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Sjögren Syndrome
Sonia L. Betancourt Cuellar, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Sjögren syndrome (SS)
            • Synonyms

              • Sicca syndrome
              • Gougerot-Sjögren disease
            • Definitions

              • Chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of exocrine glands and other extraglandular sites
              • May be primary or associated with other connective tissue diseases, most commonly rheumatoid arthritis
              • Pulmonary involvement occurs in 9-20% of patients, although subclinical lung disease may be more frequent
                • Lung involvement is more common in secondary SS than in primary SS
                • Nonspecific interstitial pneumonia (NSIP) is most common interstitial lung disease (ILD) in patients with SS (45%)
              • Lymphoproliferative disorders associated with SS
                • Reactive (benign) lymphoproliferative disorders
                  • Follicular bronchiolitis
                  • Lymphoid interstitial pneumonia (LIP)
                  • Diffuse lymphoid hyperplasia (DLH)
                • Malignant lymphoproliferative disorders
                  • Diffuse large B-cell lymphoma
                  • Mucosa-associated lymphoid tissue (MALT) lymphoma or MALToma
                    • May progress to diffuse large B-cell non-Hodgkin lymphoma (NHL)

            IMAGING

            • Radiographic Findings

              • CT Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                • Image Interpretation Pearls

                                  • Reporting Tips

                                    Selected References

                                    1. Flament T et al: Pulmonary manifestations of Sjögren's syndrome. Eur Respir Rev. 25(140):110-23, 2016
                                    2. Minato H et al: Thymic lymphoid hyperplasia with multilocular thymic cysts diagnosed before the Sjögren syndrome diagnosis. Diagn Pathol. 10:103, 2015
                                    3. Ohno Y et al: State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 17(12):69, 2015
                                    4. Ruano CA et al: Thoracic manifestations of connective tissue diseases. Curr Probl Diagn Radiol. 44(1):47-59, 2015
                                    5. Kreider M et al: Pulmonary involvement in Sjögren syndrome. Semin Respir Crit Care Med. 35(2):255-64, 2014
                                    6. Egashira R et al: CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics. 33(7):1933-49, 2013
                                    7. Stojan G et al: Pulmonary manifestations of Sjögren's syndrome. Curr Allergy Asthma Rep. 13(4):354-60, 2013
                                    8. Tsai Y et al: Thymoma associated with myasthenia gravis and Sjögren syndrome. West Indian Med J. 62(3):264-5, 2013
                                    9. Borie R et al: Severe chronic bronchiolitis as the presenting feature of primary Sjögren's syndrome. Respir Med. 105(1):130-6, 2011
                                    10. Shi JH et al: Pulmonary manifestations of Sjögren's syndrome. Respiration. 78(4):377-86, 2009
                                    11. Launay D et al: Pulmonary arterial hypertension: a rare complication of primary Sjögren syndrome: report of 9 new cases and review of the literature. Medicine (Baltimore). 86(5):299-315, 2007
                                    12. Tonami H et al: Clinical and imaging findings of lymphoma in patients with Sjögren syndrome. J Comput Assist Tomogr. 27(4):517-24, 2003
                                    13. Choi YW et al: Idiopathic multilocular thymic cyst: CT features with clinical and histopathologic correlation. AJR Am J Roentgenol. 177(4):881-5, 2001
                                    14. Levy Y et al: Malignant thymoma associated with autoimmune diseases: a retrospective study and review of the literature. Semin Arthritis Rheum. 28(2):73-9, 1998
                                    15. Matsumoto Y et al: Sjögren's syndrome associated with thymoma. J Am Acad Dermatol. 35(4):639-40, 1996
                                    16. Suster S et al: Multilocular thymic cyst: an acquired reactive process. Study of 18 cases. Am J Surg Pathol. 15(4):388-98, 1991
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Sjögren syndrome (SS)
                                              • Synonyms

                                                • Sicca syndrome
                                                • Gougerot-Sjögren disease
                                              • Definitions

                                                • Chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of exocrine glands and other extraglandular sites
                                                • May be primary or associated with other connective tissue diseases, most commonly rheumatoid arthritis
                                                • Pulmonary involvement occurs in 9-20% of patients, although subclinical lung disease may be more frequent
                                                  • Lung involvement is more common in secondary SS than in primary SS
                                                  • Nonspecific interstitial pneumonia (NSIP) is most common interstitial lung disease (ILD) in patients with SS (45%)
                                                • Lymphoproliferative disorders associated with SS
                                                  • Reactive (benign) lymphoproliferative disorders
                                                    • Follicular bronchiolitis
                                                    • Lymphoid interstitial pneumonia (LIP)
                                                    • Diffuse lymphoid hyperplasia (DLH)
                                                  • Malignant lymphoproliferative disorders
                                                    • Diffuse large B-cell lymphoma
                                                    • Mucosa-associated lymphoid tissue (MALT) lymphoma or MALToma
                                                      • May progress to diffuse large B-cell non-Hodgkin lymphoma (NHL)

                                              IMAGING

                                              • Radiographic Findings

                                                • CT Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Demographics

                                                            • Natural History & Prognosis

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  • Image Interpretation Pearls

                                                                    • Reporting Tips

                                                                      Selected References

                                                                      1. Flament T et al: Pulmonary manifestations of Sjögren's syndrome. Eur Respir Rev. 25(140):110-23, 2016
                                                                      2. Minato H et al: Thymic lymphoid hyperplasia with multilocular thymic cysts diagnosed before the Sjögren syndrome diagnosis. Diagn Pathol. 10:103, 2015
                                                                      3. Ohno Y et al: State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 17(12):69, 2015
                                                                      4. Ruano CA et al: Thoracic manifestations of connective tissue diseases. Curr Probl Diagn Radiol. 44(1):47-59, 2015
                                                                      5. Kreider M et al: Pulmonary involvement in Sjögren syndrome. Semin Respir Crit Care Med. 35(2):255-64, 2014
                                                                      6. Egashira R et al: CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics. 33(7):1933-49, 2013
                                                                      7. Stojan G et al: Pulmonary manifestations of Sjögren's syndrome. Curr Allergy Asthma Rep. 13(4):354-60, 2013
                                                                      8. Tsai Y et al: Thymoma associated with myasthenia gravis and Sjögren syndrome. West Indian Med J. 62(3):264-5, 2013
                                                                      9. Borie R et al: Severe chronic bronchiolitis as the presenting feature of primary Sjögren's syndrome. Respir Med. 105(1):130-6, 2011
                                                                      10. Shi JH et al: Pulmonary manifestations of Sjögren's syndrome. Respiration. 78(4):377-86, 2009
                                                                      11. Launay D et al: Pulmonary arterial hypertension: a rare complication of primary Sjögren syndrome: report of 9 new cases and review of the literature. Medicine (Baltimore). 86(5):299-315, 2007
                                                                      12. Tonami H et al: Clinical and imaging findings of lymphoma in patients with Sjögren syndrome. J Comput Assist Tomogr. 27(4):517-24, 2003
                                                                      13. Choi YW et al: Idiopathic multilocular thymic cyst: CT features with clinical and histopathologic correlation. AJR Am J Roentgenol. 177(4):881-5, 2001
                                                                      14. Levy Y et al: Malignant thymoma associated with autoimmune diseases: a retrospective study and review of the literature. Semin Arthritis Rheum. 28(2):73-9, 1998
                                                                      15. Matsumoto Y et al: Sjögren's syndrome associated with thymoma. J Am Acad Dermatol. 35(4):639-40, 1996
                                                                      16. Suster S et al: Multilocular thymic cyst: an acquired reactive process. Study of 18 cases. Am J Surg Pathol. 15(4):388-98, 1991