Respirology : official journal of the Asian Pacific Society of Respirology
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Treatment with pirfenidone may slow the decline in vital capacity and increase progression-free survival (PFS) in idiopathic pulmonary fibrosis (IPF). The effects of combination therapy with inhaled N-acetylcysteine (NAC) and pirfenidone are unclear. We assessed the effects of this combination therapy in patients with advanced IPF. ⋯ Combination treatment with inhaled NAC and oral pirfenidone reduced the rate of annual FVC decline and improved PFS in patients with advanced IPF.
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In this study, we evaluated survival in rheumatoid arthritis-associated pulmonary arterial hypertension (RA-PAH) compared with idiopathic pulmonary arterial hypertension (IPAH) patients, and evaluate differences in disease severity and treatment. ⋯ Compared with IPAH patients, RA-PAH patients have an older age of onset and lower baseline mPAP. RA-PAH patients have comparable survival to IPAH patients.