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The final article was written by a multidisciplinary group of experts invited by the World Association of Sarcoidosis and other Granulomatous Disorders (WASOG). Radiological definition and clinical implications are reviewed in detail. Tomassetti and co-workers reviews the challenging and poorly understood topic of interstitial lung abnormalities and its possible progression to idiopathic pulmonary fibrosis or to progressive fibrosing interstitial lung disease. The use of inotropes and of other drugs is a critical issue that is also reviewed carefully, together with the intraoperative management and monitoring, as well as ventilation and post-operative care. Haemodynamic ventricular interdependence and the possible causes of right ventricular failure and of its prevention are addressed in detail.

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When compared with elective surgery, emergency noncardiac procedures are associated with more perioperative complications and increased likelihood of worse outcome. address the challenge of the perioperative management of patients with precapillary pulmonary hypertension (PH) undergoing noncardiac non-obstetric surgery. The authors also discuss the controversial issue of the therapeutic approach for this “research entity” and state that “while identification of a histological NSIP pattern would support an immunosuppressive approach, histological confirmation of a UIP pattern would lead to a more cautious approach to immunosuppression, with frequent monitoring and earlier consideration of anti-fibrotic therapy, should there be progression”. Airway and vascular involvement are discussed in detail, as well as the problem of severe vasculopathy that can characterise some IPAF patients. Following the publication, in 2015, of the classification of IPAF proposed by the European Respiratory Society (ERS) and the American Thoracic Society (ATS), a number of articles have been published on this entity which truly requires a multidisciplinary approach. review the challenges and controversies (which are not few!) in “interstitial pneumonia with autoimmune features” (IPAF). In this issue, we publish the first of a collection of four articles that discuss hot topics with direct practical clinical consequences. Dissemination of updated knowledge on rare and ultra-rare respiratory diseases has always been a priority of the European Respiratory Review ( ERR).







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