42 Salivary conditions: Sjögren syndrome

42 Salivary conditions: Sjögren syndrome

Figure 42.1 Causes of Sjögren syndrome (SS).

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Figure 42.2 Secondary Sjögren syndrome (SS-2).

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Figure 42.3 Primary Sjögren syndrome (sicca syndrome).

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Figure 42.4 Dry mouth.

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Figure 42.5 Dry mouth.

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Figure 42.6 Salivary swelling in SS.

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Figure 42.7 Hand deformities in rheumatoid arthritis in SS.

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Figure 42.8 Algorithm for diagnosis of SS.

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Figure 42.9 Sjögren syndrome focal lymphocytic adenitis.

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Definition: The association of dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca).

Prevalence (approximate): Uncommon.

Age mainly affected: Older people.

Gender mainly affected: F > M.

Etiopathogenesis: A benign autoimmune inflammatory exocrinopathy (epithelitis) directed against alpha fodrin, a cytoskeletal protein involved in actin binding, with lymphocyte-mediated destruction of salivary, lacrimal and other exocrine glands. Tumor necrosis factor (TNF), interferon (IFN) and B cell activating factor (BAFF) are implicated. A viral etiology, possibly human retrovirus 5 (HRV-5), and a genetic predisposition may be implicated. A SS type of disease may follow HIV, EBV, HCV, or Helicobacter pylori infection, or graft-versus-host disease (Figure 42.1).

Most common is secondary SS (SS-2) which comprises dry eyes and dry mouth and an autoimmune disease – usually primary biliary cirrhosis or a connective tissue disease such as rheumatoid arthritis (Figure 42.2).

The same features in the absence of systemic autoimmune disease are termed primary SS (SS-1 or sicca syndrome) (Figure 42.3).

Diagnostic features

History

Oral: May include:

  • xerostomia and sequelae (Chapter />
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Jan 12, 2015 | Posted by in Oral and Maxillofacial Pathology | Comments Off on 42 Salivary conditions: Sjögren syndrome
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