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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

              • Autoimmune disease characterized by dryness of eyes and mouth
                • Caused by chronic lymphocytic infiltration of exocrine glands and other extraglandular sites
              • Second most common autoimmune disorder after rheumatoid arthritis
                • SS can be primary or associated with other connective tissue diseases, most commonly rheumatoid arthritis
                • Clinically significant pulmonary involvement in 9-24% of affected patients and more common in secondary than in primary SS
              • Pulmonary manifestations: Interstitial lung disease (ILD), airways disease, and lymphoproliferative disorders
              • ILD
                • Nonspecific interstitial pneumonia (NSIP) is most common ILD pattern in patients with SS (~ 45%)
              • Airways disease
                • Small airways disease
                  • Chronic bronchiolitis
                  • Follicular bronchiolitis,
                  • Constrictive bronchiolitis
                • Upper airway inflammation and dryness
              • Lymphoproliferative disorders
                • Increased risk of non-Hodgkin lymphoma (NHL)
                • Marginal zone B cell lymphoma and mucosa-associated lymphoid tissue (MALT) lymphoma or MALToma: Most common subtypes in SS

            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

                                  Selected References

                                  1. Heus A et al: Pulmonary involvement in primary Sjögren's syndrome, as measured by the ESSDAI. Scand J Rheumatol. 49(1):38-46, 2020
                                  2. Luppi F et al: Lung complications of Sjogren syndrome. Eur Respir Rev. 29(157), 2020
                                  3. Chung A et al: Pulmonary and bronchiolar involvement in Sjogren's syndrome. Semin Respir Crit Care Med. 40(2):235-54, 2019
                                  4. Natalini JG et al: Pulmonary involvement in Sjögren syndrome. Clin Chest Med. 40(3):531-44, 2019
                                  5. Ohno Y et al: State-of-the-art imaging of the lung for connective tissue disease (CTD). Curr Rheumatol Rep. 17(12):69, 2015
                                  6. Ruano CA et al: Thoracic manifestations of connective tissue diseases. Curr Probl Diagn Radiol. 44(1):47-59, 2015
                                  7. Kreider M et al: Pulmonary involvement in Sjögren syndrome. Semin Respir Crit Care Med. 35(2):255-64, 2014
                                  8. Egashira R et al: CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics. 33(7):1933-49, 2013
                                  9. Stojan G et al: Pulmonary manifestations of Sjögren's syndrome. Curr Allergy Asthma Rep. 13(4):354-60, 2013
                                  10. Tsai Y et al: Thymoma associated with myasthenia gravis and Sjögren syndrome. West Indian Med J. 62(3):264-5, 2013
                                  11. Borie R et al: Severe chronic bronchiolitis as the presenting feature of primary Sjögren's syndrome. Respir Med. 105(1):130-6, 2011
                                  12. Shi JH et al: Pulmonary manifestations of Sjögren's syndrome. Respiration. 78(4):377-86, 2009
                                  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

                                              • Autoimmune disease characterized by dryness of eyes and mouth
                                                • Caused by chronic lymphocytic infiltration of exocrine glands and other extraglandular sites
                                              • Second most common autoimmune disorder after rheumatoid arthritis
                                                • SS can be primary or associated with other connective tissue diseases, most commonly rheumatoid arthritis
                                                • Clinically significant pulmonary involvement in 9-24% of affected patients and more common in secondary than in primary SS
                                              • Pulmonary manifestations: Interstitial lung disease (ILD), airways disease, and lymphoproliferative disorders
                                              • ILD
                                                • Nonspecific interstitial pneumonia (NSIP) is most common ILD pattern in patients with SS (~ 45%)
                                              • Airways disease
                                                • Small airways disease
                                                  • Chronic bronchiolitis
                                                  • Follicular bronchiolitis,
                                                  • Constrictive bronchiolitis
                                                • Upper airway inflammation and dryness
                                              • Lymphoproliferative disorders
                                                • Increased risk of non-Hodgkin lymphoma (NHL)
                                                • Marginal zone B cell lymphoma and mucosa-associated lymphoid tissue (MALT) lymphoma or MALToma: Most common subtypes in SS

                                            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

                                                                  Selected References

                                                                  1. Heus A et al: Pulmonary involvement in primary Sjögren's syndrome, as measured by the ESSDAI. Scand J Rheumatol. 49(1):38-46, 2020
                                                                  2. Luppi F et al: Lung complications of Sjogren syndrome. Eur Respir Rev. 29(157), 2020
                                                                  3. Chung A et al: Pulmonary and bronchiolar involvement in Sjogren's syndrome. Semin Respir Crit Care Med. 40(2):235-54, 2019
                                                                  4. Natalini JG et al: Pulmonary involvement in Sjögren syndrome. Clin Chest Med. 40(3):531-44, 2019
                                                                  5. Ohno Y et al: State-of-the-art imaging of the lung for connective tissue disease (CTD). Curr Rheumatol Rep. 17(12):69, 2015
                                                                  6. Ruano CA et al: Thoracic manifestations of connective tissue diseases. Curr Probl Diagn Radiol. 44(1):47-59, 2015
                                                                  7. Kreider M et al: Pulmonary involvement in Sjögren syndrome. Semin Respir Crit Care Med. 35(2):255-64, 2014
                                                                  8. Egashira R et al: CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics. 33(7):1933-49, 2013
                                                                  9. Stojan G et al: Pulmonary manifestations of Sjögren's syndrome. Curr Allergy Asthma Rep. 13(4):354-60, 2013
                                                                  10. Tsai Y et al: Thymoma associated with myasthenia gravis and Sjögren syndrome. West Indian Med J. 62(3):264-5, 2013
                                                                  11. Borie R et al: Severe chronic bronchiolitis as the presenting feature of primary Sjögren's syndrome. Respir Med. 105(1):130-6, 2011
                                                                  12. Shi JH et al: Pulmonary manifestations of Sjögren's syndrome. Respiration. 78(4):377-86, 2009