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Idiopathic Pulmonary Fibrosis
Julia Alegría, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Idiopathic pulmonary fibrosis (IPF)
              • Synonyms

                • Idiopathic usual interstitial pneumonia (UIP)
                • Cryptogenic fibrosing alveolitis
              • Definitions

                • Fibrosing idiopathic interstitial pneumonia associated with histologic UIP pattern on surgical biopsy
                • ~ 40% of all idiopathic interstitial pneumonias

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. Krishna R et al: Idiopathic Pulmonary Fibrosis 2021
                                        2. Lynch DA et al: Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 6(2):138-53, 2018
                                        3. Raghu G et al: Diagnosis of idiopathic pulmonary fibrosis. an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 198(5):e44-68, 2018
                                        4. Raghu G et al: An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 183(6):788-824, 2011
                                        5. Mueller-Mang C et al: What every radiologist should know about idiopathic interstitial pneumonias. Radiographics. 27(3):595-615, 2007
                                        6. Lynch DA et al: High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med. 172(4):488-93, 2005
                                        7. Lynch DA: Idiopathic interstitial pneumonias: CT features. Radiology. 236(1):10-21, 2005
                                        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

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Idiopathic pulmonary fibrosis (IPF)
                                                    • Synonyms

                                                      • Idiopathic usual interstitial pneumonia (UIP)
                                                      • Cryptogenic fibrosing alveolitis
                                                    • Definitions

                                                      • Fibrosing idiopathic interstitial pneumonia associated with histologic UIP pattern on surgical biopsy
                                                      • ~ 40% of all idiopathic interstitial pneumonias

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Staging, Grading, & Classification

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. Krishna R et al: Idiopathic Pulmonary Fibrosis 2021
                                                                              2. Lynch DA et al: Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 6(2):138-53, 2018
                                                                              3. Raghu G et al: Diagnosis of idiopathic pulmonary fibrosis. an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 198(5):e44-68, 2018
                                                                              4. Raghu G et al: An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 183(6):788-824, 2011
                                                                              5. Mueller-Mang C et al: What every radiologist should know about idiopathic interstitial pneumonias. Radiographics. 27(3):595-615, 2007
                                                                              6. Lynch DA et al: High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med. 172(4):488-93, 2005
                                                                              7. Lynch DA: Idiopathic interstitial pneumonias: CT features. Radiology. 236(1):10-21, 2005