Meune Christophe, Avouac Jérôme, Airò Paolo, Beretta Lorenzo, Dieudé Philippe, Wahbi Karim, Caramaschi Paola, Tiev Kiet, Cappelli Susanna, Diot Elisabeth, Vacca Alessandra, Cracowski Jean-Luc, Sibilia Jean, Kahan André, Matucci-Cerinic Marco, Allanore Yannick
Paris Descartes University and Department of Rheumatology A, Cochin Hospital, AP-HP, Paris, France.
Arthritis Rheum. 2011 Sep;63(9):2790-6. doi: 10.1002/art.30432.
To develop a score to estimate the risk of developing pulmonary hypertension (PH) in patients with systemic sclerosis (SSc).
We first examined the prevalence and characteristics of precapillary PH confirmed by right-heart catheterization in a cross-sectional (derivation) sample of 1,165 SSc patients, and we developed a risk prediction score (RPS) based on simple clinical observations associated with PH. We next prospectively tested the 3-year predictive power of the "Cochin RPS" in a separate (validation) sample of 443 patients presenting with PH-free SSc at baseline.
In the derivation sample, age, forced vital capacity, and diffusing capacity for carbon monoxide/alveolar volume were independently associated with the presence of PH and were used to create the Cochin RPS. PH developed during followup in 20 patients in the validation sample. The area under the receiver operating characteristic curve of the Cochin RPS was 0.87 (95% confidence interval 0.79-0.95). With a cutoff value of 2.73, patients at risk of PH during followup could be identified with 89.5% sensitivity and 74.1% specificity. PH occurred in 0.6% of patients in the lowest 2 quintiles of the Cochin RPS, in 1.7% of patients in the third and fourth quintiles, and in 17.1% of patients in the highest quintile (P<0.0001 by log rank test). Patients in the highest quintile incurred a >35-fold higher risk of developing PH compared with patients in the 2 lowest quintiles (P=0.001).
Using routine clinical observations, we developed a simple score that accurately predicted the risk of PH in SSc.
制定一个评分系统，以评估系统性硬化症（SSc）患者发生肺动脉高压（PH）的风险。
我们首先在1165例SSc患者的横断面（推导）样本中，研究经右心导管检查确诊的毛细血管前性PH的患病率和特征，并基于与PH相关的简单临床观察结果制定了风险预测评分（RPS）。接下来，我们在另一个（验证）样本中对443例基线时无PH的SSc患者进行前瞻性测试，以检验“科钦RPS”的3年预测能力。
在推导样本中，年龄、用力肺活量以及一氧化碳弥散量/肺泡容积与PH的存在独立相关，并用于创建科钦RPS。在验证样本的随访期间，有20例患者发生了PH。科钦RPS的受试者工作特征曲线下面积为0.87（95%置信区间0.79 - 0.95）。截断值为2.73时，随访期间有PH风险的患者可被识别，灵敏度为89.5%，特异度为74.1%。在科钦RPS最低的两个五分位数组中，0.6%的患者发生了PH；在第三和第四五分位数组中，1.7%的患者发生了PH；在最高五分位数组中，17.1%的患者发生了PH（对数秩检验P<0.0001）。与最低的两个五分位数组中的患者相比，最高五分位数组中的患者发生PH的风险高35倍以上（P = 0.001）。
通过常规临床观察，我们制定了一个简单的评分系统，能够准确预测SSc患者发生PH的风险。
