Ruiz-Irastorza Guillermo, Egurbide Maria-Victoria, Ugalde Jon, Aguirre Ciriaco
Servicio de Medicina Interna, Hospital de Cruces, Universidad del Pais Vasco/Euskal Herriko Unibertsitatea, Bizkaia, Spain.
Arch Intern Med. 2004 Jan 12;164(1):77-82. doi: 10.1001/archinte.164.1.77.
Thrombosis is a frequent cause of morbidity and death in patients with systemic lupus erythematosus (SLE). Whether antiphospholipid syndrome (APS) is the cause of increased irreversible organ damage and mortality in lupus patients is not well established.
Prospective inception cohort of 202 patients with SLE (American College of Rheumatology criteria). Antiphospholipid syndrome was defined according to the Sapporo criteria. Irreversible damage was measured using the Systemic Lupus International Collaborating Clinics-American College of Rheumatology damage index (SDI) at 6 months and 1, 3, 5, 10, 15, 20, and 25 years after the diagnosis of SLE. All deaths were documented.
A total of 88% of patients were women. Twenty-eight patients met criteria for definite APS. Mean (SD) follow-up was 9.7 (6.0) years. Nine patients could not be contacted for follow-up. All patients with APS experienced thrombosis, most of them in the arterial bed. Damage was more severe in patients with APS than in those without APS (median SDI score, 2 vs 0 at 5 years; P<.001; 4 vs 1 at 15 years; P<.001). Cumulative survival at 15 years was lower in patients with APS than in those without APS (65% vs 90%, P =.03). Older age at diagnosis, lupus nephritis, and APS were independent predictors of mortality.
Antiphospholipid syndrome with thrombotic manifestations is a major predictor of irreversible organ damage and death in patients with SLE.
血栓形成是系统性红斑狼疮（SLE）患者发病和死亡的常见原因。抗磷脂综合征（APS）是否是狼疮患者不可逆器官损伤增加和死亡率升高的原因尚未明确。
对202例符合美国风湿病学会标准的SLE患者进行前瞻性队列研究。根据札幌标准定义抗磷脂综合征。在SLE诊断后的6个月以及1、3、5、10、15、20和25年，使用系统性红斑狼疮国际协作临床中心-美国风湿病学会损伤指数（SDI）测量不可逆损伤。记录所有死亡情况。
共有88%的患者为女性。28例患者符合明确的APS标准。平均（标准差）随访时间为9.7（6.0）年。9例患者无法联系到进行随访。所有APS患者均发生血栓形成，其中大多数发生在动脉系统。APS患者的损伤比无APS患者更严重（5年时SDI评分中位数，2比0；P<0.001；15年时4比1；P<0.001）。APS患者15年时的累积生存率低于无APS患者（65%比90%，P=0.03）。诊断时年龄较大、狼疮性肾炎和APS是死亡率的独立预测因素。
伴有血栓形成表现的抗磷脂综合征是SLE患者不可逆器官损伤和死亡的主要预测因素。
