Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2014 Feb;43(4):536-41.
doi: 10.1016/j.semarthrit.2013.08.002. Epub 2013 Sep 5.

Screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension: a systematic review

Heather Gladue  1 , Nezam Altorok  1 , Whitney Townsend  2 , Vallerie McLaughlin  3 , Dinesh Khanna  4
Affiliations

Screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension: a systematic review

Heather Gladue et al. Semin Arthritis Rheum. 2014 Feb.

Abstract

Objective: Pulmonary arterial hypertension (PAH) is a frequent complication in connective tissue diseases (CTD), especially in systemic sclerosis (SSc), and is associated with a high degree of morbidity and mortality. We undertook a systematic review for the screening tests for CTD-PAH.

Methods: A systematic literature search of PAH in CTD was performed in available databases through June 2012. Our evaluation of diagnostic tests was focused on patients with PAH confirmed by right heart catheterization (RHC).

Results: The search resulted in 2805 titles and 838 abstracts. Our final inclusion encompassed 22 articles-six of which were case-control studies and 16 were cohort studies. Twelve studies assessed the tricuspid regurgitation velocity (VTR) or equivalent right ventricular systolic pressure (RVSP) using transthoracic echocardiogram (TTE) as a threshold for RHC in patients suspected as having PAH. The screening threshold for RHC was VTR from >2.73 to >3.16 m/s without symptoms or 2.5-3.0m/s with symptoms and resulted in 20-67% of patients having RHC-proven PAH. Three studies looked at pulmonary function tests and found that a low lung diffusing capacity for carbon monoxide (DLCO) (45-70% of predicted) is associated with a 5.6-7.4% development of PAH, and a decline in DLCO% is associated with an increase in the specificity (for DLCO ≤ 60%, spec = 45%; and for DLCO ≤ 50%, spec = 90%) for PAH. Five studies assessed N-terminal prohormone of brain natriuretic peptide (NT-ProBNP), where a cutoff >239 pg/ml had a sensitivity of 90-100%.

Conclusions: Our systematic review revealed that most evidence exists for TTE, pulmonary function tests, and NT-ProBNP for screening and diagnosis of SSc-PAH; however, more robust studies are needed.

PubMed Disclaimer

Figures

Figure 1
Figure 1. Systematic Review Schematic
1 Exclusion Criteria

  1. Not written in English, concerned with human subjects, or pertaining to adult studies

  2. Not pertaining to pulmonary arterial hypertension or connective tissue diseases.

  3. Genetic studies or polymorphisms or molecular/enzymatic mechanisms except serum/plasma biomarkers.

  4. Articles related to treatment or prognosis of pulmonary hypertension.

  5. Editorials, letters, case reports, opinions, author reply or comments, etc.

2 Exclusion Criteria

  1. Does not have an abstract, unless a review article.

  2. Not a case control or cohort study, or a case-control or cohort study with n< 20 patients with connective tissue disease Not pertaining to pulmonary arterial hypertension or elevated pulmonary artery pressure.

  3. Not pertaining to connective tissue disease.

  4. Genetic studies or polymorphisms or diagnostic test or molecular/enzymatic mechanisms

  5. Editorials, letters, case reports, opinions, author reply or comments, etc. Except review articles after 2008 pertaining to pulmonary hypertension and connective tissue disease screening and diagnosis.

  6. Not having right heart catheterization to diagnose PAH.

3 Exclusion Criteria

  1. Review article.

  2. Pertaining to exercise-induced PAH only

  3. Pertaining to pre-PAH such borderline PAH

  4. Discussing prognosis, treatment response only

  5. For case-control: does not have appropriate comparator (such as CTD without PH)

  6. Does not exclude ILD or left heart disease

4 Hand Picked
See this image and copyright information in PMC

References

    1. Phung S, Strange G, Chung LP, Leong J, Dalton B, Roddy J, et al. Prevalence of pulmonary arterial hypertension in an Australian scleroderma population: screening allows for earlier diagnosis. Intern Med J. 2009;39(10):682–91. Epub 2009/02/18. - PubMed
    1. Launay D, Mouthon L, Hachulla E, Pagnoux C, de Groote P, Remy-Jardin M, et al. Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic sclerosis with and without interstitial lung disease. J Rheumatol. 2007;34(5):1005–11. Epub 2007/04/21. - PubMed
    1. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension. A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association Developed in Collaboration With the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573–619. - PubMed
    1. Galie N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009;34(6):1219–63. - PubMed
    1. Badesch DB, Champion HC, Sanchez MA, Hoeper MM, Loyd JE, Manes A, et al. Diagnosis and assessment of pulmonary arterial hypertension. Journal of the American College of Cardiology. 2009;54(1 Suppl):S55–66. Epub 2009/07/09. - PubMed

Publication types

MeSH terms

Substances