Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis;Normativa sobre el diagnóstico y tratamiento de la fibrosis pulmonar idiopática

Archivos de Bronconeumologia

Volumn 49, Issue 8, 2013, Pages 343-353

  Xaubet, Antoni ^a,b   Ancochea, Julio ^c   Bollo, Elena ^d   Fernández Fabrellas, Estrella ^e   Franquet, Tomás ^f   Molina Molina, Maria ^b,g   Montero, Maria Angeles ^h   Serrano Mollar, Anna ^b,i  

^a HOSPITAL CLÍNIC   (Spain)

^b INSTITUTO DE SALUD CARLOS III   (Spain)

^c HOSPITAL UNIVERSITARIO DE LA PRINCESA   (Spain)

^d Complejo Asistencial Universitario de León   (Spain)

^e HOSPITAL GENERAL UNIVERSITARIO DE VALENCIA   (Spain)

^f HOSPITAL DE LA SANTA CREU I SANT PAU   (Spain)

^g HOSPITAL UNIVERSITARI DE BELLVITGE   (Spain)

^h ROYAL BROMPTON HOSPITAL   (United Kingdom)

ⁱ INST D INVESTIGACIONS B   (Spain)

Author keywords

Diagnosis; Idiopathic pulmonary fibrosis; Treatment

Indexed keywords

ARTICLE; CONSENSUS DEVELOPMENT; FIBROSING ALVEOLITIS; INTERNATIONAL COOPERATION; LIFE EXPECTANCY; PATIENT ASSESSMENT; PRACTICE GUIDELINE; PREVALENCE; PROGNOSIS; TREATMENT PLANNING; ADENOVIRUS INFECTION; ANTIFIBROTIC ACTIVITY; ANTIINFLAMMATORY ACTIVITY; AUTOIMMUNITY; CELL THERAPY; CELLS; COMBINATION CHEMOTHERAPY; COMORBIDITY; CRACKLE; DIFFERENTIAL DIAGNOSIS; DISEASE COURSE; DISEASE EXACERBATION; DRUG CONTRAINDICATION; DRUG EFFICACY; DRUG INDICATION; DYSPNEA; ENVIRONMENTAL FACTOR; EPITHELIAL MESENCHYMAL TRANSITION; FIBROCYTE; FOLLOW UP; FORCED VITAL CAPACITY; GASTROESOPHAGEAL REFLUX; GENE; GENE THERAPY; GENETIC PREDISPOSITION; HEPATITIS C; HEREDITY; HERPES SIMPLEX; HIGH RESOLUTION COMPUTER TOMOGRAPHY; HIGH RISK PATIENT; HISTOPATHOLOGY; HOME OXYGEN THERAPY; HUMAN; INCIDENCE; LUNG CARCINOMA; LUNG EMPHYSEMA; LUNG LAVAGE; LUNG TRANSPLANTATION; MONOTHERAPY; MORTALITY; MUC5B GENE; MULTIDETECTOR COMPUTED TOMOGRAPHY; PALLIATIVE THERAPY; PATHOPHYSIOLOGY; PULMONARY HYPERTENSION; PULMONARY REHABILITATION; RISK FACTOR; SEX DIFFERENCE; SLEEP DISORDERED BREATHING; SYMPTOMATOLOGY; TERC GENE; TERT GENE; TRANSBRONCHIAL BIOPSY; TREATMENT INDICATION;

ACETYLCYSTEINE; ANTIFIBROTIC AGENT; AZATHIOPRINE; BIOLOGICAL MARKER; CYCLOPHOSPHAMIDE; IMATINIB; INTERSTITIAL COLLAGENASE; KL 6 PROTEIN; MATRILYSIN; NINTEDANIB; PIRFENIDONE; PLACEBO; SP A PROTEIN; SP D PROTEIN; UNCLASSIFIED DRUG;

EID: 84881029904     PISSN: 03002896     EISSN: 15792129     Source Type: Journal    
DOI: 10.1016/j.arbr.2013.06.003     Document Type: Article

References (80)

1. Idiopathic pulmonary fibrosis: Diagnosis and treatment; joint statement of the American Thoracic Society and the European Respiratory Society. Am J Respir Crit Care Med 2000, 161:646-664. American Thoracic Society.
2. Raghu G., Collard H.R., Egan J.J., Martinez F.J., Behr J., Brown K.K., et al. An official ATS/ERS/JRS/ALAT statement: Idiopathic pulmonary fibrosis: Evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011, 183:788-824.
3. Xaubet A., Ancochea J., Blanquer R., Montero C., Morell F., Rodríguez Becerra E., et al. Diagnóstico y tratamiento de las enfermedades pulmonares intersticiales difusas. Arch Bronconeumol 2003, 39:580-600.
4. Schünemann H.J., Jaeschke R., Cook D.J., Bria W.F., El-Solh A.A., Ernst A., et al. An official ATS statement: Grading the quality of evidence and strength of recommendations in ATS guidelines and recommendations. Am J Respir Crit Care Med 2006, 174:605-614.
5. Raghu G., Weycker D., Edelsberg J., Bradford W.Z., Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006, 174:810-816.
6. Navaratnam V., Fleming K.M., West J., Smith C.J., Jenkins R.G., Fogarty A., et al. The rising incidence of idiopathic pulmonary fibrosis in the UK. Thorax 2011, 66:462-467.
7. Selman M., Carrillo G., Estrada A., Mejia M., Becerril C., Cisneros J., et al. Accelerated variant of idiopathic pulmonary fibrosis. PLos ONE 2007, 2:e482.
8. Mcneal K., Schwartz D.A. The genetics and environmental causes of pulmonary fibrosis. Proc Am Thorac Soc 2012, 9:120-125.
9. Seibod M.A., Wise A.L., Speer M.C., Steele M.P., Brown K.K., Loyd J.E., et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011, 364:1503-1512.
10. Pinheiro G.A., Antao V.C., Wood J.M., Wassell J.T. Occupational risks for idiopathic pulmonary fibrosis mortality in the United States. Int J Occup Environ Health 2008, 14:117-123.
11. Taskar V.S., Coultas D.B. Is idiopathic pulmonary fibrosis an environmental disease?. Proc Am Thorac Soc 2006, 3:293-298.
12. Lee J.S., Ryu J.H., Elicker B.M., Lydell C.P., Jones K.D., Wolters P.J., et al. Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011, 184:1390-1394.
13. Flaherty K.R., Andrei A.C., King T.E., Raghu G., Colby T.V., Wells A., et al. Idiopathic interstitial pneumonia: Do community and academic physicians agree on diagnosis?. Am J Respir Crit Care Med 2007, 175:1054-1060.
14. Kim D.S., Collard H.R., King T.E. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006, 3:285-292.
15. Rosas I.O., Richards T.J., Konishi K., Zhang Y., Gibson K., Lokshin A.E., et al. MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis. PLoS Med 2008, 5:e93. 0623-0633.
16. Ohnishi H., Yokoyama A., Kondo K., Zhang Y., Gibson K., Lokshin S.F., et al. Comparative study of KL-6, surfactant protein-A, surfactantprotein-D, and monocyte chemoattractant protein-1 as serum markers for interstitial lung diseases. Am J Resp Crit Care Med 2002, 165:378-381.
17. Moeller A., Gilpin S.E., Ask K., Cox G., Cook D., Gauldie J., et al. Circulating fibrocytes are an indicator of poor prognosis in idiopathic pulmonary fibrosis. Am J Resp Crit Care Med 2009, 179:588-594.
18. Van den Blink B., Wijsenbeek M.S., Hoogsteden H.C. Serum biomarkers in idiopathic pulmonary fibrosis. Pulm Pharmacol Ther 2010, 23:515-520.
19. Leslie K.O., Gruden J.F., Parish J.M., Scholand M.B. Transbronchial biopsy interpretation in the patient with diffuse parenchymal lung disease. Arch Pathol Lab Med 2007, 131:407-423.
20. Babiak A., Hetzel J., Krishna G., Fritz P., Moeller P., Balli T., et al. Transbronchial cryobiopsy: A new tool for lung biopsies. Respiration 2009, 78:203-208.
21. Mayo J.R., Aldrich J., Muller N.L. Radiation exposure at chest CT: A statement of the Fleischner Society. Radiology 2003, 228:15-21.
22. Hansell D.M., Bankier A.A., MacMahon H., McLoud T.C., Muller N.L., Remy J. Fleischner Society: Glossary of terms for thoracic imaging. Radiology 2008, 246:697-722.
23. Churg A., Müller N.L., Flint J., Wright J.L. Chronic hypersensitivity pneumonitis. Am J Surg Pathol 2006, 30:201-208.
24. Mahler D.A., Weinberg D.H., Wells C.K., Feinstein A.R. The measurement of dyspnea. Contents, interobserver agreement, and physiologic correlates of two new clinical index. Chest 1984, 85:751-758.
25. Nishiyama O., Taniguchi H., Konodoh Y., Kimura T., Kato K., Kataoka K., et al. A simple assessment of dyspnoea as a prognostic indicator in idiopathic pulmonary fibrosis. Eur Respir J 2010, 36:1067-1072.
26. Birring S.S., Pruden B., Carr A.J., Singh S.J., Morgan M.D.L., Pavord I.D. Development of a symptom specific health status measure for patients with chronic cough: Leicester Cough Questionnaire (LCQ). Thorax 2003, 58:339-343.
27. Yorke J., Jones P.W., Swigris J.J. Development and validity testing of an IPF-specific version of the St George's respiratory questionnaire. Thorax 2010, 65:921-926.
28. Ley B., Collard H.R., King T.E. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011, 183:431-440.
29. Mura M., Porretta M.A., Bargagli E., Sergiacomi G., Zompatori M., Sverzellati N., et al. Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: A 3-year prospective study. Eur Respir J 2012, 40:101-109.
30. Nathan S.D., Shlobin O.A., Weir N. Long-term curse and prognosis of idiopathic pulmonary fibrosis in the new millennium. Chest 2011, 140:21-229.
31. Du Bois R.M., Weycker D., Albera C., Bradford W.Z., Costabel U., Kartashov A., et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: Test properties and minimal clinically important difference. Am J Respir Crit Care Med 2011, 184:1382-1389.
32. Schmidt S.L., Nambiar A.M., Tayab N., Sundaram B., Han M.K., Gross B.H., et al. Pulmonary function measures predict mortality differently in IPF versus combined pulmonary fibrosis and emphysema. Eur Respir J 2011, 38:176-183.
33. Du Bois R.M., Weycker D., Albera C., Bradford W.Z., Costabel U., Kastashou A., et al. Six-minute-walk test in idiopathic pulmonary fibrosis: Test validation and minimal clinically important difference. Am J Respir Crit Care Med 2011, 183:1231-1237.
34. Flaherty K.R., Andrei A.C., Murray S., Fraley C., Colby T.V., Travis W.D., et al. Idiopathic pulmonary fibrosis: Prognostic value of changes in physiology and six-minute-walk test. Am J Respir Crit Care Med 2006, 17:803-809.
35. Fell C.D., Liu L.X., Motika C., Kazerooni E.A., Gross B.H., Travis W.D., et al. The prognostic value of cardiopulmonary exercise testing in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009, 179:402-407.
36. Sumikawa H., Johkoh T., Colby T.V., Ichikado K., Suga M., Taniguchi H., et al. Computed tomography findings in pathological usual interstitial pneumonia: Relationship to survival. Am J Respir Crit Care Med 2008, 177:433-439.
37. Richards T.J., Kaminski N., Baribaud F., Flavin S., Brodmerkel C., Horowitz D., et al. Peripheral blood proteins predict mortality in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2012, 185:67-76.
38. Leuchte H.H., Baumgartner R.A., Nounou M.E., Vogeser M., Neurohr C., Trautnitz M., et al. Brain natriuretic peptide is a prognostic parameter in chronic lung disease. Am J Respir Crit Care Med 2006, 173:744-750.
39. Du Bois R.M., Weycker D., Albera C., Bradford W.Z., Costabel U., Kartashov A., et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011, 184:459-466.
40. Ley B., Ryerson C.J., Vittinghoff E., Ryu J.H., Tomasetti S., Lee J.S., et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med 2012, 156:684-691.
41. Kim D.S. Acute exacerbation of idiopathic pulmonary fibrosis. Clin Chest Med 2012, 33:59-68.
42. Judge E.P., Fabre A., Adamali H.I., Egan J.J. Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Eur Respir J 2012, 40:93-100.
43. Nathan S.D., Cottin V. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Eur Respir Monogr 2012, 57:148-160.
44. Lettieri C.J., Nathan S.D., Barnett S.D., Ahmad S., Shorr A. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006, 3:746-752.
45. Corte T.J., Wort S.J., Gatzoulis M.A., Macdonald P., Hansell D.M., Wells A.U. Pulmonary vascular resistance predicts early mortality in patients with diffuse fibrotic lung disease and suspected pulmonary hypertension. Thorax 2009, 64:883-888.
46. Jankowich M.D., Rounds S.I.S. Combined pulmonary fibrosis and emphysema syndrome: A review. Chest 2012, 141:222-231.
47. Raghu G., Meyer K.C. Silent gastro-esophageal reflux and microaspiration in IPF: Mounting evidence for anti-reflux therapy. Eur Respir J 2012, 39:242-245.
48. Lancaster L.H., Mason W.R., Parnell J.A., Rice T.W., Loyd J.E., Milstone A.P., et al. Obstructive sleep apnea is common in idiopathic pulmonary fibrosis. Chest 2009, 136:772-778.
49. Ozawa Y., Suda T., Naito T., Enomoto N., Hashimoto D., Fujisawa T., et al. Cumulative incidence of and predictive factors for lung cancer in IPF. Respirology 2009, 14:723-728.
50. Hubbard R.B., Smith C., le Jeune I., Gribbin J., Fogarty A.W. The association between idiopathic pulmonary fibrosis and vascular disease: A population-based study. Am J Respir Crit Care Med 2008, 178:1257-1261.
51. Lee J.S., McLaughlin S., Collard H.R. Comprehensive care of the patient with idiopathic pulmonary fibrosis. Curr Opin Pulm Med 2011, 17:348-354.
52. Richeldi L., Davies H.R., Ferrara G., Franco I. Corticosteroids for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev 2003, 3:CD002880.
53. Davies H.R., Richeldi L., Waltwers E.H. Inmunomodulatory agents for idiopathic pulmonary fibrosis. Cochrane Daatabase Syst Rev 2003, CD003134.
54. Spagnolo P., del Giovane C., Luppi F., Cerri S., Balduzzi S., Walter E.R., et al. Non-steroid agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev 2010, 9:CD003134.
55. Demedts M., Behr J., Buhl R., Costabel U., Dekhuijzen R., Jansen H.M., et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005, 353:2229-2242.
56. Raghu G., Anstrom K.J., King T.E., Lasky J.A., Martinez F.J. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012, 366:1968-1977. Idiopathic Pulmonary Fibrosis Clinical Research Network.
57. Cottin V. Changing the idiopathic pulmonary fibrosis treatment approach and improving patient outcomes. Eur Respir Rev 2012, 21:161-167.
58. Noble P.W., Albera C., Bradford W.Z., Costabel U., Glassberg M.K., Kardatzke D., et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): Two randomised trials. Lancet 2011, 377:1760-1769.
59. Taniguchi H., Ebina M., Kondoh Y., Azuma A., Ogura T., Taguchi Y., et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2010, 35:1079-1087.
60. Jiang C., Huang H., Liu J., Wang Y., Lu Z., Xu Z. Adverse events of pirfenidone for the treatment of pulmonary fibrosis: A meta-analysis of randomized controlled trials. PLoS One 2012, 7:e47024.
61. Richeldi L., Costabel U., Selman M., Kim D.S., Hansell D.M., Nicholson A.G., et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med 2011, 365:1079-1087.
62. Luppi F., Spagnolo P., Cerri S., Richeldi L. The big clinical trials in idiopathic pulmonary fibrosis. Curr Opin Pulm 2012, 18:428-432.
63. Collard H.R., Moore B., Flaherty K., Brown K., Kaner R.J., King T.E., et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007, 176:636-647.
64. Mollica C., Paone G., Conti V., Ceccarelli D., Schmid G., Mattia P., et al. Mechanical ventilation in patients with end-stage idiopathic pulmonary fibrosis. Respiration 2010, 79:209-215.
65. Molina-Molina M., Badia J.R., Marín-Arguedas A., Xaubet A., Santos M.J., Nicolás J.M., et al. Resultados y características clínicas de los pacientes con fibrosis pulmonar e insuficiencia respiratoria ingresados en una Unidad de Cuidados Intensivos. Estudio de 20 casos. Med Clin (Barc) 2003, 121:63-67.
66. Collard H.R., Anstrom J.K., Schwarz M.I., Zisman D.A. Sildefanil improves walk distance in idiopathic pulmonary fibrosis. Chest 2007, 131:897-899.
67. Pitsiou G., Papakosta D., Bouros D. Pulmonary hypertension in idiopathic pulmonary fibrosis: A review. Respiration 2011, 82:294-304.
68. Crockett A., Cranston J.M., Antic N. Domiciliary oxygen for interstitial lung disease. Cochrane Database of Systematic Reviews 2001, 3. CD002883. 10.1002/14651858.CD002883.
69. Visca D., Montgomery A., de Lauretis A., Sestini P., Soteriou H., Maher T.M., et al. Ambulatory oxygen in interstitial lung disease. Eur Respir J 2011, 38:987-990.
70. Frank R.C., Hicks S., Duck A.M., Spencer L., Leonard C.T., Barnett E. Ambulatory oxygen in idiopathic pulmonary fibrosis: Of what benefit?. Eur Respir J 2012, 40:269-270.
71. Hook J.L., Arcosoy S.M., Zemmel D., Bartels M.N., Kawut S.M., Lederer D.J. Titrated oxygen requirement and prognostication in idiopathic pulmonary fibrosis. Eur Respir J 2012, 39:359-365.
72. George T.J., Arnaoutakis G.J., Shah A.S. Lung transplant in idiopathic pulmonary fibrosis. Arch Surg 2011, 146:1204-1209.
73. Holland A.E., Hill C. Physical training for interstitial lung disease. Cochrane Database Syst Rev 2008, 4:CD006322.
74. Holland A.E., Hill C.J., Glaspone I., Goh N., McDonald C.F. Predictors of benefit following pulmonary rehabilitation for interstitial lung disease. Respir Med 2012, 106:429-435.
75. Huppmann P., Sczepanski B., Boensch M., Winterkmp S., Schönheit-Kenn U., Neurohr C., et al. Effects of in-patient pulmonary rehabilitation in patients with interstitial lung disease. Eur Respir J 2012, (Epub ahead of print).
76. McNulty K., Janes S.M. Stem cells and pulmonary fibrosis: Cause or cure?. Proc Am Thorac Soc 2012, 9:164-171.
77. Serrano-Mollar A., Nacher M., Gay-Jordi G., Closa D., Xaubet A., Bulbena O. Intratracheal transplantation of alveolar type II cells reverses bleomycin-induced lung fibrosis. Am J Respir Crit Care Med 2007, 176:1261-1268.
78. Allen S., Raut S., Woollard J., Vassallo M. Low dose diamorphine reduces breathlessness without causing a fall in oxygen saturation in elderly patients with end-stage idiopathic pulmonary fibrosis. Palliat Med 2005, 19:128-130.
79. Horton M.R., Santoprieto V., Mathew L., Horton K.M., Polito A.J., Liw M.C., et al. Thalidomide for the treatment of cough in idiopathic pulmonary fibrosis: A randomised trial. Ann Intern Med 2012, 157:398-406.
80. Egan J.J. Follow-up and nonpharmacological management of the idiopathic pulmonary fibrosis patient. Eur Respir Rev 2011, 20:114-117.