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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    British Journal of Pharmacology 03/2014; 171(14). DOI:10.1111/bph.12694 · 4.84 Impact Factor
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    • "The early detection of SSc-PAH has emerged as an essential component of disease management. A number of studies have demonstrated the significantly better prognosis of patients presenting in lower World Health Organization functional classes (WHO-FC) (that is I and II), compared to patients presenting with more advanced functional impairment (WHO-FC III or IV) [3,4]. Other studies have suggested that early commencement of therapy may delay the progression of SSc-PAH, and lead to improvements in functional class [5,6]. "
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    Arthritis research & therapy 11/2013; 15(6):R193. DOI:10.1186/ar4383 · 3.75 Impact Factor
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    • "In addition, it has been reported that during pulmonary hypertension, plasminogen activator inhibitor-1 (PAI-1) levels are high, suggesting a loss of fibrinolytic activity [11], however, PAI-1 was not determinate in our patient. The hemodynamic pattern has also been described by Condliffe et al. [12] who studied patients with connective tissue diseases and pulmonary arterial hypertension (PAH), as well as 28 patients with isolated SLE and PAH. Murata et al. [13] reported a case of PAH with Raynaud phenomenon and relative polycythemia where the right cardiac catheterization showed PAH with pulmonary artery pressures of 93/39mmHg (mean pressure 60mmHg), cardiac index of 2.2L/minute/m2, and a pulmonary vascular resistance index of 1219dyne·second/(cm5·m2). "
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