TY - JOUR
T1 - Update in systemic sclerosis-associated pulmonary arterial hypertension
AU - Gashouta, Mohamed A.
AU - Humbert, Marc
AU - Hassoun, Paul M.
N1 - Publisher Copyright:
© 2014 Elsevier Masson SAS.
PY - 2014/10/1
Y1 - 2014/10/1
N2 - Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Despite advances in treatment options for PAH, long-term prognosis remains poor for scleroderma-associated PAH (SSc-PAH). Although prompt diagnosis and treatment of PAH may have significant impact on survival rates, early detection of the syndrome continues to be challenging in SSc due to several factors ranging from limitations of the current screening tools and the complexities of the disease. In comparison with other PAH subgroups, SSc-PAH patients respond poorly to conventional forms of PAH therapy. Recent findings indicate that factors such as autoimmune and inflammatory responses, more severe vasculature remodeling, and intrinsic cardiac involvement may account for these differences.
AB - Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Despite advances in treatment options for PAH, long-term prognosis remains poor for scleroderma-associated PAH (SSc-PAH). Although prompt diagnosis and treatment of PAH may have significant impact on survival rates, early detection of the syndrome continues to be challenging in SSc due to several factors ranging from limitations of the current screening tools and the complexities of the disease. In comparison with other PAH subgroups, SSc-PAH patients respond poorly to conventional forms of PAH therapy. Recent findings indicate that factors such as autoimmune and inflammatory responses, more severe vasculature remodeling, and intrinsic cardiac involvement may account for these differences.
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U2 - 10.1016/j.lpm.2014.06.007
DO - 10.1016/j.lpm.2014.06.007
M3 - Short survey
C2 - 25179278
AN - SCOPUS:84908401106
VL - 43
SP - e293-e304
JO - Nouvelle Presse Medicale
JF - Nouvelle Presse Medicale
SN - 0755-4982
IS - 10
ER -