Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial

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Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial

BACKGROUND Patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH), in particular systemic sclerosis (SSc), had an attenuated response compared with idiopathic PAH in most trials. Thus, there is uncertainty regarding the benefit of PAH-targeted therapy in some forms of CTD-PAH. OBJECTIVE To explore the safety and efficacy of initial combination therapy wit...

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PReS-FINAL-2267: Successful treatment of pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) with combination therapy of sildenafil and ambrisentan: a case report

Methods At age 7, she developed autoimmune hepatitis. She was diagnosed with lupus nephritis (class II + V) from pathological finding and pulmonary hypertension (PH) at age 10, and treated with immunosuppressive therapy (methylprednisolone pulse therapy, cyclophosphamide pulse therapy and mycophenolate mofetil) and home oxygen therapy at night. Comprehensive examination about PH was carried out...

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Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension.

BACKGROUND Data on the effect of initial combination therapy with ambrisentan and tadalafil on long-term outcomes in patients with pulmonary arterial hypertension are scarce. METHODS In this event-driven, double-blind study, we randomly assigned, in a 2:1:1 ratio, participants with World Health Organization functional class II or III symptoms of pulmonary arterial hypertension who had not pre...

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Connective tissue disease-associated pulmonary arterial hypertension

Although rare in its idiopathic form, pulmonary arterial hypertension (PAH) is not uncommon in association with various associated medical conditions, most notably connective tissue disease (CTD). In particular, it develops in approximately 10% of patients with systemic sclerosis and so these patients are increasingly screened to enable early detection. The response of patients with systemic sc...

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Sildenafil for pulmonary arterial hypertension associated with connective tissue disease.

OBJECTIVE Pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) is difficult to manage, and has a poor prognosis. The phosphodiesterase-5 inhibitor sildenafil citrate enhances vasodilatation, has antiproliferative effects, and is effective in the treatment of PAH. We examined the efficacy and safety of oral sildenafil in patients with PAH-CTD. METHODS In a 12-wee...

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ژورنال

عنوان ژورنال: Annals of the Rheumatic Diseases

سال: 2016

ISSN: 0003-4967,1468-2060

DOI: 10.1136/annrheumdis-2016-210236