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Published ahead of print on October 17, 2008, doi:10.1164/rccm.200806-953OC

Am. J. Respir. Crit. Care Med., Volume 179, Number 2, January 2009, 151-157

A more recent version of this article appeared on January 15, 2009
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Submitted on June 24, 2008
Accepted on October 17, 2008

Connective Tissue Disease Associated Pulmonary Arterial Hypertension in the Modern Treatment Era

Robin Condliffe1*, David G Kiely2, Andrew J. Peacock3, Paul A Corris4, J Simon R Gibbs5, Florenc Vrapi6, Clare Das6, Charlie A Elliot2, Martin Johnson7, Julia DeSoyza8, Chantal Torpy5, Kim Goldsmith9, Denise Hodgkins9, Rodney J Hughes9, Joanna Pepke-Zaba9, and J Gerry Coghlan6

1 Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom; Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, United Kingdom, 2 Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom, 3 Scottish Pulmonary Vascular Disease Unit, Western Infirmary, Glasgow, United Kingdom, 4 Northern Vascular Unit, Freeman Hospital, Newcastle-upon-Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle-upon-Tyne, United Kingdom, 5 Department of Cardiology, Hammersmith Hospital, London, United Kingdom, 6 Department of Cardiology, Royal Free Hospital, London, United Kingdom, 7 Scottish Pulmonary Vascular Unit, Western Infirmary, Glasgow, United Kingdom, 8 Northern Vascular Unit, Freeman Hospital, Newcastle-upon-Tyne, United Kingdom, 9 Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, United Kingdom

* To whom correspondence should be addressed. E-mail: condliffe{at}doctors.org.uk.

Rationale: Pulmonary arterial hypertension in association with connective tissue disease (CTD-PAH) has historically had a poor prognosis with a 1 year survival in systemic sclerosis associated pulmonary arterial hypertension (SSc-PAH) of 45%. Over recent years more therapies have become available. Objectives: To investigate the survival and characteristics of all patients diagnosed with CTD-PAH in the UK pulmonary hypertension service. Methods: National registry of all incident cases of CTD-PAH diagnosed consecutively between January 2001 and June 2006. Main Results: 429 CTD-PAH patients (73% SSc-PAH) were diagnosed using a catheter-based approach. One and three year survival was 78% and 47% in isolated SSc-PAH. Survival was worse in respiratory disease associated SSc-PAH (3 year survival 28%, p=0.005) while survival in exercise-induced SSc-PAH was superior (3 year survival 86%, p=<0.001). Age, gender, mixed venous oxygen saturation and WHO functional class were independent predictors of survival in isolated SSc-PAH. 19% of patients with exercise-induced SSc-PAH and 39% of patients with isolated SSc-PAH who were in functional class I and II had evidence of disease progression. The prevalence of isolated SSc-PAH was 2.5/million. The 3 year survival of 75% in pulmonary arterial hypertension associated with systemic lupus erythematosus (SLE-PAH) was significantly better than in SSc-PAH (p=0.01). Conclusions: Survival in 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 in patients with respiratory disease associated pulmonary hypertension is inferior. SLE-PAH has a better prognosis than SSc-PAH.
Key words: pulmonary hypertension • connective tissue disease • systemic lupus erythematosus • survival • systemic sclerosis




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