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
نویسندگان
چکیده
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 at age 12.
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 کاملSildenafil dosed concomitantly with bosentan for adult pulmonary arterial hypertension in a randomized controlled trial
BACKGROUND Few controlled clinical trials exist to support oral combination therapy in pulmonary arterial hypertension (PAH). METHODS Patients with PAH (idiopathic [IPAH] or associated with connective tissue disease [APAH-CTD]) taking bosentan (62.5 or 125 mg twice daily at a stable dose for ≥3 months) were randomized (1:1) to sildenafil (20 mg, 3 times daily; n = 50) or placebo (n = 53). The...
متن کاملPulmonary arterial hypertension associated with connective tissue disease: meta-analysis of clinical trials
OBJECTIVES Few studies have focused on pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTDs). The optimal treatment for CTD-PAH has yet to be established. DESIGN Meta-analysis of the data from evaluations of treatment for PAH generally (19 studies) and CTD-PAH specifically (nine studies) to compare the effects of pulmonary vasodilative PAH agents. MEDLINE, EM...
متن کاملUpdate on the clinical utility of sildenafil in the treatment of pulmonary arterial hypertension
Sildenafil is an orally administered phosphodiesterase type 5 inhibitor that is approved for the treatment of pulmonary arterial hypertension (PAH). The hemodynamic effects of sildenafil are mitigated primarily via potentiating the effects of endogenous nitric oxide, leading to smooth muscle cell relaxation and reductions in pulmonary arterial pressures and pulmonary vascular resistance. When a...
متن کاملSildenafil 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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