ACR Meeting Abstracts

ACR Meeting Abstracts

  • Meetings
    • ACR Convergence 2024
    • ACR Convergence 2023
    • 2023 ACR/ARP PRSYM
    • ACR Convergence 2022
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • ACR Meetings

Abstract Number: 0550

Treatment Regimens and Outcomes in Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Hilde Jenssen Bjørkekjær1, Håvard Fretheim2, Helene Marjavara Inselseth3, Øyvind Midtvedt4, Imon Barua4, Torhild Garen5, Helle Bitter1, Arne Andreassen6, Øyvind Molberg4, Kaspar Broch6 and Anna-Maria Hoffmann-Vold4, 1Department of Rheumatology, Sørlandet sykehus HF, Kristiansand, Norway, 2Oslo University Hospital, Oslo, Norway, 3University of Oslo, Oslo, Norway, 4Department of Rheumatology, Oslo University Hospital, Oslo, Norway, 5Department of Rheumatology, Oslo University Hospital, Oslo, Nepal, 6Department of Cardiology, Oslo University Hospital, Oslo, Norway

Meeting: ACR Convergence 2021

Keywords: Pulmonary arterial hypertension, Systemic sclerosis, Upfront dual combination therapy

  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print
Session Information

Date: Sunday, November 7, 2021

Title: Systemic Sclerosis & Related Disorders – Basic Science Poster (0541–0559)

Session Type: Poster Session B

Session Time: 8:30AM-10:30AM

Background/Purpose: Pulmonary arterial hypertension (PAH) is a major clinical challenge in systemic sclerosis (SSc). It is associated with impaired quality of life and high mortality. In 2015, the treatment recommendations changed, recommending upfront combination therapy from the time of diagnosis. The objective of this study was to assess the impact of upfront dual combination therapy versus monotherapy on PAH progression and mortality.

Methods: We included all patients with SSc who had right heart catheterization (RHC) between 2005 and 2018 at Oslo University Hospital, and who were diagnosed with PAH, defined as a mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg and a pulmonary artery wedge pressure ≤ 15mmHg in the absence of interstitial lung disease (ILD). Longitudinal clinical characteristics including treatment were assessed; vital status were available in all patients. To study treatment regimens over time, patients were categorized into three subgroups in regard to diagnostic periods. To secure that all patients had at least 3 years of follow up, group 1 consisted of patients diagnosed between 2005 and 2009, group 2 of patients diagnosed between 2010 and 2014, and group 3 of patients diagnosed between 2015 and 2018. Treatment was initiated as soon as PAH had been confirmed, and included (1) an endothelin-1 receptor antagonist (ERA) and/or (2) a phosphodiesterase-5 inhibitor (PDE5 inhibitor). To assess the impact of treatment regimens on mortality and PAH progression, two outcomes were assessed, including (1) death and (2) PAH-progression defined as a) intensified PAH treatment, b) increasing respiratory symptoms and/or c) hemodynamic progression defined as reduction in the cardiac index (CI) to less than 2.5 l/min/m2, or mixed venous oxygen saturation (SVO2) < 65%. We constructed Kaplan-Meier plots and performed time-to-event analyses.

Results: In total, 191 patients had RHC in the study period, whereof 56 (29 %) SSc patients had a PAH diagnosis. Mean age at SSc-diagnosis was 59 years, mean age at PAH-diagnosis 66 years; 80 % were female and 93 % had limited cutaneous SSc (table 1). None of the patients in group 1 started upfront combination therapy compared to 53 % of patients in group 3. Event-free survival was better in group 3 compared to groups 1 and 2 as shown in the table and figure (p=0.05). 3-year survival increased numerically across groups 1 through 3, from 54% via 62% to 78% (p= 0.148).

Conclusion: We show that upfront dual combination therapy for SSc-PAH has been used more frequently after 2015 in Norway. This shift is associated with less progression of PAH and increased survival in patients with SSc-PAH. This may indicate that there is a benefit of upfront dual combination therapy in SSc-PAH patients in addition to higher awareness and better monitoring strategies.

Table 1. SSc: systemic sclerosis; PAH: pulmonary arterial hypertension; lcSSc: limited cutaneous systemic sclerosis; ACA: anti-centromere antibodies; NYHA: New York Heart Functional Classification; mPAP: mean pulmonary arterial pressure; PVR: pulmonary vascular resistance; PAWP: pulmonary artery wedge pressure; SvO2: mixed venous oxygen saturation; ERA: Endothelin_1-inhibitor; PDE5 inhibitor: Phosphodiesterase-5-inhibitor.

Figure 1. Kaplan Meier curve showing time to PAH progression in SSc-PAH patients segregated by the time of PAH diagnosis.


Disclosures: H. Bjørkekjær, None; H. Fretheim, Bayer, 12, Dr. Fretheim reports grants from Norwegian Women's Public Health Association, during the conduct of the study; personal fees from Bayer and travel bursaries from Actelion and GlaxoSmithKline, outside the submitted work; ., Actelion, 12, Travel bursaries from Actelion, GlaxoSmithKline, 12, travel bursaries GlaxoSmithKline, outside the submitted work;; H. Inselseth, None; . Midtvedt, None; I. Barua, None; T. Garen, None; H. Bitter, None; A. Andreassen, Actelion, 1, 6, United Therapeutics, 1, 6, Bayer, 1, 6, GSK, 1, 6; . Molberg, None; K. Broch, None; A. Hoffmann-Vold, Actelion, 1, 2, 6, Arxx Therapeutics, 1, 2, Bayer, 5, Boehringer Ingelheim, 1, 2, 5, 6, Lilly, 6, Medscape, 2, 6, Merck Sharp & Dohme, 6, Roche, 6.

To cite this abstract in AMA style:

Bjørkekjær H, Fretheim H, Inselseth H, Midtvedt , Barua I, Garen T, Bitter H, Andreassen A, Molberg , Broch K, Hoffmann-Vold A. Treatment Regimens and Outcomes in Systemic Sclerosis-associated Pulmonary Arterial Hypertension [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/treatment-regimens-and-outcomes-in-systemic-sclerosis-associated-pulmonary-arterial-hypertension/. Accessed .
  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print

« Back to ACR Convergence 2021

ACR Meeting Abstracts - https://acrabstracts.org/abstract/treatment-regimens-and-outcomes-in-systemic-sclerosis-associated-pulmonary-arterial-hypertension/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts during your browser session by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

Violation of this policy may result in the abstract being withdrawn from the meeting and other measures deemed appropriate. Authors are responsible for notifying colleagues, institutions, communications firms, and all other stakeholders related to the development or promotion of the abstract about this policy. If you have questions about the ACR abstract embargo policy, please contact ACR abstracts staff at [email protected].

Wiley

  • Online Journal
  • Privacy Policy
  • Permissions Policies
  • Cookie Preferences

© Copyright 2025 American College of Rheumatology