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

Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom.
American Journal of Respiratory and Critical Care Medicine (Impact Factor: 13). 10/2008; 179(2):151-7. DOI: 10.1164/rccm.200806-953OC
Source: PubMed


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.
To investigate the survival and characteristics of all patients diagnosed with CTD-PAH in the U.K. pulmonary hypertension service.
National registry of all incident cases of CTD-PAH diagnosed consecutively between January 2001 and June 2006.
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).
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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Available from: John Gerry Coghlan, Sep 20, 2015
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    • "Pulmonary hypertension (PH), characterized by increased pulmonary vascular resistance, remodelling of the small pulmonary arteries and right ventricular hypertrophy (RVH), is a common complication in IPF (affecting 30–40% of patients), and individuals with a dual diagnosis exhibit markedly reduced survival (Lettieri et al., 2006; Mejia et al., 2009). PH is also frequently associated with other ILDs, including systemic sclerosis-driven pulmonary fibrosis, where it also results in increased mortality (Behr and Ryu, 2008; Condliffe et al., 2009). IPF is refractory to virtually all current therapeutic options and the recently licensed anti-fibrotic, antiinflammatory drug pirfenidone achieves only a small treatment benefit at high financial cost (Noble et al., 2011). "
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