Salivary glands
Inflammation
Sjögren syndrome


Topic Completed: 1 October 2015

Revised: 20 March 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Sjögren syndrome[TI] salivary[TI]
Page views in 2018: 11,548
Page views in 2019 to date: 5,641
Cite this page: Tanakchi S Sjögren syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/salivaryglandssjogren.html. Accessed June 17th, 2019.
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

Images hosted on other servers:
Missing Image

Typical focal lymphocytic infiltrate

Missing Image

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
Back to top