系统性红斑狼疮并发继发性抗磷脂综合征肾损害11例临床病理分析

目的 分析系统性红斑狼疮（SLE）并发继发性抗磷脂综合征（APS）肾损害的临床病理表现，旨在提高对该类疾病的认识。 方法 回顾性分析北京协和医院2000年至2010年期间确诊SLE并发继发性APS（SLE伴APS）并行肾组织学检查的11例患者的资料，分析其临床病理特点，并比较其和SLE不伴APS患者在肾损害的临床病理及预后上的差异。 结果 11例SLE伴APS患者均有肾脏受累，突出表现为高血压（54.5%）、大量蛋白尿（≥3.5 g/d）（72.7%）和肾功能异常（45.5%）。SLE伴APS患者的舒张压、平均动脉压以及肾小球滤过率（eGFR）均明显高于SLE不伴APS患者（均P < 0.05）。8例（72.7%）SLE伴APS患者存在肾内血管的“血管闭塞性表现”，即符合抗磷脂综合征肾病（APSN）的病理表现，包括肾小血管、肾小球毛细血管血栓形成以及肾小动脉内膜增生、局灶性肾皮质萎缩、肾小管甲状腺样化，其中慢性APSN表现5例（45.5%），急性APSN表现4例（36.4%）（其中1例同时有急性和慢性表现）；其APSN的发生率以及急性APSN的发生率明显高于SLE不伴APS患者（P < 0.05）。 结论 SLE并发APS肾损害患者除狼疮肾炎外，多并发APSN，临床上高血压和肾功能异常更为突出。

Systemic lupus erythematosus with secondary antiphospholipid syndrome: a retrospective clinicopathological analysis of 11 cases

Objective To analyze the clinicopathological characteris tics of systemic lupus erythematosus (SLE) with secondary antiphospholid syndrome (APS) . Methods Data of 11 cases of SLE with secondary APS (SLE with APS) admitted to Peking Union Medical College Hospital from January 2000 to March 2010 were retrospectively analyzed. Kidney biopsy was performed on all of these patients. Differences of clinicopathology and outcomes between SLE with and without APS were compared. Results Renal involvement was found in all the SLE with APS patients. The prominent clinical manifestations included hypertension (54.5%), nephrotic level of proteinuria (24 h proteinuria ≥3.5 g)(72.7%) and renal insufficiency (45.5%). Diastolic blood pressure, mean arterial pressure and glomerular filtration rate in SLE with APS were significantly higher than those in SLE without APS (all P＜0.05). In 8 out of 11 cases (72.7%), APS nephropathy (APSN) in kidney biopsy was found, characterized by small vessel vaso-occlusive lesions. These included thrombotic microangiopathy (TMA), fibrous intimal hyperplasia (FIH), focal cortical atrophy (FCA) and tubular thyroidization. Among those, 5 cases (45.5%) had chronic APSN and 4 (36.4%) had acute APSN (one case had acute and chronic APSN at the same time). The incidences of APSN and acute APSN in the SLE with APS group were significantly higher than those in SLE without APS group (P＜0.05). Conclusions The major renal manifestations of SLE with APS are hypertension, nephrotic level of proteinuria and renal insufficiency. Other than lupus nephritis, also a high incidence of APSN is found in SLE with APS patients.