Review of IPF diagnosis and management recommendations in Europe

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Antoni Xaubet
Jϋrgen Behr
Elisabeth Bendstrup
Vincent Cottin
Nik Hirani
Christian Kähler
Magnus Sköld


IPF, Diagnosis, Guidelines, Recommendations, Treatment


Increased knowledge of Idiopathic Pulmonary Fibrosis (IPF) led to the development of evidence-based diagnosis and treatment guidelines. A 2011 update of the American Thoracic Society and the European Respiratory Society, together with the Japanese Respiratory Society ( JRS) and Latin AmericanThoracic Association (ALAT) provided new guidance on the diagnosis and treatment of IPF. Although the 2011 statement was a major advance, the application of guideline recommendations has identified limitations.The guidelines focus primarily on ‘definite’ IPF, most often diagnosed from typical High-Resolution Computed Tomography (HRCT) appearances.The definition of ‘probable’ and ‘possible’ IPF is an advance, but there is a lack ofmanagement guidance for these highly prevalent clinical scenarios.The integration ofHRCT and histological data in assigning of diagnostic likelihood is also important, but does not always meet the needs of some patients in whom a multidisciplinary diagnosis of definite IPF should be made.Moreover, the committee did not find sufficient evidence to support the use of any specific pharmacological therapy for patients with IPF.These issues highlight the need for updating available clinical guidelines. Since 2012, several national European recommendations documents and guidelines have been updated.These generally follow the 2011 guidelines, but reflect more recently available clinical study data. Following the publication of the CAPACITY trials showing positive effects of pirfenidone in IPF and its approval in the European Union,many of these updated guideline documents recommend that patients with mild-to-moderate IPF should be offered this therapy. This review analyses the recently developed European country updates, comparing and contrasting recommendations on the diagnosis and treatment of IPF.
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