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: 2683

Factors Associated with Disease Progression in Early-Diagnosed Pulmonary Arterial Hypertension Associated with Systemic Sclerosis

Carina Mihai1, Milos Antic2, Rucsandra Dobrota3, Diana Bonderman4, Harbajan Chadha-Boreham5, J. Gerry Coghlan6, Christopher Denton7, Martin Doelberg5, Ekkehard Gruenig8, Dinesh Khanna9, Vallerie McLaughlin10, Ulf Müller-Ladner11, Janet E. Pope12, Daniel M Rosenberg5, James R Seibold13, Madelon C Vonk14 and Oliver Distler15, 1Internal Medicine and Rheumatology Dept., Cantacuzino Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania, 2University Hospital Zurich, Zurich, Switzerland, 3Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland, 4Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Vienna, Austria, 5Actelion Pharmaceuticals Ltd., Alschwil, Switzerland, 6National Pulmonary Hypertension Service, Royal Free Hospital, London, United Kingdom, 7Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom, 8Centre for Pulmonary Hypertension, Thoraxclinic, University Hospital Heidelberg, Heidelberg, Germany, 9Division of Rheumatology and Clinical Autoimmune Center of Excellence, University of Michigan, Ann Arbor, MI, Ann Arbor, MI, 10Internal Medicine, Division of Cardiology, University of Michigan, Ann Arbor, MI, 11Justus-Liebig-University Giessen, Department of Internal Medicine and Rheumatology, Kerckhoff-Klinik, Bad Nauheim, Germany, Bad-Nauheim, Germany, 12Department of Medicine, Division of Rheumatology, University of Western Ontario, St Joseph's Health Care, London, ON, Canada, 13Scleroderma Research Consultants LLC, Litchfield, CT, 14Rheumatology, Radboud University Medical Centre, Nijmegen, Netherlands, 15Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: outcomes, pulmonary complications and systemic sclerosis

  • Tweet
  • Email
  • Print
Session Information

Date: Tuesday, November 7, 2017

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster III

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc), and its diagnosis requires right heart catheterization (RHC). The DETECT study developed an algorithm to select SSc patients with high suspicion of PAH for referral to RHC, identifying patients with early stage PAH and minimizing missed diagnoses. In this longitudinal follow up study, we aimed to identify factors associated with PAH progression in the DETECT cohort.

Methods: DETECT enrolled patients with SSc fulfilling the 1980 ACR classification criteria with a disease duration <3 years since the first non-Raynaud symptom, and who had a pulmonary diffusing capacity for carbon monoxide (DLCO) < 60% of the predicted value. Patients who had been previously diagnosed with pulmonary hypertension (PH) by RHC, who received treatment for PH, and who were at risk for developing PH other than PAH were excluded. A broad range of clinical and laboratory parameters potentially associated with PAH were assessed and RHC was performed in all patients at baseline. Patients diagnosed with PAH at baseline were followed up for up to 3 years in centers that agreed for the longitudinal part of the DETECT study, collecting data on survival, World Health Organization (WHO) Functional Class (FC), hospitalization, and PAH-specific treatment. Disease progression was defined as the occurrence of any of the following: WHO-FC worsening, PAH therapy with a drug combination, PAH-related hospitalization, or death. Associations between baseline variables and disease progression were assessed by univariable logistic regression.

Results: Of the 145 SSc patients with PH enrolled in the DETECT study, 87 patients were diagnosed with group I PH (PAH), of whom 57 participated in the longitudinal follow up study (median follow-up time 12.6 months, interquartile range 10.7-21.7 months). Among these 57 patients, 33 (57.9%) had mild PAH, in WHO FC I or II, and 52 received PAH-specific therapy. During follow-up, 25/57 (43.9%) patients had disease progression (4 deaths, 11 hospitalizations for PAH, 14 with worsening in WHO FC, and 8 received PAH-specific combination treatment), with a 1-year survival rate of 93%. The following factors [odds ratio, (95% confidence interval, CI)] were associated with disease progression: male gender [4.1 (1.1-14.1)], high Forced Vital Capacity (FVC) % predicted/ DLCO % predicted ratio [3.6 (1.2-10.7)], and high Borg dyspnoea index [1.7 (1.1-2.6)]. Low DLCO (% predicted) was also significantly associated with progression [area under the curve (95% CI) 0.8 (0.6-0.9)], but the relationship was not linear. A sensitivity analysis, excluding 4 patients with missing outcome data, was also performed and found similar results.

Conclusion: Although the large majority of these patients with early-diagnosed SSc-PAH were treated with PAH-specific drugs, more than 40% had disease progression during a rather short follow-up time, with male gender, functional capacity, and pulmonary function tests (low DLCO, high FVC%/DLCO % predicted ratio) at PAH diagnosis being associated with progression. This suggests that even mild and early detected PAH should be regarded as a high-risk complication of SSc.


Disclosure: C. Mihai, Actelion Pharmaceuticals Ltd., Geneva Romfarm, Abbvie, Roche, 5; M. Antic, None; R. Dobrota, Actelion Pharmaceuticals Ltd., Pfizer, 2; D. Bonderman, Actelion Pharmaceuticals Ltd, GlaxoSmithKline, Merck Sharp & Dohme, Bayer, Pfizer, AOP Orphan, United Therapeutics, 5; H. Chadha-Boreham, Actelion Pharmaceuticals Ltd., 3; J. G. Coghlan, Actelion Pharmaceuticals Ltd, GlaxoSmithKline, United Therapeutics, Bayer, Endotronics, 5; C. Denton, Actelion Pharmaceuticals Ltd, Pfizer, GlaxoSmithKline, Sanofi-Aventis, Novartis, 5; M. Doelberg, Actelion Pharmaceuticals Ltd., 3; E. Gruenig, Actelion Pharmaceuticals Ltd, GlaxoSmithKline, Merck Sharp & Dohme, Bayer, United Therapeutics, Pfizer, OMT, AOP Orphan, Novartis., 5; D. Khanna, Actelion Pharmaceuticals Ltd, Bayer, Bristol-Myers Squibb, Covis, Cytori, EMD Serono, Genentech/Roche, Gilead, GSK, Sanofi-Aventis, 5,NIH K24AR063120, 2; V. McLaughlin, Actelion Pharmaceuticals Ltd, Bayer, Gilead, Novartis and United Therapeutics., 5; U. Müller-Ladner, Actelion Pharmaceuticals Ltd, Pfizer and GlaxoSmithKline., 5; J. E. Pope, Actelion Pharmaceuticals Ltd, Bayer, Bristol-Myers Squibb, Merck, Roche, 5; D. M. Rosenberg, Actelion Pharmaceuticals Ltd., 3; J. R. Seibold, Athersys, BriaCell Therapeutics, Pacific Therapeutics, Cytori, 1,Actelion Pharmaceuticals, Bayer, Boehringer-Ingelheim, Covis, Cytori, Eiger, Eicos, EMD Serono, Ironwood, OctaPharma, Medac, 5; M. C. Vonk, Actelion Pharmaceuticals Ltd, Therabel and United Therapeutics., 5; O. Distler, 4 D Science, Actelion, Active Biotec, Bayer, Biogen Idec, Boehringer Ingelheim Pharma, BMS, ChemomAb, EpiPharm, Ergonex, espeRare foundation, GSK,Roche-Genentech, Inventiva, Lilly, medac, MedImmune, Mitsubishi Tanabe, Pharmacyclics, Pfizer, Sanofi, Seroda, 2.

To cite this abstract in AMA style:

Mihai C, Antic M, Dobrota R, Bonderman D, Chadha-Boreham H, Coghlan JG, Denton C, Doelberg M, Gruenig E, Khanna D, McLaughlin V, Müller-Ladner U, Pope JE, Rosenberg DM, Seibold JR, Vonk MC, Distler O. Factors Associated with Disease Progression in Early-Diagnosed Pulmonary Arterial Hypertension Associated with Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/factors-associated-with-disease-progression-in-early-diagnosed-pulmonary-arterial-hypertension-associated-with-systemic-sclerosis/. Accessed .
  • Tweet
  • Email
  • Print

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/factors-associated-with-disease-progression-in-early-diagnosed-pulmonary-arterial-hypertension-associated-with-systemic-sclerosis/

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