Idiopathic interstitial pneumonia classification

{{Idiopathic interstitial pneumonia}} Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Zaghw, M.D. [2]; Chetan Lokhande, M.B.B.S [3]

Overview

An idiopathic interstitial pneumonias (IIPs) classification faced difficulties as the disease behavior is overlapping, which makes it difficult to be classified from other pulmonary diseases of heterogenous clinical course. . However the American Thoracic Society/European Respiratory Society (ATS/ERS) Classification of 2002 defined seven specific entities provided standardized terminology and diagnostic criteria.Later, many publications have led to adopt general changes in the 2007 statement update by ATS/ERS. The new changes emphasized that the histologic diagnosis as a historical gold standard is replaced by a multidisciplinary approach. With the era of molecular markers and multidisciplinary approach, surgical biopsy to diagnose the respiratory bronchiolitis-interstitial lung disease is not needed anymore, nonspecific interstitial pneumonia is now better defined and rare disease as pleuroparenchymal fibroelastosis is introduced. The heterogeneous mixed pattern with acute exacerbation is now defined as a clinical course for idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia.

2013 Revised ATS/ERC Classification of Idiopathic Interstitial Pneumonias: Multidisciplinary Diagnoses

Major idiopathic interstitial pneumonias

• Idiopathic pulmonary fibrosis
• Idiopathic nonspecific interstitial pneumonia
• Respiratory bronchiolitis-interstitial lung disease
• Desquamative interstitial pneumonia
• Cryptogenic organizing pneumonia
• Acute interstitial pneumonia

Rare idiopathic interstitial pneumonias

• Idiopathic lymphoid interstitial pneumonia
• Idiopathic pleuroparenchymal fibroelastosis

Unclassifiable idiopathic interstitial pneumonias

• Inadequate clinical, radiologic, or pathologic data
• Major discordance between clinical, radiologic, and pathologic findings that may occur in the following situations

• Previous therapy resulting in substantial alteration of radiologic or histologic findings (e.g., biopsy of desquamative interstitial pneumonia after steroid therapy, which shows only residual nonspecific interstitial pneumonia.
• New entity, or unusual variant of recognized entity, not adequately characterized by the current American Thoracic Society/European Respiratory Society classification (e.g., variant of organizing pneumonia with supervening fibrosis).
• Multiple high-resolution computed tomography and/or pathologic patterns that may be encountered in patients with idiopathic interstitial pneumonia.

References

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