European respiratory review : an official journal of the European Respiratory Society
-
Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. ⋯ CPFE is a strong determinant of associated pulmonary hypertension (PH). In addition, CPFE has major effects on measures of physiological function, exercise capacity and prognosis, and may affect the results of pulmonary fibrosis trials. Further studies are needed to ascertain the aetiology, morbidity, mortality and management of the CPFE syndrome, with or without PH, and to evaluate novel therapeutic options in CPFE.
-
Idiopathic pulmonary fibrosis (IPF), the most prevalent of the idiopathic interstitial pneumonias, is associated with a poor prognosis. An accurate diagnosis of IPF is essential for its optimal management. The 2011 American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Latin American Thoracic Association (ALAT) recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. ⋯ However, although the 2011 ATS/ERS/JRS/ALAT statement is a major advance, the application of the guideline recommendations by clinicians has identified limitations that should be addressed in future statements. Key problems include: 1) HRCT misdiagnosis, particularly by less experienced radiologists; 2) lack of management recommendations for the highly prevalent clinical scenarios of "probable" or "possible" IPF; 3) ongoing confusion concerning the diagnostic role of bronchoalveolar lavage; and 4) the lack of integration of clinical data in the designation of the diagnostic likelihood of IPF, including the treated course of disease. These issues become evident as the recommendations are applied and highlight the need for continued guideline adjustments.
-
Flexible bronchoscopy is commonly performed by respiratory physicians and is the gold standard for directly visualising the airways, allowing for numerous diagnostic and therapeutic interventions. With the widespread use of flexible bronchoscopy and the evolution of interventional bronchoscopy with more complex and longer procedures, physicians are placing increasing importance on the use of sedation as a necessary adjunct to topical anaesthesia. ⋯ In addition, there is ongoing debate and controversy about proceduralist-administered versus anaesthetist-administered sedation whilst at the same time there is a growing body of evidence that nonanaesthetist administered sedation is safe and cost-effective. In this review we summarise the evidence for the use of sedation as an adjunct to topical anaesthesia in bronchoscopy and provide the clinician with up-to-date concise guidance for the use of pharmacological sedatives in bronchoscopy and future directions for sedation in the bronchoscopy suite.
-
The aim of this article was to provide practical recommendations to healthcare professionals interested in offering a pulmonary rehabilitation programme for patients with chronic obstructive pulmonary disease (COPD). The latest research findings were brought together and translated into clinical practice. These recommendations focus on the description of useful assessment tests and of the most common exercise modalities for patients with COPD. We provide specific details on the rationale of why and especially how to implement exercise training in patients with COPD, including the prescription of training mode, intensity and duration, as well as suggestions of guidelines for training progression.
-
Recently updated guidelines have provided revised recommendations, based on the GRADE criteria, for the diagnosis and pharmacological and non-pharmacological management of patients with idiopathic pulmonary fibrosis (IPF). Cochrane reviews are also a highly respected and reliable source of evidence-based information that identify and analyse all available data of overall treatment effects from appropriate studies. A recent update of one Cochrane review failed to identify any new evidence supporting the use of corticosteroids in IPF. ⋯ However, the pooled analysis revealed no treatment effect in terms of survival. A further meta-analysis of three phase III studies of pirfenidone treatment in IPF patients suggested a significant increase in progression-free survival. The interpretation of recent international and national European guideline updates and treatment recommendations, available clinical data from published and ongoing trials investigating potential pharmacological agents, and the individual patient's preferences, must be considered in the clinical management of this disease.