European respiratory review : an official journal of the European Respiratory Society
-
Review
Changing the idiopathic pulmonary fibrosis treatment approach and improving patient outcomes.
Idiopathic pulmonary fibrosis (IPF) is a progressively fibrotic disease, with no effective treatment and a median survival time of 2-5 yrs. The search for effective treatment has involved numerous clinical trials of investigational agents without significant success until 2011, when European approval was given for the first treatment for IPF, pirfenidone. Four key clinical trials supported the efficacy and tolerability of pirfenidone. ⋯ Evidence of beneficial effects of pirfenidone treatment was also observed with regard to several secondary end-points, including progression-free survival time, categorical FVC change, and mean change from baseline to week 72 in 6MWT distance. Pirfenidone was generally well tolerated, with the most common side-effects being gastrointestinal discomfort and photosensitivity. The pooled study results, coupled with recent data regarding the prognostic significance of changes in FVC and 6MWT, provide further evidence of a clinically meaningful treatment benefit with pirfenidone in patients with IPF.
-
Review Case Reports
State-of-the-art chronic thromboembolic pulmonary hypertension diagnosis and management.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a debilitating disease caused by chronic obstruction of pulmonary artery branches following episodes of pulmonary embolism and incomplete thrombus resolution. The prognosis of patients with CTEPH is poor unless an early diagnosis is made and treatment is initiated. Chest radiography and echocardiography are used in the initial assessment of suspected pulmonary hypertension. ⋯ However, many centres utilise CT and magnetic resonance imaging following recent advances in these noninvasive techniques. Haemodynamic evaluation via right heart catheterisation is also mandatory, as pulmonary vascular resistance is the most important determinant of both prognosis and the risk associated with pulmonary endarterectomy surgery. Accurate CTEPH diagnosis and characterisation of its extent and distribution are imperative to allow the prompt initiation of treatment, particularly surgical pulmonary endarterectomy in eligible patients.
-
Letter Case Reports
Asymptomatic bilateral pulmonary embolism in Churg-Strauss syndrome.