Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique. Élaborées par le centre national de référence et les centres de compétence pour les maladies pulmonaires rares sous l’égide de la Société de pneumologie de langue française

Cottin, Vincent; Crestani, Bruno; Valeyre, Dominique; Wallaert, B.; Cadranel, Jacques; Dalphin, J.-C.; Delaval, P.; Israel-Biet, Dominique; Kessler, Romain; Reynaud‐Gaubert, Martine; Cordier, J.-F.; Aguilaniu, B.; Bouquillon, B.; Carré, Philippe; Danel, Claire; Faivre, J; Ferreti, Gian Carlo; Just, N.; Kouzan, S; Lebargy, F.; Marchand-Adam, S.; Philippe, B.; Prévôt, Grégoire; Stach, B.; Thivolet‐Béjui, F.

doi:10.1016/j.rmr.2013.09.007

Cited by 55 publications

(30 citation statements)

References 128 publications

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“…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].…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Discussionmentioning

confidence: 99%

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

Cottin

2014

Eur Respir Rev

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This review presents the results of the 2013 Advancing IPF Research (AIR) survey, which assessed current approaches to the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) by experienced physicians. A total of 149 physicians, predominantly from European countries, replied to the 28-question survey. The results of the AIR survey were compared with a similar survey of 509 French pulmonologists conducted by the French National Reference Centre and the Network of Regional Competence Centres for Rare Lung Diseases.A number of positive findings emerged from the AIR survey, including the high level of multidisciplinary team involvement in both diagnosis and management. This survey, when taken together with the French survey, suggests that there is still a need to improve earlier diagnosis of IPF. @ERSpublications Survey on IPF in Europe reveals need to improve early diagnosis and management

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

Cottin

2014

Eur Respir Rev

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“…Other than lung transplantation, we have no specific guidelines for treating pulmonary fibrosis associated with telomere diseases, and the usual recommendations are advised [109].…”

Section: Indication For Genetic Diagnosismentioning

confidence: 99%

Management of suspected monogenic lung fibrosis in a specialised centre

et al. 2017

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At least 10% of patients with interstitial lung disease present monogenic lung fibrosis suspected on familial aggregation of pulmonary fibrosis, specific syndromes or early age of diagnosis. Approximately 25% of families have an identified mutation in genes mostly involved in telomere homeostasis, and more rarely in surfactant homeostasis.Beyond pathophysiological knowledge, detection of these mutations has practical consequence for patients. For instance, mutations involved in telomere homeostasis are associated with haematological complications after lung transplantation and may require adapted immunosuppression. Moreover, relatives may benefit from a clinical and genetic evaluation that should be specifically managed.The field of genetics of pulmonary fibrosis has made great progress in the last 10 years, raising specific problems that should be addressed by a specialised team.

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“…The recommendations were published in December 2013 [4]. Herein, we summarise the main conclusions and practical recommendations of the French guidelines.…”

Section: Guidelines Development Processmentioning

confidence: 97%

“…The present document is an English version of the executive summary and outlines the main conclusions of the French guidelines for the diagnosis and management of IPF [4].…”

Section: Introductionmentioning

confidence: 99%

Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines

Cottin¹,

Crestani²,

Valeyre³

et al. 2014

Eur Respir Rev

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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

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Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique. Élaborées par le centre national de référence et les centres de compétence pour les maladies pulmonaires rares sous l’égide de la Société de pneumologie de langue française

Abstract: CCBYNational audienc

Cited by 55 publications

References 128 publications

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

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

Management of suspected monogenic lung fibrosis in a specialised centre

Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines

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