Roccatello D, Rossi D, Giachino O, Bazzan M, Mazzucco G
Centro di Ricerche di Immunopatologia e Documentazione su Malattie Rare , Struttura Complessa a Direzione Universitaria di Immunologia Clinica, Ospedale San Giovanni Bosco, ASL 4, Torino.
G Ital Nefrol. 2007 Mar-Apr;24(2):111-20.
The diagnosis of antiphospholipid syndrome (APS) relies on clinical and laboratory criteria, which have been recently outlined in specific consensus conferences. Renal involvement in APS is not infrequent and includes different clinical patterns. For clinical purposes a distinction can be made between large vessel and microvascular involvement. Renal artery stenosis is frequent in APS. In case of microvascular involvement with an acute clinical course a differential diagnosis with other thrombotic microangiopathic diseases has to be made, taking in account thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, malignant hypertension, drug nephrotoxicity (cyclosporin) and others. The disease is often chronic, with hypertension, different degrees of renal insufficiency and mild proteinuria. In patients with systemic lupus erythematosus and antiphospholipid antibodies the prognosis of kidney disease is generally poorer than in lupus alone. Finally, the kidney is almost invariably a target in catastrophic antiphospholipid syndrome. Anticoagulation is the therapy of choice, especially in arterial stenosis and acute disease, but is probably also indicated in chronic and subacute patterns. The role of immunomodulatory therapy has to be assessed.
抗磷脂综合征(APS)的诊断依赖于临床和实验室标准,这些标准最近在特定的共识会议中已有概述。APS患者出现肾脏受累的情况并不少见,且包括不同的临床类型。从临床角度来看,可区分大血管受累和微血管受累。APS患者常出现肾动脉狭窄。如果微血管受累且临床过程呈急性,则必须与其他血栓性微血管病进行鉴别诊断,需考虑血栓性血小板减少性紫癜、溶血尿毒综合征、恶性高血压、药物性肾毒性(环孢素)等。该疾病通常为慢性,伴有高血压、不同程度的肾功能不全和轻度蛋白尿。对于患有系统性红斑狼疮和抗磷脂抗体的患者,肾脏疾病的预后通常比单纯狼疮患者更差。最后,在灾难性抗磷脂综合征中,肾脏几乎总是受累靶点。抗凝治疗是首选治疗方法,尤其适用于动脉狭窄和急性疾病,但在慢性和亚急性类型中可能也有必要使用。免疫调节治疗的作用有待评估。