RT Journal Article
SR Electronic
T1 Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 128
OP 132
DO 10.1136/annrheumdis-2017-211480
VO 77
IS 1
A1 Carina Mihai
A1 Milos Antic
A1 Rucsandra Dobrota
A1 Diana Bonderman
A1 Harbajan Chadha-Boreham
A1 John Gerry Coghlan
A1 Christopher P Denton
A1 Martin Doelberg
A1 Ekkehard Grünig
A1 Dinesh Khanna
A1 Vallerie V McLaughlin
A1 Ulf Müller-Ladner
A1 Janet E Pope
A1 Daniel M Rosenberg
A1 James R Seibold
A1 Madelon C Vonk
A1 Oliver Distler
YR 2018
UL http://ard.bmj.com/content/77/1/128.abstract
AB 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.