Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most frequent chronic idiopathic interstitial pneumonia in adults. The management of rare diseases in France has been organised by a national plan for rare diseases, which endorsed a network of expert centres for rare diseases throughout France. This article is an overview of the executive summary of the French guidelines for the management of IPF, an initiative that emanated from the French National Reference Centre and the Network of Regional Competence Centres for Rare Lung Diseases. This review aims at providing pulmonologists with a document that: 1) combines the current available evidence; 2) reviews practical modalities of diagnosis and management of IPF; and 3) is adapted to everyday medical practice. The French practical guidelines result from the combined efforts of a coordination committee, a writing committee and a multidisciplinary review panel, following recommendations from the Haute Autorité de Santé. All recommendations included in this article received at least 90% agreement by the reviewing panel. Herein, we summarise the main conclusions and practical recommendations of the French guidelines. @ERSpublications Practical guidelines for idiopathic pulmonary fibrosis are now available

Current approaches to the diagnosis and treatment of idiopathic pulmonary fibrosis in Europe: the AIR survey

“…Over twice as many French survey participants carried out genetic testing compared with AIR survey participants, reflecting potential variation in the attitudes of physicians in both surveys and in the awareness of possible genetic causes of IPF. The French guidelines for IPF diagnosis and management recommend that, when a diagnosis of IPF is suspected in a patient, clinicians should ''systematically'' search for other causes of diffuse interstitial pneumonia in the family, including clinical signs suggestive of a genetic cause [11,12]. Furthermore, the guidelines propose that patients who present with IPF in a familial context should be referred to an outpatient clinic specialising in genetics to establish a pedigree and plan genetic molecular analysis [11,12].…”

“…The French guidelines for IPF diagnosis and management recommend that, when a diagnosis of IPF is suspected in a patient, clinicians should ''systematically'' search for other causes of diffuse interstitial pneumonia in the family, including clinical signs suggestive of a genetic cause [11,12]. Furthermore, the guidelines propose that patients who present with IPF in a familial context should be referred to an outpatient clinic specialising in genetics to establish a pedigree and plan genetic molecular analysis [11,12]. Since the French guidelines (English version) were published after the AIR survey results, it is hoped their adoption into clinical practice will, in future, result in a greater level of homogeneity in practice regarding genetic testing for patients with suspected familial IPF.…”

Severe hematologic complications after lung transplantation in patients with telomerase complex mutations

“…The clinical charts of the patients were reviewed and data were collected on a standardized and anonymous collection form. Chest CT scans, biopsies and explants were systematically classified based on a multidisciplinary team discussion in each of the 4 expert centers involved in this study and classified according to the 2011 official ATS/ERS/JRS/ALAT statement for IPF [2,10] and the revised classification of idiopathic interstitial pneumonia [11]. Haematogical manifestations including myelodysplastic syndromes were classified according to the 2008 WHO classification [12].…”

Real-World Practice Patterns for Prevention and Management of Potential Adverse Events with Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis

“…Recommendations for managing photosensitivity include use of sunscreen, sun avoidance, and wearing a hat and UPF clothing. These strategies also align with recommendations in the prescribing information as well as those advised by leading experts in the field [6,9,12,21]. Data from this survey suggest that sunscreen use in patients who spend time outdoors may need to be recommended more extensively, particularly in regions such as the Southeast, where sunshine hours are elevated.…”