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

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

Correspondence and requests for reprints should be addressed to J. G. Coghlan, M.D., Department of Cardiology, Royal Free Hospital, London NW3 2QG, UK. E-mail: gerry.coghlan{at}royalfree.nhs.uk

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.

Key Words: pulmonary hypertension • connective tissue disease • systemic sclerosis • systemic lupus erythematosus • survival

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Systemic sclerosis (SSc)–associated pulmonary arterial hypertension (PAH) has historically had a poor outcome. Little is known of outcomes in exercise-induced and respiratory disease–associated SSc-PAH, or in PAH due to other connective tissue diseases. What This Study Adds to the Field Survival of patients with SSc-PAH in the modern treatment era is better than in historical series. Survival of patients with respiratory disease–associated pulmonary hypertension is inferior. SLE-PAH has a better prognosis than SSc-PAH.

 

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