Review of IPF diagnosis and management recommendations in Europe

Review of IPF diagnosis and management recommendations in Europe

Authors

  • Antoni Xaubet Servicio de Neumología, Hospital Clínic, Barcelona, Spain; Centro de Investigaciones Biomédicas en Red de Enfermedades Respiratorias (CIBERES), Spain
  • Jϋrgen Behr Asklepios Fachkliniken München Gauting, Medizinischen Klinik und Poliklinik V, Comprehensive Pneumology Center, Klinikum der Ludwig-Maximilians-Universität, Munich, Germany
  • Elisabeth Bendstrup Department of Respiratory Medicine and Allergology, Aarhus University Hospital, Aarhus, Denmark
  • Vincent Cottin Hospices Civils de Lyon, Hôpital Louis Pradel, Service de pneumologie – Centre de référence national des maladies pulmonaires rares, Université Claude Bernard Lyon 1, Lyon, France
  • Nik Hirani Royal Infirmary Edinburgh, Edinburgh, UK
  • Christian Kähler Pneumology, Internal Medicine VI, Department of Internal Medicine, Innsbruck Medical University, Innsbruck, Austria
  • Magnus Sköld Department of Medicine Solna, Division of Respiratory Medicine, Karolinska Institutet, Stockholm, Sweden

Keywords:

IPF, Diagnosis, Guidelines, Recommendations, Treatment

Abstract

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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Published

17-12-2013

Issue

Section

Review

How to Cite

1.
Xaubet A, Behr J, Bendstrup E, Cottin V, Hirani N, Kähler C, et al. Review of IPF diagnosis and management recommendations in Europe. Sarcoidosis Vasc Diffuse Lung Dis [Internet]. 2013 Dec. 17 [cited 2025 May 19];30(4):249-61. Available from: https://mattioli1885journals.com/index.php/sarcoidosis/article/view/3136