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.
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.
Authors: Sangmee Bae; Rajeev Saggar; Marcy B Bolster; Lorinda Chung; Mary Ellen Csuka; Chris Derk; Robyn Domsic; Aryeh Fischer; Tracy Frech; Avram Goldberg; Monique Hinchcliff; Vivien Hsu; Laura Hummers; Elena Schiopu; Maureen D Mayes; Vallerie McLaughlin; Jerry Molitor; Nausheen Naz; Daniel E Furst; Paul Maranian; Virginia Steen; Dinesh Khanna Journal: Ann Rheum Dis Date: 2012-02-02 Impact factor: 19.103
Authors: Rajeev Saggar; Dinesh Khanna; Daniel E Furst; Shelley Shapiro; Paul Maranian; John A Belperio; Neeraj Chauhan; Philip Clements; Alan Gorn; S Sam Weigt; David Ross; Joseph P Lynch; Rajan Saggar Journal: Arthritis Rheum Date: 2010-12
Authors: Lorinda Chung; Juliana Liu; Lori Parsons; Paul M Hassoun; Michael McGoon; David B Badesch; Dave P Miller; Mark R Nicolls; Roham T Zamanian Journal: Chest Date: 2010-05-27 Impact factor: 9.410