Long-term outcomes of domiciliary intravenous iloprost in idiopathic and connective tissue disease-associated pulmonary arterial hypertension

Respirology ◽  
2016 ◽  
Vol 22 (2) ◽  
pp. 372-377 ◽  
Author(s):  
Sheila Ramjug ◽  
Nehal Hussain ◽  
Judith Hurdman ◽  
Charlie A. Elliot ◽  
Ian Sabroe ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Long Term Outcomes ◽  
Pulmonary Arterial

Pulmonary arterial hypertension associated with connective tissue disease

ESC CardioMed ◽  
2018 ◽  
pp. 2531-2534
Author(s):  
Christopher P. Denton
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Heart Catheterization ◽  
Pulmonary Arterial ◽  
Detection And Diagnosis ◽  
Health Organization

Connective tissue disease-associated pulmonary arterial hypertension (PAH) falls within World Health Organization group 1. These patients are treated as others in this group, but there are important considerations regarding detection and diagnosis. Patients with connective tissue disease are at risk of PAH and should be screened with confirmation of diagnosis by right heart catheterization. Treatment follows the European Society of Cardiology guidelines for other forms of PAH. However, more information is available for systemic sclerosis PAH regarding screening, including the DETECT algorithm, and also in terms of long-term outcome of patients with borderline elevation of mean pulmonary arterial pressure. In cases of systemic lupus erythematosus or mixed connective tissue disease, immunosuppression should be given in conjunction with targeted PAH-specific therapy. Long-term outcomes for PAH in patients with connective tissue disease have improved since targeted specific therapies became available. Recent trials with morbidity–mortality endpoints and a high proportion of patients receiving combination treatment have shown comparable benefits for patients with connective tissue disease and PAH as for those with idiopathic PAH in contrast to the blunted response that was characteristic of earlier short-term studies assessing improvement in 6 min walk test distance.

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