Journal
ARTHRITIS AND RHEUMATISM
Volume 65, Issue 12, Pages 3194-3201Publisher
WILEY
DOI: 10.1002/art.38172
Keywords
-
Categories
Funding
- Scleroderma Foundation
- Actelion
- Gilead
- United Therapeutics
- NIH (National Institute of Arthritis and Musculoskeletal and Skin Diseases) [K24-AR-063120-02]
- Scleroderma Research Foundation
- NIH
- Pulmonary Hypertension Association
- National Heart, Lung, and Blood Institute [K23-HL-093387]
- Roche
- Bristol-Myers Squibb
- Pfizer
- Digna Biotech
- Impact Pharmaceutical Services
- Bayer
- ErgoNex
- Sanofi-Aventis
- Sinoxa
- United BioSource
- Medac
- Swedish Orphan Biovitrum
- Boehringer Ingelheim Pharma
- Novartis
- Active Biotech
- 4D Science
- Lilly
- GlaxoSmithKline
- Aires Pharmaceuticals
- Bayer Healthcare
- Merck
Ask authors/readers for more resources
ObjectivePulmonary arterial hypertension (PAH) affects up to 15% of patients with connective tissue diseases (CTDs). Previous recommendations developed as part of larger efforts in PAH did not include detailed recommendations for patients with CTD-associated PAH. Therefore, we sought to develop recommendations for screening and early detection of CTD-associated PAH. MethodsWe performed a systematic review of the literature on the screening and diagnosis of PAH in CTD. Using the RAND/University of California, Los Angeles consensus methodology, we developed case scenarios followed by 2 stages of voting. First, international experts from a variety of specialties voted anonymously on the appropriateness of each case scenario. The experts then met face-to-face to discuss and resolve discrepant votes to arrive at consensus recommendations. ResultsThe key recommendation stated that all patients with systemic sclerosis (SSc) should be screened for PAH. In addition, patients with mixed connective tissue disease or other CTDs with scleroderma features (scleroderma spectrum disorders) should be screened for PAH. It was recommended that screening pulmonary function tests (PFTs) with single-breath diffusing capacity for carbon monoxide, transthoracic echocardiogram, and measurement of N-terminal pro-brain natriuretic peptide (NT-proBNP) be performed in all patients with SSc and scleroderma spectrum disorders. In patients with SSc and scleroderma spectrum disorders, transthoracic echocardiogram and PFTs should be performed annually. The full screening panel (transthoracic echocardiogram, PFTs, and measurement of NT-proBNP) should be performed as soon as any new signs or symptoms are present. ConclusionWe provide consensus-based, evidence-driven recommendations for screening and early detection of CTD-associated PAH. It is our hope that these recommendations will lead to earlier detection of CTD-associated PAH and ultimately improve patient outcomes.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available