Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Mason F. Lord Building Center Tower, Suite 4100, Baltimore, MD 21224, USA.
J Rheumatol. 2010 Jan;37(1):98-104. doi: 10.3899/jrheum.090697. Epub 2009 Dec 1.
Clinical markers are needed to identify scleroderma patients at risk for pulmonary arterial hypertension (PAH) since early therapy may improve survival. We investigated whether increased numbers of telangiectases in scleroderma associate with measures of pulmonary vascular disease.
One hundred forty-seven consecutive adult patients with scleroderma were enrolled in this cross-sectional study and scored for the presence of matted telangiectases on 11 body areas. Per body area, telangiectases were scored as 0 if none were present, 1 if there were fewer than 10 telangiectases, and 2 if 10 or more telangiectases were counted. Linear regression analysis was performed to assess the association between right ventricular systolic pressure (RVSP) and telangiectasia score, adjusted for age, race, smoking status, scleroderma subtype, disease duration, and autoantibody status. Logistic regression analysis was performed with PAH by right-heart catheterization (RHC) as the dependent variable.
The mean telangiectasia score was 6.0 (SD 4.5, range 0-20). RVSP and telangiectasia score were positively correlated (r = 0.271, p = 0.001). The mean RVSP increased by 10.9 mm Hg for every 10-point increase in telangiectasia score (95% CI 3.6-18.3 mm Hg, p = 0.004), adjusted for potential confounders. The adjusted relative odds of PAH by RHC were 12.4 for patients with a 10-point increase in telangiectasia score (95% CI 1.78-85.9, p = 0.01).
Increased numbers of telangiectases strongly associate with the presence of pulmonary vascular disease. Telangiectases may be a clinical marker of more widespread aberrant microvascular disease in scleroderma.
需要临床标志物来识别硬皮病患者中肺动脉高压 (PAH) 的风险，因为早期治疗可能会改善生存率。我们研究了硬皮病患者中毛细血管扩张症的数量是否与肺血管疾病的测量值相关。
本横断面研究纳入了 147 例连续的成年硬皮病患者，并对 11 个身体区域的硬皮病患者的网状毛细血管扩张症进行评分。每个身体区域，如果没有毛细血管扩张症，则记为 0；如果少于 10 个毛细血管扩张症，则记为 1；如果有 10 个或更多的毛细血管扩张症，则记为 2。采用线性回归分析评估右心室收缩压（RVSP）与毛细血管扩张症评分之间的相关性，调整年龄、种族、吸烟状况、硬皮病亚型、疾病持续时间和自身抗体状态。采用逻辑回归分析以右心导管检查（RHC）为肺动脉高压（PAH）的因变量。
平均毛细血管扩张症评分为 6.0（SD 4.5，范围 0-20）。RVSP 和毛细血管扩张症评分呈正相关（r = 0.271，p = 0.001）。每增加 10 分的毛细血管扩张症评分，RVSP 增加 10.9mmHg（95%CI 3.6-18.3mmHg，p = 0.004），调整了潜在混杂因素。调整后的毛细血管扩张症评分每增加 10 分，RHC 诊断 PAH 的相对风险为 12.4（95%CI 1.78-85.9，p = 0.01）。
毛细血管扩张症的数量与肺血管疾病的存在密切相关。毛细血管扩张症可能是硬皮病中更广泛异常微血管疾病的临床标志物。
