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The new ATS/ERS/JRS IPF Guidelines
After the ATS/ERS 2000 joint statement (guidelines) for the idiopathic pulmonary fibrosis (IPF), a committee of international experts on IPF with professor Raghu Ganesh as chair and Jim Egan and Fernando Martinez as co-chairs, have been appointed by the American Thoracic Society (ATS), The European Respiratory Society (ERS) and the Japan Respiratory Society (JRS) to write evidence based guidelines for the management of IPF. Given the scope of the magnitude that the task has evolved, the committee has expanded to include members from most of the regions of the world actively involved in IPF studies. Therefore, 21 pneumonologists, 4 radiologists, and 4 pathologists are participating in this ongoing project (Table 1). The committee met in Dublin last year, this year in Toronto and last month in Modena (photo) to reach a final document.

Evidence based recommendations include diagnosis, treatment and disease monitoring. Questions addressed regarding the diagnosis include:

  1. Can high-resolution CT accurately identify UIP?
  2. Can transbronchial lung biopsy accurately identify UIP?
  3. Is pulmonary function testing required for the diagnosis of IPF?
  4. Are autoimmune serologies required for the diagnosis of IPF?
  5. Is bronchoalveolar lavage required for the diagnosis of IPF?
  6. Is a multi-disciplinary approach important to the accurate diagnosis of IPF?



Questions addressed regarding treatment include:

  1. Should patients with IPF be treated with corticosteroid monotherapy?
  2. Should patients with IPF be treated with combination corticosteroid, azathioprine, and acetylcysteine therapy?
  3. Should patients with IPF be treated with combination corticosteroid and immunomodulator therapy (e.g. azathioprine, cyclphosphamide) or immunomodulator therapy alone?
  4. Should patients with IPF be treated with acetylcysteine monotherapy?
  5. Should patients with IPF be treated with anticoagulants?
  6. Should patients with IPF be treated with interferon gamma 1-b?
  7. Should patients with IPF be treated with pirfenidone?
  8. Should patients with IPF be treated with bosentan or other endothelin antagonists?
  9. Is "no pharmacological therapy" an appropriate treatment option in IPF?



Table 1. Members of the international experts committee.
Chair Ganesh Raghu (USA)
Co-chairs: Fernando Martinez (USA), Jim Egan (Ireland)
Pulmonary Juergen Behr (Germany), Demosthenes Bouros (Greece), Kevin Brown (USA),
Carlos Carvalho(Brazil), Harold Collard (USA), Jean-Francois Cordier (France),
Ulrich Costabel (Germany),Ron Du Bois (USA), Kevin Flaherty (USA),
Dong Soon Kim (South Korea) Talmadge King (USA),Yasuhiro Kondoh (Japan),
Joseph Lasky (USA), Luca Richeldi (Italy), Jay Ryu (USA) Moises Selman (Mexico),
Jeffrey Swigris (USA), Athol Wells (UK)
Radiology David Hansell (UK), Takeshi Johkoh (Japan),David Lynch (USA), Nestor Muller (Canada)
Pathology Tom Colby (USA), Jeffrey Myers (USA), Andrew Nicholson (UK), Masahito Ebina (Japan)
EBM Gordon Rubenfeld (USA)
Task force members at the Modena 4-6 June, 2008 meeting


Pharmacologic Treatment of Pulmonary Hypertension (PH-IPF) topics include:

  1. Should patients with PH-IPF be treated with sildenafil?
  2. Should patients with PH-IPF be treated with prostanoid therapy?
  3. Should patients with PH-IPF be treated with endothelin receptor antagonist therapy?



No pharmacological treatment options included:

  1. Should patients with IPF be treated with oxygen?
  2. Should patients with IPF receive pulmonary rehabilitation?
  3. Should patients with IPF undergo lung transplantation?

Monitoring addressed the following questions:

  1. Should dyspnea and quality of life be measured over time?
  2. Should pulmonary function testing be measured over time?
  3. Should high-resolution CT be repeated over time?
  4. Should exercise testing be repeated over time?
  5. Should arterial blood gas measurements be repeated over time?
  6. Should biomarkers of disease be measured over time?

The new statement is expected to be published mid next year after review and official approval and will include practical evidence-based guidelines on all relevant topics.

© 2011 PNEUMON Magazine, Hellenic Bronchologic Society.
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