Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort
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 e...
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| Main Authors: | , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
2018
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| In: |
Annals of the rheumatic diseases
Year: 2018, Volume: 77, Issue: 1, Pages: 128-132 |
| ISSN: | 1468-2060 |
| DOI: | 10.1136/annrheumdis-2017-211480 |
| Online Access: | Verlag, Volltext: http://dx.doi.org/10.1136/annrheumdis-2017-211480 Verlag, Volltext: https://ard.bmj.com/content/77/1/128 
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| Author Notes: | Carina Mihai, Milos Antic, Rucsandra Dobrota, Diana Bonderman, Harbajan Chadha-Boreham, John Gerry Coghlan, Christopher P. Denton, Martin Doelberg, Ekkehard Grünig, Dinesh Khanna, Vallerie V. McLaughlin, Ulf Müller-Ladner, Janet E. Pope, Daniel M. Rosenberg, James R. Seibold, Madelon C. Vonk, Oliver Distler |
| Summary: | 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. |
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| Item Description: | Published online first 23 October 2017 Gesehen am 31.10.2018 |
| Physical Description: | Online Resource |
| ISSN: | 1468-2060 |
| DOI: | 10.1136/annrheumdis-2017-211480 |