Salivary glands
Inflammation
Sjögren syndrome

Author: Sally Tanakchi (see Authors page)
Editor: F. Zahra Aly, M.D., Ph.D.

Revised: 12 March 2018, last major update October 2015

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Sjögren syndrome[TI] salivary[TI]
Cite this page: Tanakchi, S. Sjögren syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/salivaryglandssjogren.html. Accessed April 20th, 2018.
Definition / general
  • Sjögren syndrome is the third most common rheumatic disorder, after rheumatoid arthritis and systemic lupus erythematosus
  • Systemic autoimmune disease that presents typically with xerostomia (dry mouth), keratoconjunctivitis sicca (dry eyes), rheumatoid arthritis and hypergammaglobulinemia
  • Incidence is 3 - 6 per 100,000 per year; estimated prevalence of up to 3% of population
  • Female:male ratio is 9:1 with average age of onset between 40 - 60 years
  • Occasionally involves lymph nodes, lung, kidney, bone marrow, skeletal muscle, skin, liver
  • Associated with autoimmune thyroiditis, systemic vasculitis and MALT lymphoma
  • Variable amyloid deposition outside salivary glands
Diagnosis
  • Requires presence of a number of clinical and pathologic features
  • Proposed diagnostic criteria: adequate biopsy of minor salivary glands (5 or more glands) with a focus score of more than 1 focus/4 mm2
Case reports
Microscopic (histologic) description
  • Extensive lymphoid infiltrate with germinal centers, often interstitial fibrosis and acinar atrophy
  • Usually no / rare epimyoepithelial islands in minor salivary glands, although may appear in major salivary glands
Microscopic (histologic) images

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Typical focal lymphocytic infiltrate

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Podoplanin

Differential diagnosis
  • Nonspecific chronic sialadenitis: may show diffuse atrophy of glandular epithelium in lobes or entire glands, duct dilatation and interstitial fibrosis; inflammatory infiltrate may be focal but is commonly interstitial and scattered