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

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

Author information

1
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@medisin.uio.no.
2
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. a.m.hoffmann-vold@medisin.uio.no.
3
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.
4
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.

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.

KEYWORDS:

AUTOANTIBODIES; OUTCOME RESEARCH; PULMONARY FIBROSIS; PULMONARY HYPERTENSION; SYSTEMIC SCLEROSIS

PMID:
28089974
DOI:
10.3899/jrheum.160867
[Indexed for MEDLINE]

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