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: 2374

Pulmonary Arterial Hypertension in Patients with Anti-PM-Scl Antibody

Hiromichi Tamaki1, Ruchi Yadav2, James Bena3 and Soumya Chatterjee4, 1Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Foundation, Cleveland, OH, 2Diagnostic Radiology, Cleveland Clinic, Cleveland, OH, 3Department of Quantitative Health Sciences, Cleveland Clinic Foundation, Cleveland, OH, 4Cleveland Clinic Foundation, Cleveland, OH

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: myositis and systemic sclerosis, Pulmonary Involvement

  • Tweet
  • Email
  • Print
Session Information

Date: Tuesday, November 10, 2015

Title: Muscle Biology, Myositis and Myopathies Poster II: Autoantibodies and Treatments in Inflammatory Myopathies

Session Type: ACR Poster Session C

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

Background/Purpose: Pulmonary arterial hypertension (PAH) may be a disease manifestation of patients with anti-PM-Scl antibody (PM-Scl). In the 2014 ACR annual meeting we reported significantly higher prevalence of precapillary PAH proven by right heart catheterization (RHC) in our patients with PM-Scl compared to historical general population. This study aimed to further characterize PAH in our cohort of patients with PM-Scl.

Methods: All patients screened for PM-Scl between October 1999 and April 2014 were evaluated through our electronic medical record. PAH had to be established by RHC and patients were further subdivided into ‘PAH with interstitial lung disease (ILD)’ and ‘PAH without ILD’. The patients were defined to have ‘PAH with ILD’ if RHC confirmed pre-capillary PAH and forced vital capacity (FVC) was < 60% of predicted. High resolution CT scans (HRCT) were reviewed by a thoracic radiologist with expertise in ILD (RY), and scored to estimate severity of ILD according to the criteria defined by Ooi et al (Acta Radiologica 2003;44:258-264). Radiographic diagnoses on HRCT [cellular non-specific interstitial pneumonia (NSIP), fibrotic NSIP, usual interstitial pneumonia (UIP), and organizing pneumonia (OP)] were determined. Also, the correlation between HRCT score and mean pulmonary artery pressure (mPAP) was analyzed.

Prevalence of PAH in the general population and in patients with SSc were identified by a systematic literature review. A meta-analysis was performed to formulate prevalence of pre-capillary PAH in SSc patients both with and without ILD. Estimates of prevalence were compared using exact binomial tests.

Results: Of the 42 patients with PM-Scl, 3 patients (7.1%) had PAH with ILD and 2 patients (4.8%) had PAH without ILD.  Among the 3 patients with PAH with ILD, 2 had cellular NSIP and 1 had UIP. Due to the small sample size it was difficult to establish any significant linear correlation between mPAP and HRCT score. Systematic literature review identified 5 studies looking at prevalence of PAH in the general population, 3 evaluating PAH with ILD in SSc and 7 assessing PAH without ILD in SSc. Based on these studies the mean prevalence of PAH in the general population was 8.6/million and the maximum estimated prevalence of PAH in the general population was 13.8/million. These numbers were statistically lower than the prevalence of pre-capillary PAH in our cohort (P<0.001) and that of PAH without ILD (P<0.001). Meta-analysis revealed the estimated prevalence of pre-capillary PAH in patients with SSc was 6.3% (95%CI 4.6-8.6%), that of PAH with ILD in patients with SSc was 2.1% (95%CI 1.3-3.5%), and that of PAH without ILD in patients with SSc was 6.4% (95%CI 4.6-9.0%). In comparison to our cohort with PM-Scl no statistically significant difference was noted but there was a trend towards a higher prevalence of PAH with ILD in our cohort compared to that in SSc patients (7.1% vs 2.1%, P=0.058). 

Conclusion: Prevalence of PAH in patients with PM-Scl may be higher than that in the general population but may be comparable to the prevalence of PAH in SSc. Prevalence of PAH with ILD may be higher in patients with PM-Scl compared to that in SSc patients. In this small study, it was difficult to ascertain any correlation between ILD severity and mPAP.


Disclosure: H. Tamaki, None; R. Yadav, None; J. Bena, None; S. Chatterjee, None.

To cite this abstract in AMA style:

Tamaki H, Yadav R, Bena J, Chatterjee S. Pulmonary Arterial Hypertension in Patients with Anti-PM-Scl Antibody [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/pulmonary-arterial-hypertension-in-patients-with-anti-pm-scl-antibody-2/. Accessed .
  • Tweet
  • Email
  • Print

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-arterial-hypertension-in-patients-with-anti-pm-scl-antibody-2/

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