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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
- Primary SjS: Dry eyes & mouth
- 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
- SjS: Chronic systemic autoimmune exocrinopathy that causes salivary & lacrimal gland tissue destruction
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
- 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
- Kise Y et al: Usefulness of a deep learning system for diagnosing Sjögren's syndrome using ultrasonography images. Dentomaxillofac Radiol. 49(3):20190348, 2020
- Lee KA et al: Ultrasonographic changes of major salivary glands in primary Sjögren's syndrome. J Clin Med. 9(3):803, 2020
- Ogle OE: Salivary gland diseases. Dent Clin North Am. 64(1):87-104, 2020
- Caraba A et al: The utility of major salivary gland ultrasonographic parameters in the diagnosis of Sjögren syndrome. Dis Markers. 2019:1716848, 2019
- 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
- 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
- Kondo K et al: Primary Sjögren's syndrome with multiple calcifications in parotid glands. Clin Case Rep. 7(11):2239-41, 2019
- 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
- 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
- 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
- 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
- Triplett JD et al: Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes). J Neurol Neurosurg Psychiatry. 90(8):882-94, 2019
- 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
- Bag AK et al: Imaging of inflammatory disorders of salivary glands. Neuroimaging Clin N Am. 28(2):255-72, 2018
- 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
- Fujita A: Imaging of Sjögren syndrome and immunoglobulin G4-related disease of the salivary glands. Neuroimaging Clin N Am. 28(2):183-97, 2018
- 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
- Mariette X et al: Primary Sjögren's syndrome. N Engl J Med. 378(10):931-9, 2018
- 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
- Abdel Razek AA et al: Imaging of sialadenitis. Neuroradiol J. 1971400916682752, 2017
- 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
- 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
- Sun Z et al: Diagnostic accuracy of parotid CT for identifying Sjögren's syndrome. Eur J Radiol. 81(10):2702-9, 2012
- Lee S et al: Mikulicz's disease: a new perspective and literature review. Eur J Ophthalmol. 16(2):199-203, 2006
- Yamamoto M et al: Clinical and pathological differences between Mikulicz's disease and Sjögren's syndrome. Rheumatology (Oxford). 44(2):227-34, 2005
- Hamilton BE et al: Earring lesions of the parotid tail. AJNR Am J Neuroradiol. 24(9): 1757-64, 2003
- 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
- Berra A et al: Role of salivary IgA in the pathogenesis of Sjogren syndrome. Clin Immunol. 104(1): 49-57, 2002
- Kalk WW et al: Parotid sialography for diagnosing Sjogren syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 94(1): 131-7, 2002
- 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
- Ihrler S et al: Lymphoepithelial duct lesions in Sjogren-type sialadenitis. Virchows Arch. 434(4): 315-23, 1999
- Ohbayashi N et al: Sjögren syndrome: comparison of assessments with MR sialography and conventional sialography. Radiology. 209(3):683-8, 1998
- Tonami H et al: MR sialography in patients with Sjogren syndrome. AJNR Am J Neuroradiol. 19(7): 1199-203, 1998
- 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
- 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
- Sheikh JS et al: Reversible parotid enlargement and pseudo-Sjogren's syndrome secondary to hypertriglyceridemia. J Rheumatol. 23(7): 1288-91, 1996
- 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
- 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
- Takashima S et al: CT and MR appearances of parotid pseudotumors in Sjogren syndrome. J Comput Assist Tomogr. 16(3): 376-83, 1992
- Takashima S et al: Sjogren syndrome: comparison of sialography and ultrasonography. J Clin Ultrasound. 20(2):99-109, 1992
- Takashima S et al: MR imaging of Sjogren syndrome: correlation with sialography and pathology. J Comput Assist Tomogr. 15(3): 393-400, 1991
- 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
- Bradus RJ et al: Parotid gland: US findings in Sjogren syndrome. Work in progress. Radiology. 169(3): 749-51, 1988
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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
- Primary SjS: Dry eyes & mouth
- 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
- SjS: Chronic systemic autoimmune exocrinopathy that causes salivary & lacrimal gland tissue destruction
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
- 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
- Kise Y et al: Usefulness of a deep learning system for diagnosing Sjögren's syndrome using ultrasonography images. Dentomaxillofac Radiol. 49(3):20190348, 2020
- Lee KA et al: Ultrasonographic changes of major salivary glands in primary Sjögren's syndrome. J Clin Med. 9(3):803, 2020
- Ogle OE: Salivary gland diseases. Dent Clin North Am. 64(1):87-104, 2020
- Caraba A et al: The utility of major salivary gland ultrasonographic parameters in the diagnosis of Sjögren syndrome. Dis Markers. 2019:1716848, 2019
- 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
- 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
- Kondo K et al: Primary Sjögren's syndrome with multiple calcifications in parotid glands. Clin Case Rep. 7(11):2239-41, 2019
- 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
- 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
- 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
- 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
- Triplett JD et al: Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes). J Neurol Neurosurg Psychiatry. 90(8):882-94, 2019
- 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
- Bag AK et al: Imaging of inflammatory disorders of salivary glands. Neuroimaging Clin N Am. 28(2):255-72, 2018
- 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
- Fujita A: Imaging of Sjögren syndrome and immunoglobulin G4-related disease of the salivary glands. Neuroimaging Clin N Am. 28(2):183-97, 2018
- 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
- Mariette X et al: Primary Sjögren's syndrome. N Engl J Med. 378(10):931-9, 2018
- 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
- Abdel Razek AA et al: Imaging of sialadenitis. Neuroradiol J. 1971400916682752, 2017
- 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
- 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
- Sun Z et al: Diagnostic accuracy of parotid CT for identifying Sjögren's syndrome. Eur J Radiol. 81(10):2702-9, 2012
- Lee S et al: Mikulicz's disease: a new perspective and literature review. Eur J Ophthalmol. 16(2):199-203, 2006
- Yamamoto M et al: Clinical and pathological differences between Mikulicz's disease and Sjögren's syndrome. Rheumatology (Oxford). 44(2):227-34, 2005
- Hamilton BE et al: Earring lesions of the parotid tail. AJNR Am J Neuroradiol. 24(9): 1757-64, 2003
- 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
- Berra A et al: Role of salivary IgA in the pathogenesis of Sjogren syndrome. Clin Immunol. 104(1): 49-57, 2002
- Kalk WW et al: Parotid sialography for diagnosing Sjogren syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 94(1): 131-7, 2002
- 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
- Ihrler S et al: Lymphoepithelial duct lesions in Sjogren-type sialadenitis. Virchows Arch. 434(4): 315-23, 1999
- Ohbayashi N et al: Sjögren syndrome: comparison of assessments with MR sialography and conventional sialography. Radiology. 209(3):683-8, 1998
- Tonami H et al: MR sialography in patients with Sjogren syndrome. AJNR Am J Neuroradiol. 19(7): 1199-203, 1998
- 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
- 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
- Sheikh JS et al: Reversible parotid enlargement and pseudo-Sjogren's syndrome secondary to hypertriglyceridemia. J Rheumatol. 23(7): 1288-91, 1996
- 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
- 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
- Takashima S et al: CT and MR appearances of parotid pseudotumors in Sjogren syndrome. J Comput Assist Tomogr. 16(3): 376-83, 1992
- Takashima S et al: Sjogren syndrome: comparison of sialography and ultrasonography. J Clin Ultrasound. 20(2):99-109, 1992
- Takashima S et al: MR imaging of Sjogren syndrome: correlation with sialography and pathology. J Comput Assist Tomogr. 15(3): 393-400, 1991
- 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
- Bradus RJ et al: Parotid gland: US findings in Sjogren syndrome. Work in progress. Radiology. 169(3): 749-51, 1988
