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Cited by 657

The DOI matches its Crossref record; 15 of the 15 references it cites were found in a registry or an index.

Connective Tissue Disease–associated Pulmonary Arterial Hypertension in the Modern Treatment Era

Open access
Condliffe, Robin, Kiely, David G., Peacock, Andrew J., Corris, Paul A., Gibbs, J. Simon R., Vrapi, Florenc, Das, Clare, Elliot, Charlie A., Johnson, Martin, DeSoyza, Julia, Torpy, Chantal, Goldsmith, Kim, Hodgkins, Denise, Hughes, Rodney J., Pepke-Zaba, Joanna, Coghlan, J. Gerry·2009·American Journal of Respiratory and Critical Care Medicine
DOI 10.1164/rccm.200806-953oc
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Abstract

RATIONALE: Pulmonary arterial hypertension in association with connective tissue disease (CTD-PAH) has historically had a poor prognosis, with a 1-year survival rate among patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) of 45%. However, more therapies have become available. OBJECTIVES: To investigate the survival and characteristics of all patients diagnosed with CTD-PAH in the U.K. pulmonary hypertension service. METHODS: National registry of all incident cases of CTD-PAH diagnosed consecutively between January 2001 and June 2006. MEASUREMENTS AND MAIN RESULTS: Patients with CTD-PAH (429; 73% SSc-PAH) were diagnosed by a catheter-based approach. One- and 3-year survival rates were 78 and 47% for patients with isolated SSc-PAH. Survival was worse for those with respiratory disease-associated SSc-PAH (3-yr survival, 28%; P = 0.005) whereas survival among patients with exercise-induced SSc-PAH was superior (3-yr survival, 86%; P = < 0.001). Age, sex, mixed venous oxygen saturation, and World Health Organization functional class were independent predictors of survival in isolated SSc-PAH. Nineteen percent of patients with exercise-induced SSc-PAH and 39% of patients with isolated SSc-PAH who were in functional classes I and II had evidence of disease progression. The prevalence of diagnosed SSc-PAH is 2.93 per 1 million. The 3-year survival rate of 75% for those with pulmonary arterial hypertension associated with systemic lupus erythematosus (SLE-PAH) was significantly better than that for patients with SSc-PAH (P = 0.01). CONCLUSIONS: Survival of patients with SSc-PAH in the modern treatment era is better than in historical series. A significant proportion of patients with mild functional impairment or exercise-induced SSc-PAH have evidence of disease progression. Survival of patients with respiratory disease-associated pulmonary hypertension is inferior. SLE-PAH has a better prognosis than SSc-PAH.

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Metadata

Authors
Condliffe, R., Kiely, D. G., Peacock, A. J., Corris, P. A., Gibbs, J. S. R., Vrapi, F., Das, C., Elliot, C. A., Johnson, M., DeSoyza, J., Torpy, C., Goldsmith, K., Hodgkins, D., Hughes, R. J., Pepke-Zaba, J., & Coghlan, J. G.
Venue
American Journal of Respiratory and Critical Care Medicine
Publisher
Oxford University Press (OUP)
Published
2009-01-15
Volume
179
Issue
2
Pages
151-157
ISSN
  • 1073-449X (ISSN-L)
  • 1535-4970
DOI
10.1164/rccm.200806-953oc
OpenAlex
W2100418954
Cited by
657
References
15
FWCI
32.83
Type
journal article
Language
en
Open access
green

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CoChat. (2026). Citation check: Connective Tissue Disease–associated Pulmonary Arterial Hypertension in the Modern Treatment Era. https://app.cochat.ai/papers/10.1164/rccm.200806-953oc
The paper
Condliffe, R., Kiely, D. G., Peacock, A. J., Corris, P. A., Gibbs, J. S. R., Vrapi, F., Das, C., Elliot, C. A., Johnson, M., DeSoyza, J., Torpy, C., Goldsmith, K., Hodgkins, D., Hughes, R. J., Pepke-Zaba, J., & Coghlan, J. G. (2009). Connective Tissue Disease–associated Pulmonary Arterial Hypertension in the Modern Treatment Era. American Journal of Respiratory and Critical Care Medicine. https://doi.org/10.1164/rccm.200806-953oc

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.