Daugas Eric, Nochy Dominique, Huong Du Le Thi, Duhaut Pierre, Beaufils Hélène, Caudwell Valérie, Bariety Jean, Piette Jean-Charles, Hill Gary
*Service d'Anatomie Pathologique, Service de Néphrologie et Institut National de la Santé et de la Recherche Médicale (INSERM) U430, Hôpital Broussais, Paris, France; Service de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière, Paris, France; and INSERM U423, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
J Am Soc Nephrol. 2002 Jan;13(1):42-52. doi: 10.1681/ASN.V13142.
In the course of the antiphospholipid syndrome (APS), the existence of vaso-occlusive lesions capable of affecting numerous organs is now well established. The renal involvement attributable to primary APS, APS nephropathy (APSN), corresponds to vaso-occlusive lesions of the intrarenal vessels, associating side-by-side, acute thromboses with chronic arterial and arteriolar lesions, leading to zones of cortical ischemic atrophy. A retrospective study of 114 lupus patients undergoing renal biopsy was undertaken to determine the following: (1) if APSN can be found in the course of systemic lupus erythematosus (SLE); (2) if certain clinical and biologic factors can permit the prediction of the presence of APSN; and (3) if APSN is a superadded renal morbidity factor in lupus patients. This study shows the following: (1) APSN occurs in SLE (32% of patients with renal biopsies) in addition to, and independently of, lupus nephritis; (2) APSN is statistically associated with lupus anticoagulant but not with anticardiolipin antibodies; (3) APSN is associated with extrarenal APS, mainly arterial thromboses and obstetrical fetal loss, but not with the venous thromboses of APS; (4) APSN is an independent risk factor, over and above lupus nephritis, that contributes to an elevated prevalence of hypertension, elevated serum creatinine, and increased interstitial fibrosis. Thus, it seems likely that, because of its associations with hypertension, elevated serum creatinine, and increased interstitial fibrosis, APSN may worsen the prognosis in these patients. APSN may also have therapeutic significance in that its recognition should permit a better balance between immunosuppressor and antithrombotic and/or vasoprotective therapy. Finally, this study suggests that APSN should be considered as an element to be included in the classification criteria of APS.
在抗磷脂综合征(APS)病程中,能够影响多个器官的血管闭塞性病变的存在现已得到充分证实。原发性APS所致的肾脏受累,即APS肾病(APSN),对应于肾内血管的血管闭塞性病变,急性血栓形成与慢性动脉和小动脉病变并存,导致皮质缺血性萎缩区域。对114例接受肾活检的狼疮患者进行了一项回顾性研究,以确定以下几点:(1)在系统性红斑狼疮(SLE)病程中是否能发现APSN;(2)某些临床和生物学因素是否能预测APSN的存在;(3)APSN是否是狼疮患者额外的肾脏发病因素。这项研究表明:(1)APSN除狼疮肾炎外且与之无关地发生在SLE中(肾活检患者中有32%);(2)APSN在统计学上与狼疮抗凝物相关,但与抗心磷脂抗体无关;(3)APSN与肾外APS相关,主要是动脉血栓形成和产科胎儿丢失,但与APS的静脉血栓形成无关;(4)APSN是一个独立的危险因素,除狼疮肾炎外,它导致高血压患病率升高、血清肌酐升高和间质纤维化增加。因此,由于其与高血压、血清肌酐升高和间质纤维化增加的关联,APSN似乎可能使这些患者的预后恶化。APSN在治疗上也可能具有重要意义,因为对其的认识应能使免疫抑制剂与抗血栓和/或血管保护治疗之间达到更好的平衡。最后,这项研究表明APSN应被视为APS分类标准中应纳入的一个要素。