Idiopathic fibrotic lung disease at a university hospital setting: management and prognostic factors

Article English OPEN
Janson, Christer ; Rad, Parya ; Karlsson, Carl-Axel (2015)
  • Publisher: Co-Action Publishing
  • Journal: European Clinical Respiratory Journal, volume 2 (eissn: 2001-8525)
  • Related identifiers: doi: 10.3402/ecrj.v2.26915, doi: 10.3402/ecrj.v2i0.26915, pmc: PMC4629758
  • Subject: Original Article | pulmonary fibrosis, mortality, diagnostics | diagnostics | fibrotic lung disease | survival | idiopathic pulmonary fibrosis | prognosis

Background: Idiopathic fibrosing interstitial pneumonia consists of many subtypes, most associated with a poor prognosis. The aim of the study was to evaluate diagnostic procedures and treatment as well as survival in patients with idiopathic fibrosing interstitial pneumonia.Methods: This study comprised 175 patients with idiopathic fibrosing interstitial pneumonia (ICD 10 code J84) that had been diagnosed at Uppsala University Hospital, during 2005 to 2012. Patient records were reviewed concerning: gender, age, smoking, occupational exposure, comorbidities, procedures, lung function, and treatment. Information on survival and cause of death was collected.Results: A total of 98% had been examined with computed tomography, 93% with spirometry, 49% with measurement of diffusion capacity, 48% with bronchoalveolar lavage, and 23% with lung biopsy. Prednisolone had been prescribed to 74% while N-acetylcysteine (NAC) and omeprazole were prescribed to 54%, respectively. Five-year survival was 46%. Mortality was associated with high age, low diffusion capacity, and the use of NAC.Conclusion: High age and a low diffusion capacity are related to shorter survival in idiopathic fibrosing interstitial pneumonia. We also unexpectedly found that the use of NAC was related to shorter survival. A relatively low proportion of the patients were examined with diffusion capacity measurement. Thus, there is a possibility to improve diagnostic procedures and thereby improve estimation of prognosis in fibrotic lung disease.Keywords: fibrotic lung disease; idiopathic pulmonary fibrosis; diagnostics; prognosis; survival(Published: 24 March 2015)Citation: European Clinical Respiratory Journal 2015, 2: 26915 - http://dx.doi.org/10.3402/ecrj.v2.26915Responsible Editor: Riitta Kaarteenaho, Oulu, Finland.
  • References (20)
    20 references, page 1 of 2

    1. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, 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.

    2. Travis WD, Costabel U, Hansell DM, King TE Jr., Lynch DA, Nicholson AG, et al. 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: 733 48.

    3. Bradley B, Branley HM, Egan JJ, Greaves MS, Hansell DM, Harrison NK, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax. 2008; 63(Suppl 5): v1 58.

    4. 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: 904 10.

    5. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med. 2000; 161: 646 64.

    6. Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011; 377: 1760 9.

    7. King TE Jr., Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014; 370: 2083 92.

    8. Idiopathic Pulmonary Fibrosis Clinical Research N, Raghu G, Anstrom KJ, King TE Jr., Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012; 366: 1968 77.

    9. Demedts M, Behr J, Buhl R, Costabel U, Dekhuijzen R, Jansen HM, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2005; 353: 2229 42.

    10. Idiopathic Pulmonary Fibrosis Clinical Research N, Martinez FJ, de Andrade JA, Anstrom KJ, King TE Jr., Raghu G. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2014; 370: 2093 101.

  • Metrics
    No metrics available
Share - Bookmark