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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Acute exacerbation (AE) of interstitial lung disease (ILD)
Synonyms
Accelerated usual interstitial pneumonia (UIP)
Definitions
Acute deterioration of idiopathic pulmonary fibrosis (IPF) may be due to infection, pulmonary embolism, pneumothorax, heart failure or AE
AE of ILD
Acute, clinically significant respiratory deterioration characterized by new widespread alveolar abnormalities
Diagnostic criteria (should meet all 4)
Previous or concurrent diagnosis of IPF
If diagnosis of IPF is not previously established, this criterion can be met by presence of radiologic &/or histopathologic changes consistent with usual interstitial pneumonia pattern on current evaluation
Acute worsening or development of dyspnea typically of < 1 month duration
CT with new bilateral ground-glass opacities &/or consolidation superimposed on background pattern consistent with UIP
If no previous CT is available, qualifier "new" can be dropped
Deterioration not fully explained by heart failure or fluid overload
AE may be idiopathic or triggered by infection, drug toxicity, or aspiration or may be postprocedural or postsurgical
Exclusion of infection or other possible triggers is no longer required for diagnosis of AE
AE can occur in other types of idiopathic interstitial pneumonia (IIP): Nonspecific interstitial pneumonia (NSIP), connective tissue disease-associated interstitial lung disease (CTD-ILD), and cluster 2 hypersensitivity pneumonitis
IMAGING
Radiographic Findings
CT Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Arai T et al: Heterogeneity of incidence and outcome of acute exacerbation in idiopathic interstitial pneumonia. Respirology. 21(8):1431-1437, 2016
Collard HR et al: Acute exacerbation of idiopathic pulmonary fibrosis. An international working group report. Am J Respir Crit Care Med. 194(3):265-75, 2016
de Andrade J et al: The Idiopathic Pulmonary Fibrosis Clinical Research Network (IPFnet): diagnostic and adjudication processes. Chest. 148(4):1034-42, 2015
Fujimoto K et al: Acute exacerbation of idiopathic pulmonary fibrosis: high-resolution CT scores predict mortality. Eur Radiol. 22(1):83-92, 2012
Suda T et al: Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med. 103(6):846-53, 2009
Akira M et al: Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 178(4):372-8, 2008
Olson AL et al: Acute exacerbations of fibrotic hypersensitivity pneumonitis: a case series. Chest. 134(4):844-50, 2008
Park IN et al: Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 132(1):214-20, 2007
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Acute exacerbation (AE) of interstitial lung disease (ILD)
Synonyms
Accelerated usual interstitial pneumonia (UIP)
Definitions
Acute deterioration of idiopathic pulmonary fibrosis (IPF) may be due to infection, pulmonary embolism, pneumothorax, heart failure or AE
AE of ILD
Acute, clinically significant respiratory deterioration characterized by new widespread alveolar abnormalities
Diagnostic criteria (should meet all 4)
Previous or concurrent diagnosis of IPF
If diagnosis of IPF is not previously established, this criterion can be met by presence of radiologic &/or histopathologic changes consistent with usual interstitial pneumonia pattern on current evaluation
Acute worsening or development of dyspnea typically of < 1 month duration
CT with new bilateral ground-glass opacities &/or consolidation superimposed on background pattern consistent with UIP
If no previous CT is available, qualifier "new" can be dropped
Deterioration not fully explained by heart failure or fluid overload
AE may be idiopathic or triggered by infection, drug toxicity, or aspiration or may be postprocedural or postsurgical
Exclusion of infection or other possible triggers is no longer required for diagnosis of AE
AE can occur in other types of idiopathic interstitial pneumonia (IIP): Nonspecific interstitial pneumonia (NSIP), connective tissue disease-associated interstitial lung disease (CTD-ILD), and cluster 2 hypersensitivity pneumonitis
IMAGING
Radiographic Findings
CT Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Arai T et al: Heterogeneity of incidence and outcome of acute exacerbation in idiopathic interstitial pneumonia. Respirology. 21(8):1431-1437, 2016
Collard HR et al: Acute exacerbation of idiopathic pulmonary fibrosis. An international working group report. Am J Respir Crit Care Med. 194(3):265-75, 2016
de Andrade J et al: The Idiopathic Pulmonary Fibrosis Clinical Research Network (IPFnet): diagnostic and adjudication processes. Chest. 148(4):1034-42, 2015
Fujimoto K et al: Acute exacerbation of idiopathic pulmonary fibrosis: high-resolution CT scores predict mortality. Eur Radiol. 22(1):83-92, 2012
Suda T et al: Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med. 103(6):846-53, 2009
Akira M et al: Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 178(4):372-8, 2008
Olson AL et al: Acute exacerbations of fibrotic hypersensitivity pneumonitis: a case series. Chest. 134(4):844-50, 2008
Park IN et al: Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 132(1):214-20, 2007
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