Ambrisentan response in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) – A subgroup analysis of the ARIES-E clinical trial
نویسندگان
چکیده
منابع مشابه
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...
متن کاملPReS-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...
متن کاملConnective 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...
متن کاملConnective tissue disease-associated pulmonary arterial hypertension in Chinese patients.
We sought to investigate the characteristics, survival and risk factors for mortality in Chinese patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) in modern therapy era. 129 consecutive adult patients who visited one of three referral centres in China with a diagnosis of CTD-APAH confirmed by right heart catheterisation during the previous 5 years w...
متن کاملConnective tissue disease-associated pulmonary arterial hypertension: "Beijing style".
Patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) experience poorer outcomes than those with idiopathic PAH (IPAH) [1–6]. In the USA, patients with systemic sclerosis (SSc) are most commonly affected by CTD-APAH and have the worst survival [1]. Similarly, 74% of a large cohort with CTD-APAH in the UK had SSc, and these patients had poorer survival t...
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ژورنال
عنوان ژورنال: Respiratory Medicine
سال: 2016
ISSN: 0954-6111
DOI: 10.1016/j.rmed.2016.06.018