Servicio de Reumatología, Hospital Universitario12 de Octubre, Universidad Complutense de Madrid, Avda de Córdoba s/n, Madrid 28041, Spain.
Unidad Multidisciplinar de Hipertension Pulmonar, Servicio de Cardiologia, CIBER en enfermedades cardiovasculares, Hospital Universitario 12 de Octubre, Madrid, Spain.
Semin Arthritis Rheum. 2018 Jun;47(6):870-876. doi: 10.1016/j.semarthrit.2017.10.006. Epub 2017 Oct 13.
To analyze the performance of the 1980 ACR and new 2013 ACR/EULAR criteria for systemic sclerosis (SSc) in cutaneous SSc (lcSSc) patients, especially those affected by lcSSc and pulmonary arterial hypertension (PAH).
All patients with a clinical lcSSc diagnosis from a prospective observational SSc cohort were included. Sociodemographic and disease-related variables were collected, and PAH confirmed by right heart catheterization (RHC). Performance of the 2013 and 1980 SSc criteria was analyzed in terms of clinical diagnosis. Descriptive and between-group analyses were performed as to the fulfillment of criterion sets, including comparison of survival.
Overall, 321 patients were included, 63% of whom fulfilled the 1980 ACR and 93% the 2013 ACR/EULAR criteria. Agreement between both criteria sets proved poor (κ = 0.23). LcSSC patients fulfilling both criterion sets were significantly younger at diagnosis, whilst presenting organ involvement, calcinosis, fingertip digital ulcers, and pitting scars more frequently than those who met the 2013 criteria only. Patients who fulfilled the 2013 but not the 1980 criteria presented a higher degree of ACA positivity and PAH. Nearly 12% of patients developed PAH. Patients who did not meet the 1980 criteria were affected by a milder disease from but demonstrated higher pulmonary vascular resistance and lower cardiac index than those fulfilling both criterion sets. Whereas patients with PAH met the 2013 criteria, only 47% fulfilled the 1980 criteria. Regardless of criterion set fulfillment, high mortality was observed in PAH patients, with no significant between-patient difference based on criterion set.
The new 2013 ARC/EULAR criteria prove more accurate than the former 1980 ACR criteria in identifying and differentiating patients with lcSSc, especially those with associated PAH. Since PAH exhibits a better prognosis if treated early, all SSc patients should undergo PAH screening.
分析 1980 年 ACR 和新的 2013 年 ACR/EULAR 系统性硬化症（SSc）标准在局限性皮肤型 SSc（lcSSc）患者中的表现，特别是在合并肺动脉高压（PAH）的患者中。
纳入前瞻性观察性 SSc 队列中具有临床诊断的局限性 SSc 患者。收集患者的社会人口统计学和疾病相关变量，并通过右心导管检查（RHC）确认 PAH。分析 2013 年和 1980 年 SSc 标准在临床诊断方面的表现。进行了描述性和组间分析，以满足标准集的要求，包括生存比较。
共有 321 例患者被纳入研究，其中 63%符合 1980 年 ACR 标准，93%符合 2013 年 ACR/EULAR 标准。两个标准集之间的一致性较差（κ=0.23）。符合两个标准集的 lcSSc 患者在诊断时年龄明显较小，而与仅符合 2013 年标准的患者相比，更常出现器官受累、钙质沉着、指尖溃疡性溃疡和凹陷性疤痕。符合 2013 年但不符合 1980 年标准的患者具有更高的 ACA 阳性率和 PAH。近 12%的患者发生 PAH。不符合 1980 年标准的患者疾病较轻，但肺血管阻力较高，心指数较低，与符合两个标准集的患者相比。虽然有 PAH 的患者符合 2013 年标准，但只有 47%符合 1980 年标准。无论是否符合标准集，PAH 患者的死亡率均较高，且基于标准集无患者间差异。
新的 2013 年 ARC/EULAR 标准在识别和区分局限性 SSc 患者，特别是合并 PAH 的患者方面比旧的 1980 年 ACR 标准更准确。由于早期治疗可改善 PAH 的预后，所有 SSc 患者都应接受 PAH 筛查。
