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
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
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
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
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
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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