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Idiopathic Lymphoid Interstitial Pneumonia
Takeshi Johkoh, MD, PhD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Lymphoid interstitial pneumonia (LIP)
              • Synonyms

                • Lymphocytic interstitial pneumonia
              • Definitions

                • Idiopathic LIP: Rare interstitial pneumonia and lymphoproliferative disorder of unknown pathogenesis characterized by extensive alveolar septal infiltration with lymphocytes and plasma cells
                • LIP is only rarely idiopathic and is more often associated with other conditions (e.g., Sjögren syndrome)
                  • Associations
                    • Autoimmune disease (e.g., Sjögren disease)
                    • Infection (e.g., HIV, HTLV-1)
                    • Immunodeficiencies

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Johkoh T et al: Rare idiopathic intestinal pneumonias (IIPs) and histologic patterns in new ATS/ERS multidisciplinary classification of the IIPs. Eur J Radiol. 84(3):542-6, 2015
                                      2. Egashira R et al: CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics. 33(7):1933-49, 2013
                                      3. Travis WD et al: An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 188(6):733-48, 2013
                                      4. Silva CI et al: Diffuse lung cysts in lymphoid interstitial pneumonia: high-resolution CT and pathologic findings. J Thorac Imaging. 21(3):241-4, 2006
                                      5. American Thoracic Society; European Respiratory Society: American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. This joint statement of the American Thoracic Society (ATS) and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 165(2):277-304, 2002
                                      6. Johkoh T et al: Lymphocytic interstitial pneumonia: follow-up CT findings in 14 patients. J Thorac Imaging. 15(3):162-7, 2000
                                      7. Johkoh T et al: Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology. 212(2):567-72, 1999
                                      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

                                                    • Lymphoid interstitial pneumonia (LIP)
                                                  • Synonyms

                                                    • Lymphocytic interstitial pneumonia
                                                  • Definitions

                                                    • Idiopathic LIP: Rare interstitial pneumonia and lymphoproliferative disorder of unknown pathogenesis characterized by extensive alveolar septal infiltration with lymphocytes and plasma cells
                                                    • LIP is only rarely idiopathic and is more often associated with other conditions (e.g., Sjögren syndrome)
                                                      • Associations
                                                        • Autoimmune disease (e.g., Sjögren disease)
                                                        • Infection (e.g., HIV, HTLV-1)
                                                        • Immunodeficiencies

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Staging, Grading, & Classification

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Johkoh T et al: Rare idiopathic intestinal pneumonias (IIPs) and histologic patterns in new ATS/ERS multidisciplinary classification of the IIPs. Eur J Radiol. 84(3):542-6, 2015
                                                                          2. Egashira R et al: CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics. 33(7):1933-49, 2013
                                                                          3. Travis WD et al: An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 188(6):733-48, 2013
                                                                          4. Silva CI et al: Diffuse lung cysts in lymphoid interstitial pneumonia: high-resolution CT and pathologic findings. J Thorac Imaging. 21(3):241-4, 2006
                                                                          5. American Thoracic Society; European Respiratory Society: American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. This joint statement of the American Thoracic Society (ATS) and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 165(2):277-304, 2002
                                                                          6. Johkoh T et al: Lymphocytic interstitial pneumonia: follow-up CT findings in 14 patients. J Thorac Imaging. 15(3):162-7, 2000
                                                                          7. Johkoh T et al: Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology. 212(2):567-72, 1999