French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2017 update. Short-length version

Cottin, Vincent; Crestani, Bruno; Cadranel, Jacques; Cordier, J.-F.; Marchand-Adam, S.; Prévôt, Grégoire; Wallaert, B.; Camus, P.; Dalphin, J.-C.; Dromer, C.; Gomez, E.; Israel-Biet, Dominique; Jouneau, S.; Kessler, Romain; Marquette, Charles‐Hugo; Reynaud‐Gaubert, Martine; Aguilaniu, B.; Bonnet, D.; Carré, Philippe; Danel, Claire; Faivre, J; Ferretti, G.; Just, N.; Lebargy, F.; Philippe, B.; Terrioux, P.; Thivolet‐Béjui, F.; Trumbic, B.; Valeyre, Dominique

doi:10.1016/j.rmr.2017.07.019

Cited by 5 publications

(5 citation statements)

References 135 publications

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“…Placeholder for table 13 and references cited in table 13 285,290 Treatment of pulmonary fibrosis Decisions about pharmacologic treatment are guided by the underlying diagnosis of fILD 288 .…”

Section: Management General Measuresmentioning

confidence: 99%

Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement

Cottin¹,

Selman²,

Inoue³

et al. 2022

Am J Respir Crit Care Med

105

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Background. The presence of emphysema is relatively common in patients with fibrotic interstitial lung disease. This has been designated combined pulmonary fibrosis and emphysema (CPFE). The lack of consensus over definitions and diagnostic criteria has limited CPFE research. Goals.The objectives of this taskforce were to review the terminology, definition, characteristics, pathophysiology, and research priorities of CPFE, and to explore whether CPFE is a syndrome.Methods. This research statement was developed by a committee including 19 pulmonologists, 5 radiologists, 3 pathologists, 2 methodologists, and 2 patient representatives. The final document was supported by a focused systematic review that identified and summarized all recent publications related to CPFE.Results. This taskforce identified that patients with CPFE are predominantly male, with history of smoking, severe dyspnea, relatively preserved airflow rates and lung volumes on spirometry, severely impaired diffusion capacity for carbon monoxide, exertional hypoxemia, frequent pulmonary hypertension, and a dismal prognosis. The committee proposes to identify CPFE as a syndrome given the clustering of pulmonary fibrosis and emphysema, shared pathogenetic pathways, unique considerations related to disease progression, increased risk of complications (pulmonary hypertension, lung cancer, mortality), and implications for clinical trial design. There are varying features of interstitial lung disease and emphysema in CPFE. The committee offers a research definition and classification criteria, and proposes that studies on CPFE include a comprehensive description of radiologic and, when available, pathological patterns including some recently described patterns such as smoking-related interstitial fibrosis.Conclusions. This statement delineates the syndrome of CPFE and highlights research priorities.

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Section: Management General Measuresmentioning

confidence: 99%

Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement

Cottin¹,

Selman²,

Inoue³

et al. 2022

Am J Respir Crit Care Med

105

View full text Add to dashboard Cite

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“…In addition, a program dedicated to lung cancer and ILD screening could be proposed to SRG variant carriers, the modalities of which remain to be determined. Low-dose annual chest CT scan is now recommended for lung cancer screening in high-risk populations in several countries 40 and might be evaluated for SRG variant carriers. The age at the first CT scan and the frequency of repeated assessments need to be carefully considered because of the unknown long-term risk associated with irradiation in this relatively young population.…”

Section: Discussionmentioning

confidence: 99%

“…In total, 101 adults with a diagnosis of SRG-associated ILD during 2008-2022 were referred for inclusion; we included 99 adults with a pathogenic SRG variant (SFTPA1, SFTPA2, SFTPC, ABCA3 or NKX2-1) (Table 1), including 74 who were previously reported (Figure S1) 15,21,23,27,39 40 , and 15 after genetic counseling in a context of familial ILD. The 99 patients belonged to 69 families with 1 to 10 members per family.…”

Section: Srg Cohort Patient Characteristicsmentioning

confidence: 99%

High risk of lung cancer in surfactant-related gene variant carriers

Brudon,

Legendre,

Mageau

et al. 2024

Eur Respir J

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RationaleSeveral rare surfactant-related genes variants associated with interstitial lung disease are suspected to be associated with lung cancer, but data are missing.ObjectivesWe aimed to study the epidemiology and phenotype of lung cancer in an international cohort of surfactant-related gene (SRG) variant carriers.MethodsWe conducted a cross-sectional study of all adults with SRG variants in the OrphaLung network and compared lung cancer risk with telomerase-related gene (TRG) variant carriers.ResultsWe identified 99 SRG adult variant carriers (SFTPA1[n=18],SFTPA2[n=31],SFTPC[n=24],ABCA3[n=14] andNKX2–1[n=12]),including 20 (20.2%) with lung cancer (SFTPA1[n=7]; SFTPA2[n=8],SFTPC[n=3],NKX2–1[n=2] andABCA3[n=0]). Among SRG variant carriers, the odds of lung cancer was associated with age (odds ratio [OR] 1.04 [95% CI 1.01–1.08]), smoking (OR 20.7 [6.60–76.2]) andSFTPA1/SFTPA2variants (OR 3.97 [1.39–13.2]). Adenocarcinoma was the only histological type reported, with PDL1 expression≥1% of tumor cells in 3 cases. Cancer staging was localized (I/II) in 8 (40%) individuals, locally advanced (III) in 2 (10%) and metastatic (IV) in 10 (50%). We found no somatic variant eligible for targeted therapy. Seven cancers were surgically removed, 10 received systemic therapy, and 3 received the best supportive care according to their stage and performance status. The median overall survival was 24 months, with stage I/II cancers showing better survival. We identified 233 TRG variant carriers. The comparative risk (subdistribution hazard ratio) for lung cancer in SRG patientsversusTRG patients was 18.1 [7.1–44.7].ConclusionThe high risk of lung cancer among SRG variant carriers suggests specific screening and diagnostic and therapeutic challenges. The benefit of regular CT scan follow-up should be evaluated.

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“…Le diagnostic de la FPI est désormais bien codifié, et implique des pneumologues, des radiologues et des anatomo-pathologistes spécialisés, au sein d'équipes multidisciplinaires [17,18]. Il repose sur la présence de signes spécifiques observés sur un scanner thoracique : aspect dit de pneumopathie interstitielle commune, associant des réticulations, des bronchectasies par traction, des images en rayon de miel, avec une distribution sous-pleurale et un gradient apico-dorsal, ainsi que l'exclusion de toute autre cause de fibrose pulmonaire (Figure 3).…”

Section: Le Diagnostic De Fpiunclassified

La fibrose pulmonaire idiopathique

et al. 2022

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La fibrose pulmonaire idiopathique (FPI) est une maladie pulmonaire chronique, évolutive et mortelle dont l’origine est inconnue. Elle se caractérise par une cicatrisation aberrante de l’épithélium alvéolaire aboutissant à une accumulation de matrice extracellulaire (MEC). Les foyers fibroblastiques, constitués de fibroblastes et de myofibroblastes, sont responsables de la production excessive de MEC. Les deux seules molécules thérapeutiques disponibles sur le marché permettent seulement de ralentir l’évolution de la maladie. Dans cette revue, nous présentons les mécanismes impliqués dans la progression de la maladie, ses traitements et les modèles d’étude.

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French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2017 update. Short-length version

Cited by 5 publications

References 135 publications

Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement

Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement

High risk of lung cancer in surfactant-related gene variant carriers

La fibrose pulmonaire idiopathique

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