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
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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