ATS/ERS Criteria for Diagnosis of Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic interstitial pneumonias (IIPs) are part of a broad, heterogeneous group of interstitial lung diseases that encompasses more than 200 acute or chronic diseases with varying degrees of inflammation or fibrosis.

ATS/ERS Criteria for Diagnosis of Idiopathic Pulmonary Fibrosis (IPF) in Absence of Surgical Lung Biopsy

Major Criteria

1. Exclusion of other known causes of ILD such as certain drug toxicities, environmental exposures, and connective tissue diseases

2. Abnormal pulmonary function studies that include evidence of restriction (reduced VC, often with an increased FEV₁/FVC ratio) and impaired gas exchange [increased P(A–a)O₂, decreased PaO₂ with rest or exercise or decreased DLCO]

3. Bibasilar reticular abnormalities with minimal ground glass opacities on HRCT scans

4. Transbronchial lung biopsy or BAL showing no features to support an alternative diagnosis

    

Minor Criteria

1. Age >50 yr

2. Insidious onset of otherwise unexplained dyspnea on exertion

3. Duration of illness >3 mo

4. Bibasilar, inspiratory crackles (dry or “Velcro”-type in quality)

Definition of abbreviations

BAL = bronchoalveolar lavage;

DLCO = diffusing capacity of the lung for CO;

HRCT = high-resolution computerized tomography;

ILD = interstitial lung disease;

P(A–a)O₂ = alveolar–arterial pressure difference for O₂;

VC = vital capacity.

In the immunocompetent adult, the presence of all of the major diagnostic criteria as well as at least three of the four minor criteria increases the likelihood of a correct clinical diagnosis of IPF.

 

References:

1. Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J. 2002 May;19(5):794-6. [Medline]

2. Demedts M, Wells AU, Anto JM, Costabel U, Hubbard R, Cullinan P, Slabbynck H, Rizzato G, Poletti V, Verbeken EK, Thomeer MJ, Kokkarinen J, Dalphin JC, Taylor  AN.Interstitial lung diseases: an epidemiological overview. Eur Respir J Suppl. 2001 Sep;32:2s-16s. [Medline]

3. American Thoracic Society; European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002 Jan 15;165(2):277-304. [Medline]

 

Created: Jun 04, 2006