Morrisroe K B, Stevens W, Nandurkar H, Prior D, Thakkar V, Roddy J, Zochling J, Sahhar J, Tymms K, Sturgess A, Major G, Kermeen F, Hill C, Walker J, Nash P, Gabbay E, Youssef P, Proudman S M, Nikpour M
Department of Rheumatology, St. Vincent's Hospital, Melbourne, Australia.
Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-133-7. Epub 2014 Feb 24.
To determine the prevalence and correlates of antiphospholipid antibodies (APLA) in systemic sclerosis (SSc).
Nine hundred and forty SSc patients were tested for APLA using an ELISA assay at recruitment. Clinical manifestations were defined as present, if ever present from SSc diagnosis. Logistic regression analysis was used to determine the associations of APLA.
One or more types of APLA were present in 226 (24.0%) patients. Anticardiolipin (ACA) IgG (ACA-IgG) antibodies were associated with right heart catheter-diagnosed pulmonary arterial hypertension (PAH), with higher titres corresponding with a higher likelihood of PAH (moderate titre (20-39 U/ml) ACA-IgG odds ratio [OR] 1.70, 95% CI: 1.01-2.93, p=0.047; high titre (>40 U/ml) ACA-IgG OR 4.60, 95% CI:1.02-20.8, p=0.047). Both ACA-IgM (OR 2.04, 95% CI: 1.4-3.0, p<0.0001) and ACA-IgG (OR 1.84, 95% CI: 1.2-2.8, p=0.005) were associated with interstitial lung disease (ILD). Increasing ACA-IgM and IgG titres were associated with increased likelihood of ILD. ACA-IgG was a marker of coexistent pulmonary hypertension and ILD (ILD-PH) (OR 2.10, 95% CI: 1.1-4.2, p=0.036). We also found an association between ACA-IgG and digital ulcers (OR 1.76, 95% CI: 1.16-2.67, p=0.008) and ACA-IgM and Raynaud's phenomenon (OR 2.39, 95% CI: 1.08-5.27, p=0.031). There was no association between APLA and SSc disease subtype, peak skin score, presence of other autoantibodies, mortality or other disease manifestations.
The association of APLA with PAH, ILD, ILD-PH, Raynaud's phenomenon and digital ulcers suggests that endothelial abnormalities and small vessel thrombosis may be important in the pathogenesis of these disease features.
确定系统性硬化症（SSc）中抗磷脂抗体（APLA）的患病率及其相关因素。
940例SSc患者在入组时采用酶联免疫吸附测定（ELISA）法检测APLA。临床表现定义为自SSc诊断起曾出现过即为存在。采用逻辑回归分析确定APLA的相关性。
226例（24.0%）患者存在一种或多种类型的APLA。抗心磷脂（ACA）IgG（ACA-IgG）抗体与经右心导管诊断的肺动脉高压（PAH）相关，滴度越高，PAH的可能性越高（中等滴度（20 - 39 U/ml）ACA-IgG比值比[OR] 1.70，95%可信区间：1.01 - 2.93，p = 0.047；高滴度（>40 U/ml）ACA-IgG OR 4.60，95%可信区间：1.02 - 20.8，p = 0.047）。ACA-IgM（OR 2.04，95%可信区间：1.4 - 3.0，p<0.0001）和ACA-IgG（OR 1.84，95%可信区间：1.2 - 2.8，p = 0.005）均与间质性肺疾病（ILD）相关。ACA-IgM和IgG滴度升高与ILD可能性增加相关。ACA-IgG是并存肺动脉高压和ILD（ILD-PH）的标志物（OR 2.10，95%可信区间：1.1 - 4.2，p = 0.036）。我们还发现ACA-IgG与指端溃疡相关（OR 1.76，95%可信区间：1.16 - 2.67，p = 0.008），ACA-IgM与雷诺现象相关（OR 2.39，95%可信区间：1.08 - 5.27，p = 0.031）。APLA与SSc疾病亚型、皮肤峰值评分、其他自身抗体的存在、死亡率或其他疾病表现之间无相关性。
APLA与PAH、ILD、ILD-PH、雷诺现象和指端溃疡之间的关联表明，内皮异常和小血管血栓形成可能在这些疾病特征的发病机制中起重要作用。
