Salivary glands
Sjogren syndrome

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

Revised: 29 October 2015, last major update October 2015

Copyright: (c) 2002-2015,, Inc.

PubMed Search: Sjogren syndrome [title] salivary
Definition / General
  • Sjogren 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
  • 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/4mm square
Case Reports
Micro 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
Micro Images
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Typical focal lymphocytic infiltrate

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Differential Diagnosis
  • Non-specific 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