January - March 2017: 
Volume 30, Issue 1

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The new ATS-ERS-JRS-ALAT Guidelines for diagnosis of Idiopathic Pulmonary Fibrosis (IPF)
Authors Information
First Department of Pneumonology, Interstitial Lung Disease Unit, Hospital for Diseases of the Chest ‘Sotiria’, Athens;  Medical School, National and Kapodistrian University of Athens, Greece
Full text

Idiopathic Pulmonary Fibrosis (IPF) is the most frequent idiopathic interstitial  “pneumonia”1-3  and one of the almost 500 interstitial lung diseases.  IPF is a specific form of chronic, progressive fibrosing interstitial pneumonia  of unknown cause occurring in adults. Radiologic and/or histopathologic  patterns are consistent with usual interstitial pneumonia (UIP)4-6

The diagnosis of IPF is a difficult and dynamic one. According to existing  guidelines1  it is based on the exclusion of known causes of interstitial lung  disease and the presence of a UIP pattern on high resolution computed  tomography (HRCT) or the presence of a definite or possible UIP pattern on  HRCT with a surgical lung biopsy showing definite or possible UIP pattern.  In this way a number of combinations arise (Table 1) making the diagnosis  questionable in certain cases, making necessary the reference of the case to  a specialized center where a multidisciplinary discussion (MDD) will decide  if the data are enough to diagnose IPF or the case remain unclassifiable5-7 .  Under these circumstances the diagnosis in many cases can remain unclassifiable  and clarification of diagnostic interventions as defined in the 2011  guidelines is the subject of the ongoing development of new diagnostic  guidelines under the auspices of the American Thoracic Society, the European  Respiratory Society, the Japan Respiratory society and  the Latin American Thoracic Society (ATS/ERS/JRS/ALAT). 

TABLE 1. Table showing the various diagnostic combinations according to ra-diologic (HRCT) and histologic pattern. A number of combinations need further evaluation for their diagnostic accuracy.

The chair of the committee is Ganesh Raghu (USA) and  co-chairs Martine Remy-Jardine (EU), Jeff Myers (USA) and  Luca Richeldi (EU). Pulmonologists are 18, radiologists 5  and pathologists 4 (Table 2). A number of specific questions  are to be addressed utilizing full guideline methodology  including PICO questions, systematic reviews, and the  GRADE approach including but not limited to: 

1. Genetic testing 

2. Specific biomarkers 

3. Volumetric HRCT. 

TABLE 2. Members of the new diagnostic guidelines committee.

Furthermore, interesting questions to be discussed  are the following: 

Should patients with newly detected ILD who are  clinically suspected of having IPF and have a HRCT scan  pattern consistent with probable or possible UIP undergo? 

1. Transbronchial biopsy 

2. Bronchoalveolar lavage 

3. Surgical lung biopsy 

4. Surgical lung biopsy more than one wedge lung biopsy  from different parts of the same lung. 

5. Multidisciplinary decision 

6. Lung cryobiopsy 

7. Lung tissue analyzed by molecular techniques. 

Other significant questions for decisions  are the following: 

1. Should patients with newly detected ILD who are  clinically suspected of having IPF but have the combination  of: a) unclassifiable histopathology and b) a  HRCT pattern of possible UIP or inconsistent with UIP  be diagnosed with IPF? 

2. Should patients with newly detected ILD who are  clinically suspected of having IPF but have honeycomb  cysts in the upper lobe on HRCT without air trapping  be diagnosed with IPF? 

3. Should we abandon the term ‘idiopathic’ as a prefix  for pulmonary fibrosis? 

4. In the absence of any clinical features of connective  tissue disease how useful is serology especially in the  elderly population? 

5. How does the presence or absence of mutations affect  our interaction with the patient and more importantly,  should the recommendation be in favor of testing, how  do we advise relatives about a ‘positive’ result – should  they be tested and if so when and what should they  do in the future? 

These much needed updated guidelines are expected  to elucidate many unresolved aspects of this devastating  disease.  

  1. Raghu G, Collard HR, Egan JJ, et al; ATS/ERS/JRS/ALAT Com-mittee 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; 183:788–824.
  2. Travis WD, Costabel U, Hansell DM, 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;188:733-48.
  3. Bouros D. Idiopathic interstitial pneumonias: Classification revision. Pneumon 2010, 23:359-62.
  4. Tzilas V, Bouros D. Usual interstitial pneumonia pattern in the diagnosis of idiopathic pulmonary fibrosis? Lancet Respir Med 2016;4:770-72.
  5. Tzouvelekis A, Tzilas V, Papiris S, Aidinis V, Bouros D. Diagnostic and prognostic challenges in Idiopathic Pulmonary Fibrosis: A patient’s “Q and A” approach. Pulm Pharmacol Ther 2017;42:21-4.
  6. Tzouvelekis A, Herazo-Maya J, Sakamoto K, Bouros D. Biomarkers in the Evaluation and Management of Idiopathic Pulmonary Fibrosis. Curr Top Med Chem 2016;16:1587-98.
  7. Bouros D, Tzouvelekis A. Idiopathic pulmonary fibrosis: on the move. Lancet Respir Med 2014;2:17-9.