Sildenafil in idiopathic pulmonary fibrosis.

N Engl J Med

Published: November 2010

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http://dx.doi.org/10.1056/NEJMc1010408DOI Listing

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Article Synopsis
  • Pulmonary veno-occlusive disease (PVOD) has a poorer prognosis than idiopathic pulmonary arterial hypertension (PAH), with median survival of only 1 year post-diagnosis.
  • Treatments for PAH have shown limited effectiveness for PVOD, with risks such as pulmonary oedema and mixed results from case reports on medication effects.
  • Lung transplantation is the best long-term treatment for suitable patients, but candidates over a certain age or with certain cancers are typically excluded; a case study describes a successful 5-year sildenafil treatment for a 59-year-old patient with PVOD and multiple myeloma.
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A Case of Interstitial Lung Disease-Related Pulmonary Hypertension Successfully Treated with Inhaled Iloprost.

Life (Basel)

August 2024

Division of Pulmonology and Critical Care Medicine, Department of Internal Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine, Busan 48108, Republic of Korea.

Pulmonary hypertension (PH) associated with interstitial lung disease (ILD) (PH-ILD) significantly worsens clinical symptoms and survival, with no effective treatment available. This case report presents the successful treatment of PH-ILD with inhaled iloprost in a patient with idiopathic pulmonary fibrosis (IPF). The patient, a 68-year-old female, was diagnosed with IPF in 2018 and was maintained on pirfenidone.

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Introduction: Venoarterial extracorporeal membrane oxygenation (ECMO) is a rescue therapy that can stabilize patients with hemodynamic compromise. Indications continue to evolve, including drug overdose. However, the indication merely for vasoplegic shock following drug overdose is controversial.

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Objective: Randomized controlled trials(RCTs) of multiple drugs for Idiopathic pulmonary fibrosis(IPF) have been reported and achieved a certain degree of efficacy, however, the difference in safety and efficacy of them for IPF is not yet well understood. The aim of this network meta-analysis is to assess their safety and efficacy in the treatment of IPF and differences in this safety and efficacy comprehensively.

Methods: The PubMed, EMbase, CENTRAL and MEDLINE were retrieved to find out the RCTs of drugs in the treatment of IPF.

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Background: Achieving and maintaining a low-risk profile is associated with favorable outcome in pulmonary arterial hypertension (PAH). The effects of treatment on risk profile are variable among patients.

Objective: To Identify variables that might predict the response to treatment with phosphodiesterase-5 inhibitors (PDE-5i) in PAH.

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