抗RNA聚合酶III阳性系统性硬化症中心肺疾病的发展：来自未选择的前瞻性患者队列的比较分析

Cardiopulmonary Disease Development in Anti-RNA Polymerase III-positive Systemic Sclerosis: Comparative Analyses from an Unselected, Prospective Patient Cohort.

作者信息

Hoffmann-Vold Anna-Maria, Midtvedt Øyvind, Tennøe Anders H, Garen Torhild, Lund May Brit, Aaløkken Trond M, Andreassen Arne K, Elhage Fadi, Brunborg Cathrine, Taraldsrud Eli, Molberg Øyvind

机构信息

From the Department of Rheumatology, the Department of Respiratory Medicine, the Department of Radiology and Nuclear Medicine, and the Department of Cardiology, Oslo University Hospital - Rikshospitalet; Institute of Clinical Medicine, University of Oslo; Institutes of Immunology, Oslo University Hospital; Oslo Centre for Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital, Oslo, Norway.

A.M. Hoffmann-Vold, MD, PhD, Department of Rheumatology, Oslo University Hospital - Rikshospitalet, and Institute of Clinical Medicine, University of Oslo; Ø. Midtvedt, MD, Department of Rheumatology, Oslo University Hospital - Rikshospitalet; A.H. Tennøe, MD, Department of Rheumatology, Oslo University Hospital - Rikshospitalet, and Institute of Clinical Medicine, University of Oslo; T. Garen, MS, Department of Rheumatology, Oslo University Hospital - Rikshospitalet; M.B. Lund, MD, PhD, Professor, Institute of Clinical Medicine, University of Oslo, and Department of Respiratory Medicine, Oslo University Hospital - Rikshospitalet; T.M. Aaløkken, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital - Rikshospitalet; A.K. Andreassen, PhD, Department of Cardiology, Oslo University Hospital - Rikshospitalet; F. Elhage, MD, Institutes of Immunology, Oslo University Hospital; C. Brunborg, MS, Oslo Centre for Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital; E. Taraldsrud, MD, Professor, Department of Respiratory Medicine, Oslo University Hospital - Rikshospitalet; Ø. Molberg, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital - Rikshospitalet, and Institute of Clinical Medicine, University of Oslo.

出版信息

J Rheumatol. 2017 Apr;44(4):459-465. doi: 10.3899/jrheum.160867. Epub 2017 Jan 15.

DOI:10.3899/jrheum.160867

PMID:28089974

Abstract

OBJECTIVE

Extensive skin disease and renal crisis are hallmarks of anti-RNA polymerase III (RNAP)-positive systemic sclerosis (SSc), while lung and heart involvement data are conflicting. Here, the aims were to perform time-course analyses of interstitial lung disease (ILD) and pulmonary hypertension (PH) in the RNAP subset of a prospective unselected SSc cohort and to use the other autoantibody subsets as comparators.

METHODS

The study cohort included 279 patients with SSc from the observational Oslo University Hospital cohort with complete data on (1) SSc-related autoantibodies, (2) paired, serial analyses of lung function and fibrosis by computed tomography, and (3) PH verified by right heart catheterization.

RESULTS

RNAP was positive in 33 patients (12%), 79% of which had diffuse cutaneous SSc. Pulmonary findings were heterogeneous; 49% had no signs of fibrosis while 18% had > 20% fibrosis at followup. Forced vital capacity at followup was < 80% in 39% of the RNAP subset, comparable to the antitopoisomerase subset (ATA; 47%), but higher than anticentromere (ACA; 13%). Accumulated frequency of PH in the RNAP subset (12%) was lower than in ACA (18%). At 93% and 78%, the 5- and 10-year survival rates in RNAP were comparable to the ATA and ACA subsets.

CONCLUSION

In this cohort, the RNAP subset was marked by cardiopulmonary heterogeneity, ranging from mild ILD to development of severe ILD in 18%, and PH development in 12%. These data indicate that cardiopulmonary risk stratification early in the disease course is particularly important in RNAP-positive SSc.

摘要

目的

广泛的皮肤病变和肾危象是抗RNA聚合酶III（RNAP）阳性系统性硬化症（SSc）的特征，而肺部和心脏受累的数据存在矛盾。在此，目的是对一个前瞻性未选择的SSc队列中RNAP亚组的间质性肺疾病（ILD）和肺动脉高压（PH）进行时间进程分析，并将其他自身抗体亚组用作对照。

方法

研究队列包括来自奥斯陆大学医院观察性队列的279例SSc患者，这些患者具有以下完整数据：（1）与SSc相关的自身抗体；（2）通过计算机断层扫描对肺功能和纤维化进行配对、系列分析；（3）通过右心导管检查证实的PH。

结果

33例患者（12%）RNAP呈阳性，其中79%为弥漫性皮肤型SSc。肺部表现各异；49%无纤维化迹象，而18%在随访时有>20%的纤维化。随访时，RNAP亚组中39%的患者用力肺活量<80%，与抗拓扑异构酶亚组（ATA；47%）相当，但高于抗着丝点抗体亚组（ACA；13%）。RNAP亚组中PH的累积发生率（12%）低于ACA（18%）。RNAP亚组5年和10年生存率分别为93%和78%，与ATA和ACA亚组相当。