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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
- Previous or concurrent diagnosis of IPF
- 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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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
- Previous or concurrent diagnosis of IPF
- 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
