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Familial idiopathic pulmonary fibrosis

MedGen UID:
1667203
•Concept ID:
C4721952
•
Disease or Syndrome
Synonyms: Familial Idiopathic Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis, Familial
SNOMED CT: Familial idiopathic pulmonary fibrosis (426437004)
 
OMIM®: 178500

Professional guidelines

PubMed

Progressive pulmonary fibrosis: an expert group consensus statement.
Rajan SK, Cottin V, Dhar R, Danoff S, Flaherty KR, Brown KK, Mohan A, Renzoni E, Mohan M, Udwadia Z, Shenoy P, Currow D, Devraj A, Jankharia B, Kulshrestha R, Jones S, Ravaglia C, Quadrelli S, Iyer R, Dhooria S, Kolb M, Wells AU
Eur Respir J 2023 Mar;61(3) Epub 2023 Mar 30 doi: 10.1183/13993003.03187-2021. PMID: 36517177Free PMC Article
Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.
Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, Kreuter M, Lynch DA, Maher TM, Martinez FJ, Molina-Molina M, Myers JL, Nicholson AG, Ryerson CJ, Strek ME, Troy LK, Wijsenbeek M, Mammen MJ, Hossain T, Bissell BD, Herman DD, Hon SM, Kheir F, Khor YH, Macrea M, Antoniou KM, Bouros D, Buendia-Roldan I, Caro F, Crestani B, Ho L, Morisset J, Olson AL, Podolanczuk A, Poletti V, Selman M, Ewing T, Jones S, Knight SL, Ghazipura M, Wilson KC
Am J Respir Crit Care Med 2022 May 1;205(9):e18-e47. doi: 10.1164/rccm.202202-0399ST. PMID: 35486072Free PMC Article
Idiopathic pulmonary fibrosis: Current and future treatment.
Glass DS, Grossfeld D, Renna HA, Agarwala P, Spiegler P, DeLeon J, Reiss AB
Clin Respir J 2022 Feb;16(2):84-96. Epub 2022 Jan 10 doi: 10.1111/crj.13466. PMID: 35001525Free PMC Article

Recent clinical studies

Etiology

Genomic Fingerprint Associated with Familial Idiopathic Pulmonary Fibrosis: A Review.
Ding D, Gao R, Xue Q, Luan R, Yang J
Int J Med Sci 2023;20(3):329-345. Epub 2023 Jan 31 doi: 10.7150/ijms.80358. PMID: 36860670Free PMC Article
Incidence and Progression of Fibrotic Lung Disease in an At-Risk Cohort.
Steele MP, Peljto AL, Mathai SK, Humphries S, Bang TJ, Oh A, Teague S, Cicchetti G, Sigakis C, Kropski JA, Loyd JE, Blackwell TS, Brown KK, Schwarz MI, Warren RA, Powers J, Walts AD, Markin C, Fingerlin TE, Yang IV, Lynch DA, Lee JS, Schwartz DA
Am J Respir Crit Care Med 2023 Mar 1;207(5):587-593. doi: 10.1164/rccm.202206-1075OC. PMID: 36094461Free PMC Article
Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF).
Krauss E, Gehrken G, Drakopanagiotakis F, Tello S, Dartsch RC, Maurer O, Windhorst A, von der Beck D, Griese M, Seeger W, Guenther A
BMC Pulm Med 2019 Jul 18;19(1):130. doi: 10.1186/s12890-019-0895-6. PMID: 31319833Free PMC Article
Features and outcome of familial idiopathic pulmonary fibrosis.
Ravaglia C, Tomassetti S, Gurioli C, Piciucchi S, Dubini A, Gurioli C, Casoni GL, Romagnoli M, Carloni A, Tantalocco P, Buccioli M, Chilosi M, Poletti V
Sarcoidosis Vasc Diffuse Lung Dis 2014 Apr 18;31(1):28-36. PMID: 24751451
The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
Seibold MA, Smith RW, Urbanek C, Groshong SD, Cosgrove GP, Brown KK, Schwarz MI, Schwartz DA, Reynolds SD
PLoS One 2013;8(3):e58658. Epub 2013 Mar 20 doi: 10.1371/journal.pone.0058658. PMID: 23527003Free PMC Article

Diagnosis

Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF).
Krauss E, Gehrken G, Drakopanagiotakis F, Tello S, Dartsch RC, Maurer O, Windhorst A, von der Beck D, Griese M, Seeger W, Guenther A
BMC Pulm Med 2019 Jul 18;19(1):130. doi: 10.1186/s12890-019-0895-6. PMID: 31319833Free PMC Article
Features and outcome of familial idiopathic pulmonary fibrosis.
Ravaglia C, Tomassetti S, Gurioli C, Piciucchi S, Dubini A, Gurioli C, Casoni GL, Romagnoli M, Carloni A, Tantalocco P, Buccioli M, Chilosi M, Poletti V
Sarcoidosis Vasc Diffuse Lung Dis 2014 Apr 18;31(1):28-36. PMID: 24751451
Familial idiopathic pulmonary fibrosis.
Wytrychowski K, Hans-Wytrychowska A, Nowakowska B
Adv Exp Med Biol 2013;788:363-7. doi: 10.1007/978-94-007-6627-3_49. PMID: 23835999
Idiopathic pulmonary fibrosis: is it a familial disease?
Allam JS, Limper AH
Curr Opin Pulm Med 2006 Sep;12(5):312-7. doi: 10.1097/01.mcp.0000239546.24831.61. PMID: 16926644
Inherited interstitial lung disease.
Garcia CK, Raghu G
Clin Chest Med 2004 Sep;25(3):421-33, v. doi: 10.1016/j.ccm.2004.05.001. PMID: 15331184

Therapy

Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF).
Krauss E, Gehrken G, Drakopanagiotakis F, Tello S, Dartsch RC, Maurer O, Windhorst A, von der Beck D, Griese M, Seeger W, Guenther A
BMC Pulm Med 2019 Jul 18;19(1):130. doi: 10.1186/s12890-019-0895-6. PMID: 31319833Free PMC Article
Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels.
Koga Y, Hachisu Y, Tsurumaki H, Yatomi M, Kaira K, Ohta S, Ono J, Izuhara K, Dobashi K, Hisada T
Medicina (Kaunas) 2019 May 17;55(5) doi: 10.3390/medicina55050161. PMID: 31108995Free PMC Article
Familial idiopathic pulmonary fibrosis: serial high-resolution computed tomography findings in 9 patients.
Nishiyama O, Taniguchi H, Kondoh Y, Kimura T, Katoh T, Oishi T, Matsumoto S, Yokoi T, Takagi K, Shimokata K, Johkoh T, Müller NL
J Comput Assist Tomogr 2004 Jul-Aug;28(4):443-8. doi: 10.1097/00004728-200407000-00002. PMID: 15232373
Nationwide prevalence of sporadic and familial idiopathic pulmonary fibrosis: evidence of founder effect among multiplex families in Finland.
Hodgson U, Laitinen T, Tukiainen P
Thorax 2002 Apr;57(4):338-42. doi: 10.1136/thorax.57.4.338. PMID: 11923553Free PMC Article

Prognosis

Genomic Fingerprint Associated with Familial Idiopathic Pulmonary Fibrosis: A Review.
Ding D, Gao R, Xue Q, Luan R, Yang J
Int J Med Sci 2023;20(3):329-345. Epub 2023 Jan 31 doi: 10.7150/ijms.80358. PMID: 36860670Free PMC Article
Incidence and Progression of Fibrotic Lung Disease in an At-Risk Cohort.
Steele MP, Peljto AL, Mathai SK, Humphries S, Bang TJ, Oh A, Teague S, Cicchetti G, Sigakis C, Kropski JA, Loyd JE, Blackwell TS, Brown KK, Schwarz MI, Warren RA, Powers J, Walts AD, Markin C, Fingerlin TE, Yang IV, Lynch DA, Lee JS, Schwartz DA
Am J Respir Crit Care Med 2023 Mar 1;207(5):587-593. doi: 10.1164/rccm.202206-1075OC. PMID: 36094461Free PMC Article
Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF).
Krauss E, Gehrken G, Drakopanagiotakis F, Tello S, Dartsch RC, Maurer O, Windhorst A, von der Beck D, Griese M, Seeger W, Guenther A
BMC Pulm Med 2019 Jul 18;19(1):130. doi: 10.1186/s12890-019-0895-6. PMID: 31319833Free PMC Article
Familial idiopathic pulmonary fibrosis: clinical features and outcome.
Lee HL, Ryu JH, Wittmer MH, Hartman TE, Lymp JF, Tazelaar HD, Limper AH
Chest 2005 Jun;127(6):2034-41. doi: 10.1378/chest.127.6.2034. PMID: 15947317
Genetic predisposition of idiopathic pulmonary fibrosis.
Mageto YN, Raghu G
Curr Opin Pulm Med 1997 Sep;3(5):336-40. doi: 10.1097/00063198-199709000-00004. PMID: 9331534

Clinical prediction guides

Incidence and Progression of Fibrotic Lung Disease in an At-Risk Cohort.
Steele MP, Peljto AL, Mathai SK, Humphries S, Bang TJ, Oh A, Teague S, Cicchetti G, Sigakis C, Kropski JA, Loyd JE, Blackwell TS, Brown KK, Schwarz MI, Warren RA, Powers J, Walts AD, Markin C, Fingerlin TE, Yang IV, Lynch DA, Lee JS, Schwartz DA
Am J Respir Crit Care Med 2023 Mar 1;207(5):587-593. doi: 10.1164/rccm.202206-1075OC. PMID: 36094461Free PMC Article
Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF).
Krauss E, Gehrken G, Drakopanagiotakis F, Tello S, Dartsch RC, Maurer O, Windhorst A, von der Beck D, Griese M, Seeger W, Guenther A
BMC Pulm Med 2019 Jul 18;19(1):130. doi: 10.1186/s12890-019-0895-6. PMID: 31319833Free PMC Article
Features and outcome of familial idiopathic pulmonary fibrosis.
Ravaglia C, Tomassetti S, Gurioli C, Piciucchi S, Dubini A, Gurioli C, Casoni GL, Romagnoli M, Carloni A, Tantalocco P, Buccioli M, Chilosi M, Poletti V
Sarcoidosis Vasc Diffuse Lung Dis 2014 Apr 18;31(1):28-36. PMID: 24751451
Familial idiopathic pulmonary fibrosis: clinical features and outcome.
Lee HL, Ryu JH, Wittmer MH, Hartman TE, Lymp JF, Tazelaar HD, Limper AH
Chest 2005 Jun;127(6):2034-41. doi: 10.1378/chest.127.6.2034. PMID: 15947317
Genetic predisposition of idiopathic pulmonary fibrosis.
Mageto YN, Raghu G
Curr Opin Pulm Med 1997 Sep;3(5):336-40. doi: 10.1097/00063198-199709000-00004. PMID: 9331534

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