Connective Tissue Disease Associated Pulmonary Arterial Hypertension: Special Cases?

2009 ◽  
pp. 305-314
Author(s):  
Gerry Coghlan
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Special Cases ◽  
Pulmonary Arterial

scholarly journals Replacing a phosphodiesterase-5 inhibitor with riociguat in patients with connective tissue disease-associated pulmonary arterial hypertension: a case series

2017 ◽  
Vol 7 (3) ◽  
pp. 741-746 ◽  
Author(s):  
Amresh Raina ◽  
Raymond L. Benza ◽  
Harrison W. Farber
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Clinical Response ◽  
Connective Tissue Disease ◽  
Case Series ◽  
Response To Treatment ◽  
Tolerability Profile ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Pulmonary Arterial

Patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-PAH-CTD) such as systemic sclerosis (SSc) have a poorer response to treatment and increased mortality compared with patients with idiopathic PAH. Current treatment options for PAH-CTD include prostanoids, phosphodiesterase type-5 inhibitors (PDE-5i), endothelin receptor antagonists, and the soluble guanylate cyclase stimulator riociguat. In this case series, we describe three patients with PAH-CTD related to limited scleroderma who were switched from a PDE-5i to riociguat due to insufficient clinical response. The switch to riociguat was associated with an improvement in respiratory and hemodynamic parameters and a favorable tolerability profile. These cases demonstrate that switching to riociguat is a therapeutic option in patients with PAH-CTD who have not achieved a satisfactory clinical response to a PDE-5i.

scholarly journals Pulmonary arterial hypertension associated with connective tissue disease: meta-analysis of clinical trials

BMJ Open ◽  
2013 ◽  
Vol 3 (8) ◽  
pp. e003113 ◽  
Author(s):  
Masataka Kuwana ◽  
Hiroshi Watanabe ◽  
Nobushige Matsuoka ◽  
Naonobu Sugiyama
Keyword(s):  
Clinical Trials ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Meta Analysis ◽  
Pulmonary Arterial

scholarly journals Clinical experience with bosentan and sitaxentan in connective tissue disease-associated pulmonary arterial hypertension

Rheumatology ◽  
2010 ◽  
Vol 49 (11) ◽  
pp. 2147-2153 ◽  
Author(s):  
C. J. Valerio ◽  
C. E. Handler ◽  
P. Kabunga ◽  
C. J. Smith ◽  
C. P. Denton ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Clinical Experience ◽  
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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