Régional Universitaire de Lille, Lille, France; University Lille Nord de France, Lille, France.
Kidney Int. 2016 Dec;90(6):1321-1331. doi: 10.1016/j.kint.2016.07.021. Epub 2016 Sep 17.
Thrombotic microangiopathy (TMA) is a poorly recognized cause of collapsing glomerulopathy. The frequency and significance of collapsing glomerulopathy associated with renal TMA have not been specifically studied in native kidney biopsy specimens. Here we retrospectively documented clinicopathologic features of 53 patients with histologically proven TMA in the native kidney, with special emphasis on changes due to focal segmental glomerulosclerosis (FSGS). Histological TMA was related to hypertensive nephropathy in 21 patients, genetic complement abnormalities in 9, drugs in 7, and to other causes in 16 patients. Almost half (26 patients) presented with arteriolar, 6 with glomerular, and 21 with mixed TMA. Using the Columbia classification system for the 53 patients with histological TMA, 33 had concurrent FSGS lesions with collapsing glomerulopathy the dominant variant in 19 patients (58% of the FSGS cases), not otherwise specified in 9 patients, cellular in 3, and perihilar or tip lesions in 1 patient each. The presence of FSGS was associated with a poor renal prognosis, with no prognostic difference between collapsing glomerulopathy and other FSGS variants. Thus, collapsing glomerulopathy is frequently found in native kidney biopsies with TMA, suggesting that endothelial injury may play an important role in the pathophysiology of FSGS.
血栓性微血管病(TMA)是一种未被充分认识的导致肾小球病塌陷的原因。在原发性肾活检标本中,尚未专门研究与肾 TMA 相关的塌陷性肾小球病的频率和意义。在这里,我们回顾性地记录了 53 例经组织学证实的原发性 TMA 患者的临床病理特征,特别强调了局灶节段性肾小球硬化(FSGS)引起的变化。组织学 TMA 与 21 例高血压性肾病、9 例遗传补体异常、7 例药物和 16 例其他原因有关。几乎一半(26 例)患者表现为小动脉性、6 例为肾小球性、21 例为混合性 TMA。在对 53 例组织学 TMA 患者进行哥伦比亚分类系统分析时,33 例同时存在 FSGS 病变,其中 19 例(FSGS 病例的 58%)以塌陷性肾小球病为主,9 例未另作说明,3 例为细胞性,1 例为近侧或尖端病变。FSGS 的存在与肾脏预后不良相关,塌陷性肾小球病与其他 FSGS 变异型之间无预后差异。因此,在 TMA 的原发性肾活检中经常发现塌陷性肾小球病,这表明内皮损伤可能在 FSGS 的病理生理学中发挥重要作用。