Skip to main navigation menu Skip to main content Skip to site footer

Original article

Vol. 148 No. 0102 (2018)

Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre

  • Sabina A. Guler
  • Pascal Zumstein
  • Sabina Berezowska
  • Alexander Poellinger
  • Thomas Geiser
  • Manuela Funke-Chambour
DOI
https://doi.org/10.4414/smw.2018.14577
Cite this as:
Swiss Med Wkly. 2018;148:w14577
Published
04.01.2018

Summary

BACKGROUND

Idiopathic pulmonary fibrosis (IPF) differs substantially from other idiopathic interstitial pneumonias regarding disease trajectory and the appropriate management strategies, making meticulous diagnosis essential. However, patient characteristics and clinical practice vary between clinical trials, and real life and registries provide the opportunity to critically analyse current clinical practices in order to ultimately improve patient care.

METHODS: We aimed to identify characteristics of our baseline IPF cohort at initiation of a web-based registry for patients with idiopathic interstitial pneumonia. Baseline and 6-month follow-up data from all consecutive IPF patients consulting at our centre over 2 years were analysed.

RESULTS

Forty IPF patients were included for baseline and 23 for longitudinal analysis. Besides many similarities to other IPF populations, our cohort included considerably fewer women. Forced vital capacity impairment in our cohort was more severe and mortality prediction poorer than in clinical trials, which emphasises the importance to confirm the applicability of clinical trial results with data from real life settings.

CONCLUSION

Registries for rare diseases such as IPF are a valuable resource for studying the course of the disease under current compliance with diagnostic and treatment guidelines and to appreciate local epidemiological particularities.

References

  1. Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar HD, Schroeder DR, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998;157(1):199–203. doi:.https://doi.org/10.1164/ajrccm.157.1.9704130
  2. Ley B, Collard HR, King TE, Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–40. doi:.https://doi.org/10.1164/rccm.201006-0894CI
  3. King TE, Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al.; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92. doi:.https://doi.org/10.1056/NEJMoa1402582
  4. Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al.; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82. doi:.https://doi.org/10.1056/NEJMoa1402584
  5. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al.; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. 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(6):788–824. doi:.https://doi.org/10.1164/rccm.2009-040GL
  6. Travis WD, Costabel U, Hansell DM, King TE, Jr, Lynch DA, Nicholson AG, et al.; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6):733–48. doi:.https://doi.org/10.1164/rccm.201308-1483ST
  7. Behr J, Günther A, Ammenwerth W, Bittmann I, Bonnet R, Buhl R, et al. S2K-Leitlinie zur Diagnostik und Therapie der idiopathischen Lungenfibrose [German guideline for diagnosis and management of idiopathic pulmonary fibrosis]. Pneumologie. 2013;67(2):81–111. Article in German.
  8. Cottin V, Crestani B, Valeyre D, Wallaert B, Cadranel J, Dalphin JC, et al., Societe de Pneumologies de Langue Francaise. 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 [French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis. From the National Reference and the Competence centers for rare diseases and the Société de Pneumologie de Langue Française]. Rev Mal Respir. 2013;30(10):879–902. Article in French. doi:.https://doi.org/10.1016/j.rmr.2013.09.007
  9. Behr J, Kreuter M, Hoeper MM, Wirtz H, Klotsche J, Koschel D, et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015;46(1):186–96. doi:.https://doi.org/10.1183/09031936.00217614
  10. Moodley Y, Goh N, Glaspole I, Macansh S, Walters EH, Chapman S, et al.; Australian IPF Registry Steering Committee. Australian Idiopathic Pulmonary Fibrosis Registry: vital lessons from a national prospective collaborative project. Respirology. 2014;19(7):1088–91. doi:.https://doi.org/10.1111/resp.12358
  11. Ferrara G, Carlson L, Palm A, Einarsson J, Olivesten C, Sköld M ; for the Swedish Idiopathic Pulmonar. Idiopathic pulmonary fibrosis in Sweden: report from the first year of activity of the Swedish IPF-Registry. Eur Clin Respir J. 2016;3(1):31090. doi:.https://doi.org/10.3402/ecrj.v3.31090
  12. Musellim B, Okumus G, Uzaslan E, Akgün M, Cetinkaya E, Turan O, et al.; Turkish Interstitial Lung Diseases Group. Epidemiology and distribution of interstitial lung diseases in Turkey. Clin Respir J. 2014;8(1):55–62. doi:.https://doi.org/10.1111/crj.12035
  13. Singh S, Collins BF, Sharma BB, Joshi JM, Talwar D, Katiyar S, et al. Interstitial Lung Disease in India. Results of a Prospective Registry. Am J Respir Crit Care Med. 2017;195(6):801–13. doi:.https://doi.org/10.1164/rccm.201607-1484OC
  14. Alhamad EH. Interstitial lung diseases in Saudi Arabia: A single-center study. Ann Thorac Med. 2013;8(1):33–7. doi:.https://doi.org/10.4103/1817-1737.105717
  15. Hyldgaard C, Hilberg O, Muller A, Bendstrup E. A cohort study of interstitial lung diseases in central Denmark. Respir Med. 2014;108(5):793–9. doi:.https://doi.org/10.1016/j.rmed.2013.09.002
  16. Harari S, Madotto F, Caminati A, Conti S, Cesana G. Epidemiology of Idiopathic Pulmonary Fibrosis in Northern Italy. PLoS One. 2016;11(2):e0147072. doi:.https://doi.org/10.1371/journal.pone.0147072
  17. Jo HE, Glaspole I, Grainge C, Goh N, Hopkins PM, Moodley Y, et al. Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J. 2017;49(2):1601592. doi:. Correction in: Eur Respir J 2017;49:1651592. doi:.https://doi.org/10.1183/13993003.01592-2016
  18. Coultas DB, Zumwalt RE, Black WC, Sobonya RE. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med. 1994;150(4):967–72. doi:.https://doi.org/10.1164/ajrccm.150.4.7921471
  19. Ley B, Collard HR. Epidemiology of idiopathic pulmonary fibrosis. Clin Epidemiol. 2013;5:483–92. doi:.https://doi.org/10.2147/CLEP.S54815
  20. Baumgartner KB, Samet JM, Coultas DB, Stidley CA, Hunt WC, Colby TV, et al.; Collaborating Centers. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Am J Epidemiol. 2000;152(4):307–15. doi:.https://doi.org/10.1093/aje/152.4.307
  21. Ekström M, Gustafson T, Boman K, Nilsson K, Tornling G, Murgia N, et al. Effects of smoking, gender and occupational exposure on the risk of severe pulmonary fibrosis: a population-based case-control study. BMJ Open. 2014;4(1):e004018. doi:.https://doi.org/10.1136/bmjopen-2013-004018
  22. Keller R, Radtke T, Krebs H, Hornung R. Der Tabakkonsum der Schweizer Wohnbevölkerung in den Jahren 2001 bis 2010. Tabakmonitoring – Schweizerische Umfrage zum Tabakkonsum. Psychologisches Institut der Universität Zürich, Sozial- und Gesundheitspsychologie. 2011.
  23. Tofovic SP, Zhang X, Jackson EK, Zhu H, Petrusevska G. 2-methoxyestradiol attenuates bleomycin-induced pulmonary hypertension and fibrosis in estrogen-deficient rats. Vascul Pharmacol. 2009;51(2-3):190–7. doi:.https://doi.org/10.1016/j.vph.2009.06.002
  24. Mehrad M, Trejo Bittar HE, Yousem SA. Sex steroid receptor expression in idiopathic pulmonary fibrosis. Hum Pathol. 2017;66:200–5. doi:.https://doi.org/10.1016/j.humpath.2017.02.012
  25. Ancochea J, Miravitlles M, García-Río F, Muñoz L, Sánchez G, Sobradillo V, et al. Underdiagnosis of chronic obstructive pulmonary disease in women: quantification of the problem, determinants and proposed actions. Arch Bronconeumol. 2013;49(6):223–9.
  26. Ley B, Bradford WZ, Vittinghoff E, Weycker D, du Bois RM, Collard HR. Predictors of Mortality Poorly Predict Common Measures of Disease Progression in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2016;194(6):711–8. doi:.https://doi.org/10.1164/rccm.201508-1546OC
  27. Richeldi L, Cottin V, du Bois RM, Selman M, Kimura T, Bailes Z, et al. Nintedanib in patients with idiopathic pulmonary fibrosis: Combined evidence from the TOMORROW and INPULSIS(®) trials. Respir Med. 2016;113:74–9. doi:.https://doi.org/10.1016/j.rmed.2016.02.001
  28. Cottin V, Maher T. Long-term clinical and real-world experience with pirfenidone in the treatment of idiopathic pulmonary fibrosis. Eur Respir Rev. 2015;24(135):58–64. doi:.. Corrected in: Eur Respir Rev. 2015;24(137):545. http://err.ersjournals.com/content/24/137/545.1.https://doi.org/10.1183/09059180.00011514
  29. Hughes G, Toellner H, Morris H, Leonard C, Chaudhuri N. Real World Experiences: Pirfenidone and Nintedanib are Effective and Well Tolerated Treatments for Idiopathic Pulmonary Fibrosis. J Clin Med. 2016;5(9):78. doi:.https://doi.org/10.3390/jcm5090078
  30. Kreuter M, Ehlers-Tenenbaum S, Palmowski K, Bruhwyler J, Oltmanns U, Muley T, et al. Impact of Comorbidities on Mortality in Patients with Idiopathic Pulmonary Fibrosis. PLoS One. 2016;11(3):e0151425. doi:.https://doi.org/10.1371/journal.pone.0151425
  31. King TE, Jr, Albera C, Bradford WZ, Costabel U, du Bois RM, Leff JA, et al.; Implications for the Design and Execution of Clinical Trials. All-cause mortality rate in patients with idiopathic pulmonary fibrosis. Implications for the design and execution of clinical trials. Am J Respir Crit Care Med. 2014;189(7):825–31. doi:.https://doi.org/10.1164/rccm.201311-1951OC
  32. Akhtar AA, Ali MA, Smith RP. Depression in patients with idiopathic pulmonary fibrosis. Chron Respir Dis. 2013;10(3):127–33. doi:.https://doi.org/10.1177/1479972313493098
  33. 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(5):723–8. doi:.https://doi.org/10.1111/j.1440-1843.2009.01547.x
  34. Alakhras M, Decker PA, Nadrous HF, Collazo-Clavell M, Ryu JH. Body mass index and mortality in patients with idiopathic pulmonary fibrosis. Chest. 2007;131(5):1448–53. doi:.https://doi.org/10.1378/chest.06-2784
  35. Mermigkis C, Stagaki E, Tryfon S, Schiza S, Amfilochiou A, Polychronopoulos V, et al. How common is sleep-disordered breathing in patients with idiopathic pulmonary fibrosis? Sleep Breath. 2010;14(4):387–90. doi:.https://doi.org/10.1007/s11325-010-0336-5
  36. Raghu G, Freudenberger TD, Yang S, Curtis JR, Spada C, Hayes J, et al. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J. 2006;27(1):136–42. doi:.https://doi.org/10.1183/09031936.06.00037005
  37. Holland AE, Fiore JF, Jr, Bell EC, Goh N, Westall G, Symons K, et al. Dyspnoea and comorbidity contribute to anxiety and depression in interstitial lung disease. Respirology. 2014;19(8):1215–21. doi:.https://doi.org/10.1111/resp.12360
  38. Guler SA, Berezowska SA, Christe A, Geiser T, Funke-Chambour M. Multidisciplinary discussion for diagnosis of interstitial lung disease in real life. Swiss Med Wkly. 2016;146:w14318. Available at: https://smw.ch/en/article/doi/smw.2016.14318/.
  39. Flaherty KR, King TE, Jr, Raghu G, Lynch JP, 3rd, Colby TV, Travis WD, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med. 2004;170(8):904–10. doi:.https://doi.org/10.1164/rccm.200402-147OC
  40. Tomassetti S, Piciucchi S, Tantalocco P, Dubini A, Poletti V. The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review. Eur Respir Rev. 2015;24(135):69–77. doi:.https://doi.org/10.1183/09059180.00011714

Most read articles by the same author(s)