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Sjogren’s syndrome is a late onset chronic systemic autoimmune disease characterized by lymphocytic infiltration and destruction of the salivary and lacrimal glands resulting in dry mouth(xerostomia) and dryness and atrophy of conjunctiva and cornea (keratoconjunctivits sicca).
- Swedish Ophthalmologist Henrik Sjögren who first described it (1933)
- It is an autoimmune disorder.
- Autoimmune ; HLA-B8 / DR3 ,although DR4 is more closely associated with syndrome occuring with raynaud’s phenomenon
- Antibodies to the Ro antigen occur in excess in relatives of pts with sjogren’s syndrome.
- It is more commonly seen in females.
- It can be primary when it is called sicca syndrome and it may also be secondary to other autoimmune disorders; rheumatoid arthritis being most commonly associated disorder.
There are two forms of Sjögren's syndrome:
Primary Sjögren's syndrome develops on its own, not because of any other health condition.
Secondary Sjögren's syndrome develops in addition to other autoimmune diseases like rheumatoid arthritis, lupus and psoriatic arthritis.
The two main symptoms of Sjogren's syndrome are:
Dry eyes. -eyes might burn, itch or feel gritty
Dry mouth. -mouth might feel like it's full of cotton, making it difficult to swallow or speak.
The major signs and symptoms are:
- Dryness of skin.
- Dryness of eyes.
- Dryness of nose.
- Dryness of vagina.
- Rheumatoid arthritis.
Other minor signs and symptoms include:
- Gastrointestinal problems.
- Inflammation of lungs, kidneys, liver or pancreas.
- Ache or pain in the muscles and joints
Investigations
1. Schirmer tear test : which measures tear flow over 5 minutes using absorbent paper strips placed on the lower eyelid, normal result is more than 6mm of wetting. |
2. Salivary gland biopsy : demonstrating focal lymphocytic infiltrate. |
3. Most patients have an elevated ESR and hypergammaglobulinemia. |
4. Autoantibodies frequently detected including: rheumatoid factor, antinuclear antibody, anti-Ro and anti-Lo. |
5. Chest x-ray and lung function test should be performed. Note: antinuclear antibody negative disease exists. |
Management
- Lacrimal substitutes should used during the day in combination with more viscous lubricating application at night.
- Soft contact lenses for corneal protection in patients with filamentary keratitis.
- Artificial saliva sprays, saliva-stimulating tablets, and pastilles and oral gels can be tried for xerostomia but often chewing gum is most effective.
- Postprandial oral hygiene and prompt treatment of oral candidiasis.
- Immunosuppression dose not improve sicca symptoms but is essential for progressive interstitial lung disease( glucocorticoid and cyclophosphamide) and for interstitial nephritis (if hydroxychloroquine is ineffective alone)
