Niznik Stanley, Rapoport Micha J, Avnery Orly, Kidon Mona, Shavit Ronen, Ellis Martin H, Agmon-Levin Nancy
Clinical Immunology, Angioedema and Allergy Institute, The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, Tel Aviv, Israel.
Department of Internal Medicine 'C', Shamir Medical Center, Zerifin, Israel.
Rheumatology (Oxford). 2024 May 2;63(5):1397-1402. doi: 10.1093/rheumatology/kead399.
APS-associated heart valve disease (HVD) is well described. Nonetheless, limited data exist on clinical parameters associated with the course of primary APS (pAPS) patients with HVD. The goal of this study was to assess clinical features and related outcomes in patients with APS-associated HVD.
In this multicentre retrospective study, we identified 33 pAPS patients with HVD (pAPS-HVD group) and compared their clinical course with 128 pAPS patients with normal heart valves on echocardiography (pAPS-control group).
pAPS-HVD patients had more cerebrovascular events (56.3% vs 25%, P = 0.005) and livedo reticularis (24.2% vs 7.8%, P = 0.013) than pAPS-controls. Furthermore, catastrophic-APS (CAPS) (12.1% vs 2.4%, P = 0.034), recurrent thrombosis (33.3% vs 4.7%, P < 0.001) and need for advanced therapy (i.e. IVIG, plasmapheresis or rituximab) were more frequent in pAPS-HVD patients. Anti-β2-glycoprotein 1 IgG (84.8% vs 63.2%, P = 0.034), anti-cardiolipin IgG (90.9% vs 64.8%, P = 0.005) and triple positive aPL (75.8% vs 56.5%, P = 0.047) were commoner in pAPS-HVD patients vs pAPS-controls. Ten of the 33 patients with pAPS-HVD underwent valve surgery, which was associated with male gender, smoking, arterial limb ischaemia and livedo reticularis.
pAPS-HVD patients had a more severe APS clinical course including CAPS and thrombotic events as well as a specific serology, namely IgG isotype aPL antibodies and triple positivity. Our data suggest that pAPS-HVD represents a high-risk subgroup of APS patients.
抗磷脂综合征（APS）相关心脏瓣膜病（HVD）已有充分描述。然而，关于原发性APS（pAPS）合并HVD患者病程相关的临床参数数据有限。本研究的目的是评估APS相关HVD患者的临床特征及相关结局。
在这项多中心回顾性研究中，我们确定了33例pAPS合并HVD患者（pAPS-HVD组），并将其临床病程与128例超声心动图显示心脏瓣膜正常的pAPS患者（pAPS对照组）进行比较。
与pAPS对照组相比，pAPS-HVD患者发生脑血管事件（56.3%对25%，P = 0.005）和网状青斑（24.2%对7.8%，P = 0.013）的情况更多。此外，pAPS-HVD患者发生灾难性APS（CAPS）（12.1%对2.4%，P = 0.034）、复发性血栓形成（33.3%对4.7%，P < 0.001）以及需要接受高级治疗（即静脉注射免疫球蛋白、血浆置换或利妥昔单抗）的情况更为频繁。与pAPS对照组相比，pAPS-HVD患者中抗β2糖蛋白1 IgG（84.8%对63.2%，P = 0.034）、抗心磷脂IgG（90.9%对64.8%，P = 0.005）和三联阳性抗磷脂抗体（aPL）（75.8%对56.5%，P = 0.047）更为常见。33例pAPS-HVD患者中有10例接受了瓣膜手术，这与男性、吸烟、肢体动脉缺血和网状青斑有关。
pAPS-HVD患者的APS临床病程更为严重，包括CAPS和血栓形成事件，以及特定的血清学表现，即IgG同种型aPL抗体和三联阳性。我们的数据表明，pAPS-HVD代表了APS患者中的一个高危亚组。
