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Sjogren’s Syndrome – Quick review

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Sjogren’s syndrome is an auto-immune disease characterized predominantly by destruction of exocrine glands, particularly salivary and eye glands.

May be primary or secondary when it occurs in association with other connective tissue disorders (e.g. Rheumatoid Arthritis)

Pathogenesis

  • Auto-immune with a speculative role of molecular mimicry of environmental pathogens
  • Auto-antibody titers (Anti-Ro & Anti-La) do not correlate with disease activity

Clinical Criteria

  1. Ocular symptoms
    1. Persistent, troublesome dry eyes every day > 3 months
    2. Recurrent sensation of sand or gravel in the eyes
    3. Use of tear substitutes > 3 times/day
  3. Oral symptoms
    1. Feeling of dry mouth every day > 3 months
    2. Recurrent feeling of swollen salivary glands as an adult
    3. Need to drink liquids to aid swallowing food
  5. Objective evidence of dry eyes
    1. Schirmer Test
    2. Rose Bengal Staining
  7. Objective evidence of salivary gland involvement
    1. Salivary gland scintigraphy
    2. Parotid serigraphy
    3. Unstimulated whole sialomentry
  9. Lacrimal gland biopsy with changes suggestive of Sjogren’s Syndrome
  10. Auto-antibodies
    1. Anti SS-A (Ro) or Anti SS-B (La)
    2. ANA
    3. IgM Rheumatoid factor

For primary Sjogren’s syndrome four out of six criteria are required (including positive biopsy or antibodies).

For secondary Sjogren’s syndrome an established diagnosis of a connective tissue disorder plus two objective tests of dry mouth or eyes are required.

Exclusions to the criteria

  1. Previous radiotherapy to head & neck
  2. Lymphoma
  3. Sarcoidosis
  4. Graft vs Host disease
  5. Certain infections e.g. hepatitis C, HTLV-1, HIV

Symptom complexes can be divided into glandular (involvement of exocrine glands) and extra-glandular.

Glandular symptoms

  1. Sicca complex (xerophthalmia & xerostomia)
  2. Nose/throat – e.g. Epistaxis, hoarseness of voice
  3. Pulmonary – e.g. Dry cough
  4. Genitourinary- e.g. Dyspareunia, dysuria, vaginal dryness

Extra-glandular symptoms

  1. Skin & vascular – e.g. Vasculitis, Raynaud’s, photosenstivity
  2. Airways – Interstitial pneumonitis, COPD, BOOP
  3. Musculoskeletal – Polyarthritis, myopathy, polymyositis
  4. Renal – Renal tubular acidosis, interstitial nephritis, glomerulonephritis
  5. Neurologic – Peripheral neuropathy, Multiple sclerosis like lesions, TIA & stroke, Cognitive dysfunction, spinal cord dysfunction including transverse myelitis
  6. Gastrointestinal – Hepatomegaly, Primary biliary cirrhosis
  7. Neoplasia – Lymphadenopathy, lymphoma, pseudolymphoma
  8. Association with other auto-immune syndromes

Treatment

Symptomatic – artificial tears, artificial saliva etc

NSAIDS, Hydroxychloroquine, Prednisone, Methotrexate

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This entry was posted on Tuesday, February 24th, 2009 at 2:00 pm and is filed under Review. You can follow any responses to this entry through the RSS 2.0 feed. You can leave a response, or trackback from your own site.

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