Skip to main content

Main menu

  • Home
  • Content
    • First Release
    • Current
    • Archives
    • Collections
    • Audiovisual Rheum
    • COVID-19 and Rheumatology
  • Resources
    • Guide for Authors
    • Submit Manuscript
    • Payment
    • Reviewers
    • Advertisers
    • Classified Ads
    • Reprints and Translations
    • Permissions
    • Meetings
    • FAQ
    • Policies
  • Subscribers
    • Subscription Information
    • Purchase Subscription
    • Your Account
    • Terms and Conditions
  • About Us
    • About Us
    • Editorial Board
    • Letter from the Editor
    • Duncan A. Gordon Award
    • Privacy/GDPR Policy
    • Accessibility
  • Contact Us
  • JRheum Supplements
  • Services

User menu

  • My Cart
  • Log In
  • Log Out

Search

  • Advanced search
The Journal of Rheumatology
  • JRheum Supplements
  • Services
  • My Cart
  • Log In
  • Log Out
The Journal of Rheumatology

Advanced Search

  • Home
  • Content
    • First Release
    • Current
    • Archives
    • Collections
    • Audiovisual Rheum
    • COVID-19 and Rheumatology
  • Resources
    • Guide for Authors
    • Submit Manuscript
    • Payment
    • Reviewers
    • Advertisers
    • Classified Ads
    • Reprints and Translations
    • Permissions
    • Meetings
    • FAQ
    • Policies
  • Subscribers
    • Subscription Information
    • Purchase Subscription
    • Your Account
    • Terms and Conditions
  • About Us
    • About Us
    • Editorial Board
    • Letter from the Editor
    • Duncan A. Gordon Award
    • Privacy/GDPR Policy
    • Accessibility
  • Contact Us
  • Follow jrheum on Twitter
  • Visit jrheum on Facebook
  • Follow jrheum on LinkedIn
  • Follow jrheum on YouTube
  • Follow jrheum on Instagram
  • Follow jrheum on RSS
Abstract

Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody.

Virginia D Steen, Mary Lucas, Noreen Fertig and Thomas A Medsger Jr
The Journal of Rheumatology November 2007, 34 (11) 2230-2235;
Virginia D Steen
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Mary Lucas
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Noreen Fertig
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Thomas A Medsger Jr
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • References
  • Info & Metrics
  • PDF
  • eLetters
PreviousNext
Loading

Abstract

OBJECTIVE: Pulmonary arterial hypertension (PAH) and severe pulmonary fibrosis (SPF) are the most common causes of death in scleroderma. Our study focuses on lung disease in patients with a nucleolar antibody in comparison to other scleroderma-specific autoantibodies. METHODS: Patients initially seen between 1972 and 1995 (and followed through 2004) with [systolic pulmonary artery pressure (sPAH) (PASP > 50 mm Hg] or SPF [forced vital capacity (FVC%) < 55% predicted) were grouped by the presence of anticentromere antibody (ACA), an isolated antinucleolar antibody (ANoA), or an antitopoisomerase antibody-I (TOPO). RESULTS: Twenty percent of ACA, 23% of TOPO, and 32% of ANoA patients had severe lung disease (p < 0.005). In ANoA patients with PAH without severe fibrosis, the FVC was lower (71% predicted) than in ACA patients, suggesting they had some interstitial fibrosis. However, they had a higher FVC%/diffusing capacity for carbon monoxide (DLCO)% ratio than the ACA patients (2.4 vs 1.8). pulmonary hypertension in TOPO patients was associated with a lower FVC%/DLCO% ratio and lower levels of PAP than either the PAH in ACA or ANoA patients. CONCLUSION: Scleroderma-specific autoantibodies are associated with characteristic subgroups of lung disease. The ANoA patients have a unique mixture of PAH and SPF subgroups of lung disease. Scleroderma-specific autoantibodies and the FVC%/DLCO% ratio are helpful in determining whether a patient has PAH alone, PAH along with pulmonary fibrosis, or secondary PAH from chronic hypoxia with SPF.

PreviousNext
Back to top

In this issue

The Journal of Rheumatology
Vol. 34, Issue 11
1 Nov 2007
  • Table of Contents
  • Table of Contents (PDF)
  • Index by Author
Download PDF
Article Alerts
Sign In to Email Alerts with your Email Address
Email Article

Thank you for your interest in spreading the word about The Journal of Rheumatology.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody.
(Your Name) has forwarded a page to you from The Journal of Rheumatology
(Your Name) thought you would like to see this page from the The Journal of Rheumatology web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Citation Tools
Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody.
Virginia D Steen, Mary Lucas, Noreen Fertig, Thomas A Medsger
The Journal of Rheumatology Nov 2007, 34 (11) 2230-2235;

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero

 Request Permissions

Share
Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody.
Virginia D Steen, Mary Lucas, Noreen Fertig, Thomas A Medsger
The Journal of Rheumatology Nov 2007, 34 (11) 2230-2235;
del.icio.us logo Digg logo Reddit logo Twitter logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One
Bookmark this article

Jump to section

  • Article
  • References
  • Info & Metrics
  • PDF
  • eLetters

Related Articles

Cited By...

Similar Articles

Content

  • First Release
  • Current
  • Archives
  • Collections
  • Audiovisual Rheum
  • COVID-19 and Rheumatology

Resources

  • Guide for Authors
  • Submit Manuscript
  • Author Payment
  • Reviewers
  • Advertisers
  • Classified Ads
  • Reprints and Translations
  • Permissions
  • Meetings
  • FAQ
  • Policies

Subscribers

  • Subscription Information
  • Purchase Subscription
  • Your Account
  • Terms and Conditions

More

  • About Us
  • Contact Us
  • My Alerts
  • My Folders
  • Privacy/GDPR Policy
  • RSS Feeds
The Journal of Rheumatology
The content of this site is intended for health care professionals.
Copyright © 2022 by The Journal of Rheumatology Publishing Co. Ltd.
Print ISSN: 0315-162X; Online ISSN: 1499-2752
Powered by HighWire