Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort - Forschungsportal der Justus-Liebig-Universität Gießen:

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Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort

Autorenliste: Mihai, Carina; Antic, Milos; Dobrota, Rucsandra; Bonderman, Diana; Chadha-Boreham, Harbajan; Coghlan, John Gerry; Denton, Christopher P.; Doelberg, Martin; Gruenig, Ekkehard; Khanna, Dinesh; McLaughlin, Vallerie V.; Mueller-Ladner, Ulf; Pope, Janet E.; Rosenberg, Daniel M.; Seibold, James R.; Vonk, Madelon C.; Distler, Oliver

Jahr der Veröffentlichung: 2018

Seiten: 128-132

Zeitschrift: Annals of the Rheumatic Diseases

Bandnummer: 77

Heftnummer: 1

ISSN: 0003-4967

eISSN: 1468-2060

DOI Link: https://doi.org/10.1136/annrheumdis-2017-211480

Verlag: Elsevier

Abstract:

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.

Zitierstile

Harvard-Zitierstil: Mihai, C., Antic, M., Dobrota, R., Bonderman, D., Chadha-Boreham, H., Coghlan, J., et al. (2018) Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort, Annals of the Rheumatic Diseases, 77(1), pp. 128-132. https://doi.org/10.1136/annrheumdis-2017-211480

APA-Zitierstil: Mihai, C., Antic, M., Dobrota, R., Bonderman, D., Chadha-Boreham, H., Coghlan, J., Denton, C., Doelberg, M., Gruenig, E., Khanna, D., McLaughlin, V., Mueller-Ladner, U., Pope, J., Rosenberg, D., Seibold, J., Vonk, M., & Distler, O. (2018). Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort. Annals of the Rheumatic Diseases. 77(1), 128-132. https://doi.org/10.1136/annrheumdis-2017-211480

Zitierstile

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