Néphrologie et Médecine Interne, Hôpital de Valenciennes, France.
Médecine Interne 2, French National Reference Center for SLE and APS, Hôpital Pitié-Salpêtrière, Paris, France.
J Autoimmun. 2022 Oct;132:102889. doi: 10.1016/j.jaut.2022.102889. Epub 2022 Aug 18.
Antiphospholipid syndrome (APS) nephropathy (APSN) is a rare pattern with specific features resulting from microvascular lesions. The prognosis of APSN, outside of lupus nephritis, is unknown. The aim of this study was to describe the renal, vascular and overall outcomes of patients with APSN.
Retrospective multicenter study of patients with antiphospholipid antibodies (aPL) associated with histological APSN lesions and no other nephropathy, identified through a national call for medical records. End-stage renal disease (ESRD)-free survival, thrombosis recurrence-free survival and overall survival were assessed.
Thirty patients were included (19 women) with a median age of 40 years (34-52 years). Fifteen patients had APS, 26/28 had lupus anticoagulant, and 15/26 had triple positivity for aPL. Median eGFR was 50 (31-60) mL/min/1.73 m. Glomerular thrombotic microangiopathy was found in 12/24 cases, fibrous intimal hyperplasia in 12/22 cases and focal cortical atrophy in 17/29 cases. Nineteen patients had moderate to severe interstitial fibrosis (>25%). Six patients developed ESRD at a median follow-up of 6.2 (1.8-9.1) years. The ESRD-free survival rates at 5 and 10 years were 80.0% (95% CI 57.6%-91.4%) and 72.7% (95% CI, 46.9%-87.4%) respectively. None of the histological factors considered was significantly associated with a decrease in eGFR at 12 months. Thrombosis recurrence-free survival was 77.8% (95% CI 48.2%-91.6%) at 10 years. Overall survival was 94% at 10 years (95% CI 65.0%-99.2%).
The renal prognosis of isolated APSN is poor. The severe fibrotic lesions observed are suggestive of late diagnosis.
抗磷脂综合征（APS）相关性肾病（APSN）是一种由微血管病变引起的罕见的具有特定特征的病理类型。除狼疮性肾炎外，APSN 的预后尚不清楚。本研究旨在描述 APSN 患者的肾脏、血管和总体预后。
通过全国范围内的病历征集，回顾性分析了伴有组织学 APSN 病变且无其他肾病的抗磷脂抗体（aPL）相关患者的多中心研究。评估了终末期肾病（ESRD）无进展生存率、血栓复发无进展生存率和总生存率。
共纳入 30 例患者（19 名女性），中位年龄为 40 岁（34-52 岁）。15 例患者患有 APS，26/28 例患者存在狼疮抗凝物，15/26 例患者 aPL 三项阳性。中位 eGFR 为 50（31-60）mL/min/1.73m。24 例中发现肾小球血栓性微血管病，22 例中发现纤维内膜增生，29 例中发现局灶性皮质萎缩。19 例患者中存在中重度间质纤维化（>25%）。中位随访 6.2（1.8-9.1）年后，有 6 例患者发展为 ESRD。5 年和 10 年 ESRD 无进展生存率分别为 80.0%（95%CI 57.6%-91.4%）和 72.7%（95%CI 46.9%-87.4%）。12 个月时，考虑的组织学因素均与 eGFR 下降无显著相关性。10 年时，血栓复发无进展生存率为 77.8%（95%CI 48.2%-91.6%）。10 年总体生存率为 94%（95%CI 65.0%-99.2%）。
孤立性 APSN 的肾脏预后较差。观察到的严重纤维化病变提示诊断较晚。
