Sjogren's syndrome def., symptoms, signs, investigations, treatment

Here, we are going to discuss Sjogren's syndrome regarding its definition, types, associated disorders, clinical picture, diagnostic investigations and treatment .

Definition of Sjogren's syndrome : 

It is a kerotoconjunctivitis sicca with xerostomia, the disease may be primary or secondary in association with other auto immune disorder often rheumatoid disease, SLE or Iymphoproliferative diseases .

Types of Sjogren's syndrome

Primary Sjogren's syndrome i.e sicca syndrome is absence of  rheumatoid arthritis or any other a oimm
Secondary Sjogren's syndrome i.e. there is associated another autoimmune or C.T. disease e.g. rheumatoid disease, SLE, PM, primary biliary cirrhosis or thyroditis.

Associated autoimmune disorders in cases of Sjoqren's syndrome:

SLE
- Chronic active hepatitis 
- Primary biliary cirrhosis.
- Progressive systemic sclerosis.
- Myasthenia gravis .

Clinical picture of Sjogren's syndrome

• Occular dryness confirmed by schirmer tear test using a standard strip of filter paper placed under each
sjogrens-syndromelower eye lid. Wetting of < 5 mm in 5 minutes indicates defective teat production (A young person normally moistens 15 mm).
• Xerostomia .
• Salivary and parotid enlargement is seen .
• Non erosive arthritis, Raynaud's phenomenon.
• Cryoglobulinaemia.
• Peripheral neuropathy.
• Lymphadenopathy, there is increased incidence of lymphoma.
• Renal tubular acidosis, glomerulonephritis.

Investigations
Rheumatoid factor  :  +ve.
• +ve ANA in 80% of cases.
• Antisalivary duct Ab (Siogren's Ab).
• Anti Ro.
• Antiparietal cell antibodies.
• Rose Bengal staining of the eye shows punctate or filamentary keratitis.

Treatment of Sjogren's syndrome

Xerophthalmia can be treated with artificial tears e.g (hypromellose) and ,lubricating ointment at night. Soft contact lenses can be useful for corneal protection.
• Sugar free chewing gum or lozenges can stimulate saliva flow. Using a saliva substitute containing carboxy methylcellulose as mouth wash, drugs causing disease salivary secretion should be avoided (anticholinergics) .
Vaginal dryness is treated with lubricants e.g K-Y jelly.
• Extra glandular and musculoskeletal manifestations may respond to steroids, azathioprine can be added.
• If there is massive lymphadenopathy, biopsy should be performed to exclude malignancy.

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