Seminars in arthritis and rheumatism
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Semin. Arthritis Rheum. · Feb 2017
Changes in gene expression profiles in patients with pulmonary arterial hypertension associated with scleroderma treated with tadalafil.
Pulmonary arterial hypertension (PAH) is one of the most devastating complications in scleroderma (SSc) patients and has a poorer outcome than other PAH subgroups. Tadalafil (Adcirca®) is a phosphodiesterase-5 inhibitor (PDE5-I) approved by the FDA for treatment of PAH; however, its effectiveness specifically in SSc-PAH patients is unclear. We investigated whether there were differences in gene expression associated with 16 weeks of treatment with tadalafil and, if so, whether these changes differed with respect to treatment outcome. ⋯ The results of this pilot study suggest that outcomes of SSc-PAH patients treated with tadalafil are associated with specific changes in gene expression and biological pathways.