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Real-world management of patients with pulmonary arterial hypertension: insights from EXPOSURE
Global Epidemiology, Actelion Pharmaceuticals Ltd, A Janssen Pharmaceutical Company of Johnson & Johnson, Allschwil, Switzerland.
Pulmonary Hypertension Unit, Cardiology Department, CIBERCV, Hospital 12 de Octubre, Madrid, Spain.
Global Medical Affairs, Actelion Pharmaceuticals Ltd, A Janssen Pharmaceutical Company of Johnson & Johnson, Allschwil, Switzerland.
Statistics and Decision Sciences, Janssen-Cilag S.p.A, Cologno Monzese, Italy.
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2024 (English)In: Advances in Therapy, ISSN 0741-238X, E-ISSN 1865-8652, Vol. 41, no 3, p. 1103-1119Article in journal (Refereed) Published
Abstract [en]

Introduction: Further insights into real-world management and outcomes of patients with pulmonary arterial hypertension (PAH) are needed. This interim analysis of the ongoing, multicentre, prospective EXPOSURE (EUPAS19085) observational study describes characteristics, treatment patterns and outcomes of patients with PAH initiating a new PAH-specific therapy in Europe/Canada.

Methods and Results: All analyses were descriptive. In total, 1944 patients with follow-up information were included; the majority were female, with World Health Organization functional class II/III symptoms and with idiopathic PAH or connective tissue disease-associated PAH. Most incident patients (N = 1100; diagnosed for ≤ 6 months) initiated treatment as monotherapy (48%) or double therapy (43%). Of those initiating monotherapy, 38% (199/530) escalated to double therapy (median [Q1, Q3] time to escalation 3.4 [1.9, 6.6] months), and of those initiating double therapy, 17% (78/457) escalated to triple therapy (median [Q1, Q3] time to escalation 7.0 [3.4, 12.7] months) during the observation period (median [Q1, Q3]: 17.0 [7.5, 29.9] months). The majority of the 834 prevalent patients (diagnosed > 6 months) entered the study on initiation of combination therapy and most did not change treatment regimen during the observation period (median [Q1, Q3]: 19.6 [10.2, 32.2] months). One-year survival was 88% for incident patients and 90% for prevalent patients.

Conclusions: Results from EXPOSURE suggest a shift towards combination therapy and the alignment of real-world treatment patterns with current guideline recommendations. While survival estimates are encouraging, the extent of monotherapy use at treatment initiation and follow-up highlight an opportunity for further improvements through optimisation of treatment strategies in line with current guidelines.

A graphical abstract is also available with this article.

Trial Registration Number: EUPAS19085.

Place, publisher, year, edition, pages
Springer Nature, 2024. Vol. 41, no 3, p. 1103-1119
Keywords [en]
Epidemiology, Outcomes, Pulmonary arterial hypertension, Real-world evidence, Survival, Treatment patterns
National Category
Cardiac and Cardiovascular Systems
Identifiers
URN: urn:nbn:se:umu:diva-219825DOI: 10.1007/s12325-023-02730-8ISI: 001142087300001PubMedID: 38216826Scopus ID: 2-s2.0-85182202295OAI: oai:DiVA.org:umu-219825DiVA, id: diva2:1830116
Available from: 2024-01-22 Created: 2024-01-22 Last updated: 2024-05-07Bibliographically approved

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Söderberg, Stefan

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