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Parotid Sjögren Syndrome
Bronwyn E. Hamilton, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Sjögren syndrome (SjS)
            • Synonyms

              • Sicca syndrome, sicca complex
            • Definitions

              • SjS: Chronic systemic autoimmune exocrinopathy that causes salivary & lacrimal gland tissue destruction
                • Primary SjS: Dry eyes & mouth
                  • Usually no other autoimmune diseases
                  • Extraglandular manifestations can include peripheral & CNS involvement
                • Secondary SjS: Dry eyes & mouth associated with other autoimmune diseases most often RA
              • Mikulicz syndrome
                • Autoimmune exocrinopathy of salivary & lacrimal glands
                • Previously thought to be variant of primary SjS
                • Now considered part of IgG4-related disease spectrum
              • Juvenile SjS
                • Predilection for male children until teen years
                • Usually resolves with puberty

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nonvascular Interventions

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Chu C et al: Evaluation of salivary gland fat fraction values in patients with primary Sjögren's syndrome by mDIXON quant imaging: Initial findings. Eur J Radiol. 123:108776, 2020
                                              2. Kise Y et al: Usefulness of a deep learning system for diagnosing Sjögren's syndrome using ultrasonography images. Dentomaxillofac Radiol. 49(3):20190348, 2020
                                              3. Lee KA et al: Ultrasonographic changes of major salivary glands in primary Sjögren's syndrome. J Clin Med. 9(3):803, 2020
                                              4. Ogle OE: Salivary gland diseases. Dent Clin North Am. 64(1):87-104, 2020
                                              5. Caraba A et al: The utility of major salivary gland ultrasonographic parameters in the diagnosis of Sjögren syndrome. Dis Markers. 2019:1716848, 2019
                                              6. Ging K et al: Peripheral and central nervous system involvement in a patient with primary Sjögren's syndrome: a case report. J Med Case Rep. 13(1):165, 2019
                                              7. Kise Y et al: Preliminary study on the application of deep learning system to diagnosis of Sjögren's syndrome on CT images. Dentomaxillofac Radiol. 48(6):20190019, 2019
                                              8. Kondo K et al: Primary Sjögren's syndrome with multiple calcifications in parotid glands. Clin Case Rep. 7(11):2239-41, 2019
                                              9. Martín-Nares E et al: Aquaporin-4-IgG positive neuromyelitis optica spectrum disorder and systemic autoimmune diseases overlap syndrome: a single-center experience. Lupus. 28(11):1302-1311, 2019
                                              10. Milic V et al: Disease activity and damage in patients with primary Sjogren's syndrome: prognostic value of salivary gland ultrasonography. PLoS One. 14(12):e0226498, 2019
                                              11. Saha D et al: Lymphoepithelial sialadenitis involving HIV-infected and Sjogren syndrome patients: a cytologic study. Indian J Otolaryngol Head Neck Surg. 71(2):176-81, 2019
                                              12. Scofield RH et al: American Indians Have a higher risk Of Sjögren's syndrome and more disease activity than caucasians and african-americans. Arthritis Care Res (Hoboken). 72(8):1049-56, 2019
                                              13. Triplett JD et al: Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes). J Neurol Neurosurg Psychiatry. 90(8):882-94, 2019
                                              14. Świecka M et al: Imaging methods in primary Sjögren's syndrome as potential tools of disease diagnostics and monitoring. Reumatologia. 57(6):336-42, 2019
                                              15. Bag AK et al: Imaging of inflammatory disorders of salivary glands. Neuroimaging Clin N Am. 28(2):255-72, 2018
                                              16. Baldini C et al: Imaging in primary Sjögren's syndrome: the 'obsolete and the new'. Clin Exp Rheumatol. 36 Suppl 112(3):215-21, 2018
                                              17. Fujita A: Imaging of Sjögren syndrome and immunoglobulin G4-related disease of the salivary glands. Neuroimaging Clin N Am. 28(2):183-97, 2018
                                              18. Kakugawa T et al: Lymphocytic focus score is positively related to airway and interstitial lung diseases in primary Sjögren's syndrome. Respir Med. 137:95-102, 2018
                                              19. Mariette X et al: Primary Sjögren's syndrome. N Engl J Med. 378(10):931-9, 2018
                                              20. Ogura I et al: Magnetic resonance sialography and salivary gland scintigraphy of parotid glands in Sjögren's syndrome. Chin J Dent Res. 21(1):63-8, 2018
                                              21. Abdel Razek AA et al: Imaging of sialadenitis. Neuroradiol J. 1971400916682752, 2017
                                              22. Liang Y et al: Primary Sjogren's syndrome and malignancy risk: a systematic review and meta-analysis. Ann Rheum Dis. 73(6):1151-6, 2014
                                              23. Routsias JG et al: Malignant lymphoma in primary Sjögren's syndrome: an update on the pathogenesis and treatment. Semin Arthritis Rheum. 43(2):178-86, 2013
                                              24. Sun Z et al: Diagnostic accuracy of parotid CT for identifying Sjögren's syndrome. Eur J Radiol. 81(10):2702-9, 2012
                                              25. Lee S et al: Mikulicz's disease: a new perspective and literature review. Eur J Ophthalmol. 16(2):199-203, 2006
                                              26. Yamamoto M et al: Clinical and pathological differences between Mikulicz's disease and Sjögren's syndrome. Rheumatology (Oxford). 44(2):227-34, 2005
                                              27. Hamilton BE et al: Earring lesions of the parotid tail. AJNR Am J Neuroradiol. 24(9): 1757-64, 2003
                                              28. Nikitakis NG et al: Primary Sjogren syndrome in childhood: report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 96(1):42-7, 2003
                                              29. Berra A et al: Role of salivary IgA in the pathogenesis of Sjogren syndrome. Clin Immunol. 104(1): 49-57, 2002
                                              30. Kalk WW et al: Parotid sialography for diagnosing Sjogren syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 94(1): 131-7, 2002
                                              31. McGuirt WF Jr et al: The role of parotid biopsy in the diagnosis of pediatric Sjogren syndrome. Arch Otolaryngol Head Neck Surg. 128(11): 1279-81, 2002
                                              32. Ihrler S et al: Lymphoepithelial duct lesions in Sjogren-type sialadenitis. Virchows Arch. 434(4): 315-23, 1999
                                              33. Ohbayashi N et al: Sjögren syndrome: comparison of assessments with MR sialography and conventional sialography. Radiology. 209(3):683-8, 1998
                                              34. Tonami H et al: MR sialography in patients with Sjogren syndrome. AJNR Am J Neuroradiol. 19(7): 1199-203, 1998
                                              35. Izumi M et al: Premature fat deposition in the salivary glands associated with Sjogren syndrome: MR and CT evidence. AJNR Am J Neuroradiol. 18(5): 951-8, 1997
                                              36. Izumi M et al: MR imaging of the parotid gland in Sjögren's syndrome: a proposal for new diagnostic criteria. AJR Am J Roentgenol. 166(6):1483-7, 1996
                                              37. Sheikh JS et al: Reversible parotid enlargement and pseudo-Sjogren's syndrome secondary to hypertriglyceridemia. J Rheumatol. 23(7): 1288-91, 1996
                                              38. Som PM et al: Nodal inclusion cysts of the parotid gland and parapharyngeal space: a discussion of lymphoepithelial, AIDS-related parotid, and branchial cysts, cystic Warthin's tumors, and cysts in Sjögren's syndrome. Laryngoscope. 105(10):1122-8, 1995
                                              39. Honda E et al: Fractal dimensions of ductal patterns in the parotid glands of normal subjects and patients with Sjogren syndrome. Invest Radiol. 27(10): 790-5, 1992
                                              40. Takashima S et al: CT and MR appearances of parotid pseudotumors in Sjogren syndrome. J Comput Assist Tomogr. 16(3): 376-83, 1992
                                              41. Takashima S et al: Sjogren syndrome: comparison of sialography and ultrasonography. J Clin Ultrasound. 20(2):99-109, 1992
                                              42. Takashima S et al: MR imaging of Sjogren syndrome: correlation with sialography and pathology. J Comput Assist Tomogr. 15(3): 393-400, 1991
                                              43. Mizuno Y et al: Recurrent parotid gland enlargement as an initial manifestation of Sjogren syndrome in children. Eur J Pediatr. 148(5): 414-6, 1989
                                              44. Bradus RJ et al: Parotid gland: US findings in Sjogren syndrome. Work in progress. Radiology. 169(3): 749-51, 1988
                                              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 (SjS)
                                                        • Synonyms

                                                          • Sicca syndrome, sicca complex
                                                        • Definitions

                                                          • SjS: Chronic systemic autoimmune exocrinopathy that causes salivary & lacrimal gland tissue destruction
                                                            • Primary SjS: Dry eyes & mouth
                                                              • Usually no other autoimmune diseases
                                                              • Extraglandular manifestations can include peripheral & CNS involvement
                                                            • Secondary SjS: Dry eyes & mouth associated with other autoimmune diseases most often RA
                                                          • Mikulicz syndrome
                                                            • Autoimmune exocrinopathy of salivary & lacrimal glands
                                                            • Previously thought to be variant of primary SjS
                                                            • Now considered part of IgG4-related disease spectrum
                                                          • Juvenile SjS
                                                            • Predilection for male children until teen years
                                                            • Usually resolves with puberty

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Nonvascular Interventions

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Chu C et al: Evaluation of salivary gland fat fraction values in patients with primary Sjögren's syndrome by mDIXON quant imaging: Initial findings. Eur J Radiol. 123:108776, 2020
                                                                                          2. Kise Y et al: Usefulness of a deep learning system for diagnosing Sjögren's syndrome using ultrasonography images. Dentomaxillofac Radiol. 49(3):20190348, 2020
                                                                                          3. Lee KA et al: Ultrasonographic changes of major salivary glands in primary Sjögren's syndrome. J Clin Med. 9(3):803, 2020
                                                                                          4. Ogle OE: Salivary gland diseases. Dent Clin North Am. 64(1):87-104, 2020
                                                                                          5. Caraba A et al: The utility of major salivary gland ultrasonographic parameters in the diagnosis of Sjögren syndrome. Dis Markers. 2019:1716848, 2019
                                                                                          6. Ging K et al: Peripheral and central nervous system involvement in a patient with primary Sjögren's syndrome: a case report. J Med Case Rep. 13(1):165, 2019
                                                                                          7. Kise Y et al: Preliminary study on the application of deep learning system to diagnosis of Sjögren's syndrome on CT images. Dentomaxillofac Radiol. 48(6):20190019, 2019
                                                                                          8. Kondo K et al: Primary Sjögren's syndrome with multiple calcifications in parotid glands. Clin Case Rep. 7(11):2239-41, 2019
                                                                                          9. Martín-Nares E et al: Aquaporin-4-IgG positive neuromyelitis optica spectrum disorder and systemic autoimmune diseases overlap syndrome: a single-center experience. Lupus. 28(11):1302-1311, 2019
                                                                                          10. Milic V et al: Disease activity and damage in patients with primary Sjogren's syndrome: prognostic value of salivary gland ultrasonography. PLoS One. 14(12):e0226498, 2019
                                                                                          11. Saha D et al: Lymphoepithelial sialadenitis involving HIV-infected and Sjogren syndrome patients: a cytologic study. Indian J Otolaryngol Head Neck Surg. 71(2):176-81, 2019
                                                                                          12. Scofield RH et al: American Indians Have a higher risk Of Sjögren's syndrome and more disease activity than caucasians and african-americans. Arthritis Care Res (Hoboken). 72(8):1049-56, 2019
                                                                                          13. Triplett JD et al: Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes). J Neurol Neurosurg Psychiatry. 90(8):882-94, 2019
                                                                                          14. Świecka M et al: Imaging methods in primary Sjögren's syndrome as potential tools of disease diagnostics and monitoring. Reumatologia. 57(6):336-42, 2019
                                                                                          15. Bag AK et al: Imaging of inflammatory disorders of salivary glands. Neuroimaging Clin N Am. 28(2):255-72, 2018
                                                                                          16. Baldini C et al: Imaging in primary Sjögren's syndrome: the 'obsolete and the new'. Clin Exp Rheumatol. 36 Suppl 112(3):215-21, 2018
                                                                                          17. Fujita A: Imaging of Sjögren syndrome and immunoglobulin G4-related disease of the salivary glands. Neuroimaging Clin N Am. 28(2):183-97, 2018
                                                                                          18. Kakugawa T et al: Lymphocytic focus score is positively related to airway and interstitial lung diseases in primary Sjögren's syndrome. Respir Med. 137:95-102, 2018
                                                                                          19. Mariette X et al: Primary Sjögren's syndrome. N Engl J Med. 378(10):931-9, 2018
                                                                                          20. Ogura I et al: Magnetic resonance sialography and salivary gland scintigraphy of parotid glands in Sjögren's syndrome. Chin J Dent Res. 21(1):63-8, 2018
                                                                                          21. Abdel Razek AA et al: Imaging of sialadenitis. Neuroradiol J. 1971400916682752, 2017
                                                                                          22. Liang Y et al: Primary Sjogren's syndrome and malignancy risk: a systematic review and meta-analysis. Ann Rheum Dis. 73(6):1151-6, 2014
                                                                                          23. Routsias JG et al: Malignant lymphoma in primary Sjögren's syndrome: an update on the pathogenesis and treatment. Semin Arthritis Rheum. 43(2):178-86, 2013
                                                                                          24. Sun Z et al: Diagnostic accuracy of parotid CT for identifying Sjögren's syndrome. Eur J Radiol. 81(10):2702-9, 2012
                                                                                          25. Lee S et al: Mikulicz's disease: a new perspective and literature review. Eur J Ophthalmol. 16(2):199-203, 2006
                                                                                          26. Yamamoto M et al: Clinical and pathological differences between Mikulicz's disease and Sjögren's syndrome. Rheumatology (Oxford). 44(2):227-34, 2005
                                                                                          27. Hamilton BE et al: Earring lesions of the parotid tail. AJNR Am J Neuroradiol. 24(9): 1757-64, 2003
                                                                                          28. Nikitakis NG et al: Primary Sjogren syndrome in childhood: report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 96(1):42-7, 2003
                                                                                          29. Berra A et al: Role of salivary IgA in the pathogenesis of Sjogren syndrome. Clin Immunol. 104(1): 49-57, 2002
                                                                                          30. Kalk WW et al: Parotid sialography for diagnosing Sjogren syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 94(1): 131-7, 2002
                                                                                          31. McGuirt WF Jr et al: The role of parotid biopsy in the diagnosis of pediatric Sjogren syndrome. Arch Otolaryngol Head Neck Surg. 128(11): 1279-81, 2002
                                                                                          32. Ihrler S et al: Lymphoepithelial duct lesions in Sjogren-type sialadenitis. Virchows Arch. 434(4): 315-23, 1999
                                                                                          33. Ohbayashi N et al: Sjögren syndrome: comparison of assessments with MR sialography and conventional sialography. Radiology. 209(3):683-8, 1998
                                                                                          34. Tonami H et al: MR sialography in patients with Sjogren syndrome. AJNR Am J Neuroradiol. 19(7): 1199-203, 1998
                                                                                          35. Izumi M et al: Premature fat deposition in the salivary glands associated with Sjogren syndrome: MR and CT evidence. AJNR Am J Neuroradiol. 18(5): 951-8, 1997
                                                                                          36. Izumi M et al: MR imaging of the parotid gland in Sjögren's syndrome: a proposal for new diagnostic criteria. AJR Am J Roentgenol. 166(6):1483-7, 1996
                                                                                          37. Sheikh JS et al: Reversible parotid enlargement and pseudo-Sjogren's syndrome secondary to hypertriglyceridemia. J Rheumatol. 23(7): 1288-91, 1996
                                                                                          38. Som PM et al: Nodal inclusion cysts of the parotid gland and parapharyngeal space: a discussion of lymphoepithelial, AIDS-related parotid, and branchial cysts, cystic Warthin's tumors, and cysts in Sjögren's syndrome. Laryngoscope. 105(10):1122-8, 1995
                                                                                          39. Honda E et al: Fractal dimensions of ductal patterns in the parotid glands of normal subjects and patients with Sjogren syndrome. Invest Radiol. 27(10): 790-5, 1992
                                                                                          40. Takashima S et al: CT and MR appearances of parotid pseudotumors in Sjogren syndrome. J Comput Assist Tomogr. 16(3): 376-83, 1992
                                                                                          41. Takashima S et al: Sjogren syndrome: comparison of sialography and ultrasonography. J Clin Ultrasound. 20(2):99-109, 1992
                                                                                          42. Takashima S et al: MR imaging of Sjogren syndrome: correlation with sialography and pathology. J Comput Assist Tomogr. 15(3): 393-400, 1991
                                                                                          43. Mizuno Y et al: Recurrent parotid gland enlargement as an initial manifestation of Sjogren syndrome in children. Eur J Pediatr. 148(5): 414-6, 1989
                                                                                          44. Bradus RJ et al: Parotid gland: US findings in Sjogren syndrome. Work in progress. Radiology. 169(3): 749-51, 1988