Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA.
Division of Rheumatology, Department of Medicine, University of California, San Francisco, CA, USA.
Clin Rheumatol. 2018 Sep;37(9):2429-2437. doi: 10.1007/s10067-018-4148-5. Epub 2018 May 26.
Two major complications in scleroderma patients that cause substantial morbidity and mortality are ischemic digital lesions (DL) and pulmonary hypertension (PH). The clinician's ability to predict which patients will develop these complications is imperfect. We conducted a prospective observational cohort study of 300 patients with scleroderma who were followed for at least a 5-year period. At baseline, patients lacked evidence of PH and were without a current DL. At each 6-month visit, the patient was examined for signs/symptoms of PH and/or a DL. The primary outcomes were (1) PH defined as a mean pulmonary artery pressure ≥ 25 mmHg by right heart catheterization and (2) ≥ 1 DL defined as new onset of severe vascular compromise. Thirty patients (10%) developed PH (11 group 1/PAH, 4 group II, 15 group III) and 69 developed DL. The average time from enrollment until diagnosis of PH was 3.2 ± 2 years. In multivariable analyses, patients who developed PH were more likely to have diffuse disease (HR 3.2, p = 0.004), a forced vital capacity (FVC)/diffusing capacity of the lungs for carbon monoxide (DLCO) ratio > 1.6 (HR 1.7, p = 0.008), and elevated RVSP (HR = 1.07, p = 0.007). Patients who developed PAH were more likely to have a FVC/DLCO ratio > 1.6 (HR = 5.8, p = 0.014), and patients who developed group III PH were less likely to have an elevated FVC (HR = 0.92, p = 0.001). Patients were more likely to develop a DL if they had a history of prior DL (HR = 7.0, p < 0.001), or were men (HR = 2.3, p = 0.007). In a prevalent cohort of scleroderma patients, individuals who develop PH or DL have simple to measure clinical features that can predict these complications years before they occur.
在硬皮病患者中，两种主要的并发症会导致严重的发病率和死亡率，分别是缺血性指端病变（DL）和肺动脉高压（PH）。临床医生预测哪些患者会出现这些并发症的能力并不完善。我们对 300 名硬皮病患者进行了前瞻性观察队列研究，这些患者的随访时间至少为 5 年。在基线时，患者没有 PH 的证据，也没有当前的 DL。在每 6 个月的就诊时，对患者进行 PH 和/或 DL 的体征/症状检查。主要结局是（1）通过右心导管检查定义为平均肺动脉压≥25mmHg 的 PH；（2）≥1 个 DL 定义为新出现严重血管功能障碍。30 名患者（10%）出现 PH（11 名 1 组/特发性肺动脉高压，4 名 2 组，15 名 3 组），69 名出现 DL。从入组到诊断 PH 的平均时间为 3.2±2 年。多变量分析显示，发生 PH 的患者更有可能患有弥漫性疾病（HR 3.2，p=0.004）、用力肺活量（FVC）/一氧化碳弥散量（DLCO）比值>1.6（HR 1.7，p=0.008）和 RVSP 升高（HR=1.07，p=0.007）。发生特发性肺动脉高压的患者更有可能出现 FVC/DLCO 比值>1.6（HR=5.8，p=0.014），而发生 3 组 PH 的患者 FVC 升高的可能性较小（HR=0.92，p=0.001）。如果患者有先前 DL 的病史（HR=7.0，p<0.001）或为男性（HR=2.3，p=0.007），则更有可能发生 DL。在硬皮病患者的现患队列中，发生 PH 或 DL 的个体具有简单的临床特征，可以在这些并发症发生前数年预测这些并发症。
