Salivary glands
Inflammatory
Sjögren syndrome
Last author update: 1 October 2015
Last staff update: 23 March 2023 (update in progress)
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PubMed Search: Sjögren syndrome[TI] salivary[TI]
Table of Contents
Definition / general | Diagnosis | Case reports | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosis | Additional referencesCite this page: Tanakchi S, Aly FZ. Sjögren syndrome. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/salivaryglandssjogren.html. Accessed June 1st, 2023.
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
- 50 year old woman (J Ultrasound 2012;15:108)
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:
Typical focal lymphocytic infiltrate
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
Additional references
