Objective Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc). In this longitudinal study, we aimed to identify factors associated with an unfavourable outcome in patients with SSc with early PAH (SSc-PAH) from the DETECT cohort.
Methods Patients with SSc-PAH enrolled in DETECT were observed for up to 3 years. Associations between cross-sectional variables and disease progression (defined as the occurrence of any of the following events: WHO Functional Class worsening, combination therapy for PAH, hospitalisation or death) were analysed by univariable logistic regression.
Results Of 57 patients with PAH (median observation time 12.6 months), 25 (43.9%) had disease progression. The following factors (OR (95% CI)) were associated with disease progression: male gender (4.1 (1.2 to 14.1)), high forced vital capacity % predicted/carbon monoxide lung diffusion capacity (DLCO)% predicted ratio (3.6 (1.2 to 10.7)), high Borg Dyspnoea Index (1.7 (1.1 to 2.6)) and low DLCO% predicted (non-linear relationship).
Conclusion More than 40% of early-diagnosed patients with SSc-PAH had disease progression during a short follow-up time, with male gender, functional capacity and pulmonary function tests at PAH diagnosis being associated with progression. This suggests that even mild PAH should be considered a high-risk complication of SSc.
- systemic sclerosis
- cardiovascular disease
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Handling editor Tore K Kvien
Contributors All authors participated in study design. OD, MA, DK, JGC, CPD, EG, DB, UM-L, JEP, MCV, JRS and VVM recruited patients to the DETECT study and collected data. HC-B and DMR conducted the statistical analyses. CM, RD and OD drafted the manuscript, which was critically reviewed, edited and approved for submission by all authors.
Funding Actelion Pharmaceuticals Ltd. funded the DETECT study including is longitudinal phase presented in this manuscript.
Competing interests CM has/had consultancy relationship and/or has received honoraria from Actelion Pharmaceuticals, AbbVie, Roche and Geneva Romfarm in the area of systemic sclerosis and its complications. MA: no disclosures. RD has received research funding from Actelion Pharmaceuticals and Pfizer (Articulum fellowship). DB has/had consultancy relationship and/or has received research funding from Actelion Pharmaceuticals, GlaxoSmithKline, Merck Sharp & Dohme, Bayer, Pfizer, AOP Orphan and United Therapeutics. JGC has/had consultancy relationship and/or has received research funding from Actelion Pharmaceuticals, GlaxoSmithKline, United Therapeutics, Bayer and Endotronix. CPD has/had consultancy relationships, received lecture honoraria and/or has received research funding from Actelion Pharmaceuticals, Pfizer, GlaxoSmithKline, Sanofi-Aventis and Novartis. EG received speaker honoraria/advisory board fees and has taken part in clinical trials from Actelion Pharmaceuticals, GlaxoSmithKline, Merck Sharp & Dohme, Bayer, United Therapeutics, Pfizer, OMT, AOP Orphan and Novartis. DK has/had consultancy relationship and/or has received research funding from Actelion Pharmaceuticals, Bayer, Bristol-Myers Squibb, Covis, Cytori, EMD Serono, Genentech/Roche, Gilead, GSK, Sanofi-Aventis and NIH K24AR063120. VVM has acted as a consultant and/or received honoraria/lecture fees from Actelion Pharmaceuticals, Bayer, Gilead and United Therapeutics. She has received research funding (to the University of Michigan) from Actelion Pharmaceuticals, Bayer, Novartis and United Therapeutics. UM-L has acted as a consultant and lecturer for Actelion Pharmaceuticals, Pfizer and GlaxoSmithKline. JEP consults for Actelion Pharmaceuticals, Bayer, Bristol-Myers Squibb, Merck and Roche with respect to potential SSc treatment. JRS has/had consultancy relationships with Acer, Anthera, arGen-X, aTyr, Bayer, Blade, Boehringer Ingelheim, Bristol Myers Squibb, Biogen Idec, Covis, Eiger, EMD Serono, Genkyotex, Gilead, Ironwood, Medac, MedImmune, Mitsubishi, Octapharma, Roche-Genentech, Sanofi, Teva and United Therapeutics. MCV has/had consultancy relationship and/or has received research funding from Actelion Pharmaceuticals, Therabel and United Therapeutics. HC-B, MD and DMR are employees of Actelion Pharmaceuticals. OD has/had consultancy relationship and/or has received research funding from 4D Science, Actelion Pharmaceuticals, Active Biotec, Bayer, Biogen Idec, Boehringer Ingelheim Pharma, Bristol-Myers Squibb, ChemomAb, EpiPharm, Ergonex, espeRare Foundation, GlaxoSmithKline, Roche-Genentech, Inventiva, Lilly, Medac, MedImmune, Mitsubishi Tanabe, Pharmacyclics, Pfizer, Sanofi, Serodapharm and Sinoxa in the area of potential treatments of scleroderma and its complications. He has a patent on mir-29 for the treatment of systemic sclerosis licensed.
Patient consent Obtained.
Ethics approval Local institutional review boards/ethics committees of participating centres.
Provenance and peer review Not commissioned; externally peer reviewed.