-Sjögren's syndrome.

Sjögren's syndrome

(Sicca syndrome.)

*Def:-

It is chronic inflammatory autoimmune disorder in which immune cells attack and destroy the exocrine glands(1)that produce tears and saliva.

*synonymes:-

• Mikulicz disease.
• Sicca syndrome.

*It is named after Swedish ophthalmologist Henrik Sjögren(2)(1899–1986) who first described it.

*Pathology:-

It is characterized by unusual infiltration of these glands that are responsible for fluid production by lymphocytes, thus causing decreased saliva (dry mouth), decreased tear production (dry eyes), and drying of other mucous membranes.

*Types:-

1-TypeI(1ry or glandular type):-

sicca symptoms(dry mouth and dry eyes):-

• Difficulty chewing or swallowing.
• Hoarseness.
• High incidence of dental decay and periodontal disease.
• Halitosis.
• painless parotid enlargment.
• Red, itchy, or painful eyes.
• Gritty or sandy sensation in the eyes.
• Blurry vision.

2-Type II (2ry or extraglandular):-The same as type I plus:-

• Collagen diseases as Rheumatoid arthritis (30%) and SLE (10%).
• Lymphadenopathy (high incidence of lymphoma)
• Hashimoto's thyroiditis
• Renal affection.

*Diagnosis:-

• Anti-nuclear antibody (ANA)
• Rheumatoid factor
• Antibodies specific to Sjögren’s syndrome: Anti-SS-A (also called Ro) and Anti-SS-B (also called La)- (frequently positive)
• HLA-DR positive glandular cells
• Salivary gland biopsy
• Positive Schrimer’s test and Rose Bengal staining of eye (cornea or conjunctiva)
• Ultrasound examination of the salivary glands is the simplest confirmatory test and has the added advantage of being non-invasive with no complications. The parenchyma of the gland demonstrates multiple, small-2-6 mm hypoechoic lesions which are representations of the lymphocytic infiltrates. Often sialectasis with calculi are demonstrated if the disease is advanced.

*Treatment:-

Treatment is based on the symptoms:-

• Dry eyes are treated with artificial tears, a tear stimulant, or eye lubricant.
• Dry mouth may be helped by frequent small drinks of water or chewing gum to stimulate saliva production.
• Arthritis symptoms are treated with anti-inflammatory medicines, such as aspirin, acetaminophen, and other NSAIDs.

Dr Ibrahim

Return to list of medical syndromes here

*Reference:-

(1) Delaleu N, Immervoll H, Cornelius J, Jonsson R (2008). "Biomarker profiles in serum and saliva of experimental Sjögren's syndrome: associations with specific autoimmune manifestations". Arthritis Res. Ther. 10 (1): R22. doi:10.1186/ar2375. PMID 18289371. PMC 2374466..
(2)Sjögren H. Zur Kenntnis der keratoconjunctivitis sicca. Doctoral thesis, 1933.