Idiopathic interstitial pneumonia
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Idiopathic Interstitial Pneumonia Microchapters |
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Differentiating Idiopathic interstitial pneumonia from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Idiopathic interstitial pneumonia On the Web |
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American Roentgen Ray Society Images of Idiopathic interstitial pneumonia |
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Directions to Hospitals Treating Idiopathic interstitial pneumonia |
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Risk calculators and risk factors for Idiopathic interstitial pneumonia |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Zaghw, M.D. [2]
Overview
The idiopathic interstitial pneumonias are a broad range of interstitial lung diseases of unknown etiology characterized by expansion of the interstitial compartment initially by inflammatory cells ending by fibrosis due to fibroblasts proliferation and subsequent collagen deposition. It generally manifests non specifically as breathlessness and/or cough and characterized by bilateral abnormal opacities of various types on conventional chest radiographs or computed tomographic (CT) scans.
Historical Perspective
Averill Liebow published a paper in 1975 about definition and classification of interstitial pneumonias in human pathology. His classification was based on histological morphology in different types of idiopathic interstitial pneumonias. Usual interstitial pneumonia (UIP) and desquamative interstitial pneumonia (DIP) have persisted as important histologic groups. However others of his classification as bronchiolitis obliterans with classical interstitial pneumonia (BIP) and giant cell interstitial pneumonia (GIP) have disappeared from subsequent classification. Liebow emphasized that it was histological patterns rather than distinctive diagnostic entities, that could occur in a variety of clinical contexts. Regardless of the clinical context, however, he maintained that precise histological classification of interstitial pneumonias provides clues to etiology, pathogenesis, natural history, and prognosis. In other words, although individual histological variations do not offer distinguished diagnostic entities, However each significantly limits the differential diagnosis and can predict treatment response and outcome.
Classification
Pathophysiology
Causes
Differentiating Idiopathic interstitial pneumonia from other Diseases
Epidemiology and Demographics
The true incidence is unknown. UIP is the most common (50%-60%) of the idiopathic interstitial pneumonia, then NSIP 14%-36%, then DIP/RB-ILD (10%-17%) and AIP (0%-2%)[1]
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings |CT | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies