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Acute Exacerbation of Interstitial Lung Disease
Takeshi Johkoh, MD, PhD
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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

                                1. Arai T et al: Heterogeneity of incidence and outcome of acute exacerbation in idiopathic interstitial pneumonia. Respirology. 21(8):1431-1437, 2016
                                2. 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
                                3. de Andrade J et al: The Idiopathic Pulmonary Fibrosis Clinical Research Network (IPFnet): diagnostic and adjudication processes. Chest. 148(4):1034-42, 2015
                                4. Fujimoto K et al: Acute exacerbation of idiopathic pulmonary fibrosis: high-resolution CT scores predict mortality. Eur Radiol. 22(1):83-92, 2012
                                5. Suda T et al: Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med. 103(6):846-53, 2009
                                6. 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
                                7. Olson AL et al: Acute exacerbations of fibrotic hypersensitivity pneumonitis: a case series. Chest. 134(4):844-50, 2008
                                8. 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

                                                              1. Arai T et al: Heterogeneity of incidence and outcome of acute exacerbation in idiopathic interstitial pneumonia. Respirology. 21(8):1431-1437, 2016
                                                              2. 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
                                                              3. de Andrade J et al: The Idiopathic Pulmonary Fibrosis Clinical Research Network (IPFnet): diagnostic and adjudication processes. Chest. 148(4):1034-42, 2015
                                                              4. Fujimoto K et al: Acute exacerbation of idiopathic pulmonary fibrosis: high-resolution CT scores predict mortality. Eur Radiol. 22(1):83-92, 2012
                                                              5. Suda T et al: Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med. 103(6):846-53, 2009
                                                              6. 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
                                                              7. Olson AL et al: Acute exacerbations of fibrotic hypersensitivity pneumonitis: a case series. Chest. 134(4):844-50, 2008
                                                              8. Park IN et al: Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 132(1):214-20, 2007