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Airway-Centered Interstitial Fibrosis
Tomás Franquet, MD, PhD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Airway-centered interstitial fibrosis (ACIF)
            • Synonyms

              • Centrilobular fibrosis
              • Idiopathic bronchiolocentric interstitial pneumonia
              • Peribronchiolar metaplasia
            • Definitions

              • Distinctive pattern of fibrosis centered on membranous and respiratory bronchioles
              • Response to variety of environmental exposures or insults
                • Cluster 2 hypersensitivity pneumonitis (HP)
                • Gastroesophageal reflux disease (GERD)
                  • Hiatal hernia
                  • Systemic sclerosis in setting of common variable immune deficiency (CVID)
                • Adverse drug reaction
                • Inhalational injury (e.g., chemical fumes)
                • Chronic/recurrent infection
                • Smoking (< 50%); may contribute to airway injury

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Bois MC et al: Could prominent airway-centered fibroblast foci in lung biopsies predict underlying chronic microaspiration in idiopathic pulmonary fibrosis patients? Hum Pathol. 53:1-7, 2016
                                      2. Kuranishi LT et al: Airway-centered interstitial fibrosis: etiology, clinical findings and prognosis. Respir Res. 16:55, 2015
                                      3. Virk RK et al: Interstitial lung diseases that are difficult to classify: a review of bronchiolocentric interstitial lung disease. Arch Pathol Lab Med. 139(8):984-8, 2015
                                      4. Allaix ME et al: Idiopathic pulmonary fibrosis and gastroesophageal reflux. Implications for treatment. J Gastrointest Surg. 18(1):100-4; discussion 104-5, 2014
                                      5. Herbst JB et al: Hypersensitivity pneumonia: role of surgical lung biopsy. Arch Pathol Lab Med. 136(8):889-95, 2012
                                      6. Myers JL: Hypersensitivity pneumonia: the role of lung biopsy in diagnosis and management. Mod Pathol. 25 Suppl 1:S58-67, 2012
                                      7. Fenton ME et al: Hypersensitivity pneumonitis as a cause of airway-centered interstitial fibrosis. Ann Allergy Asthma Immunol. 99(5):465-6, 2007
                                      8. Churg A et al: Airway-centered interstitial fibrosis: a distinct form of aggressive diffuse lung disease. Am J Surg Pathol. 28(1):62-8, 2004
                                      9. Colombat M et al: Lung transplantation in a patient with airway-centered fibrosis. Am J Surg Pathol. 28(11):1540-2, 2004
                                      10. Yousem SA et al: Idiopathic bronchiolocentric interstitial pneumonia. Mod Pathol. 15(11):1148-53, 2002
                                      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

                                                  • Airway-centered interstitial fibrosis (ACIF)
                                                • Synonyms

                                                  • Centrilobular fibrosis
                                                  • Idiopathic bronchiolocentric interstitial pneumonia
                                                  • Peribronchiolar metaplasia
                                                • Definitions

                                                  • Distinctive pattern of fibrosis centered on membranous and respiratory bronchioles
                                                  • Response to variety of environmental exposures or insults
                                                    • Cluster 2 hypersensitivity pneumonitis (HP)
                                                    • Gastroesophageal reflux disease (GERD)
                                                      • Hiatal hernia
                                                      • Systemic sclerosis in setting of common variable immune deficiency (CVID)
                                                    • Adverse drug reaction
                                                    • Inhalational injury (e.g., chemical fumes)
                                                    • Chronic/recurrent infection
                                                    • Smoking (< 50%); may contribute to airway injury

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Microscopic Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      DIAGNOSTIC CHECKLIST

                                                                      • Consider

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Bois MC et al: Could prominent airway-centered fibroblast foci in lung biopsies predict underlying chronic microaspiration in idiopathic pulmonary fibrosis patients? Hum Pathol. 53:1-7, 2016
                                                                          2. Kuranishi LT et al: Airway-centered interstitial fibrosis: etiology, clinical findings and prognosis. Respir Res. 16:55, 2015
                                                                          3. Virk RK et al: Interstitial lung diseases that are difficult to classify: a review of bronchiolocentric interstitial lung disease. Arch Pathol Lab Med. 139(8):984-8, 2015
                                                                          4. Allaix ME et al: Idiopathic pulmonary fibrosis and gastroesophageal reflux. Implications for treatment. J Gastrointest Surg. 18(1):100-4; discussion 104-5, 2014
                                                                          5. Herbst JB et al: Hypersensitivity pneumonia: role of surgical lung biopsy. Arch Pathol Lab Med. 136(8):889-95, 2012
                                                                          6. Myers JL: Hypersensitivity pneumonia: the role of lung biopsy in diagnosis and management. Mod Pathol. 25 Suppl 1:S58-67, 2012
                                                                          7. Fenton ME et al: Hypersensitivity pneumonitis as a cause of airway-centered interstitial fibrosis. Ann Allergy Asthma Immunol. 99(5):465-6, 2007
                                                                          8. Churg A et al: Airway-centered interstitial fibrosis: a distinct form of aggressive diffuse lung disease. Am J Surg Pathol. 28(1):62-8, 2004
                                                                          9. Colombat M et al: Lung transplantation in a patient with airway-centered fibrosis. Am J Surg Pathol. 28(11):1540-2, 2004
                                                                          10. Yousem SA et al: Idiopathic bronchiolocentric interstitial pneumonia. Mod Pathol. 15(11):1148-53, 2002