Sjogren’s syndrome - Connective tissue disorders

Sjogren’s¨ syndrome

Definition

A chronic inflammatory disorder of the lacrimal and salivary glands.

Sex

9F : 1M

Aetiology

Sjogren’s¨ syndrome may be primary, or secondary to rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis or polymyositis. There is an association with non-Hogkin B cell lymphoma.

Pathophysiology

There is lymphocytic infiltration of salivary glands and other exocrine glands in the respiratory and gastrointestinal tract, the skin and the vagina. There is glandular enlargement, with atrophy of the acini and proliferation of the duct lining.

Clinical features

Ocular manifestations: Sensation of persistent grittiness, photosensitivity, tiredness and an inability to produce tears (keratoconjunctivitis sicca).

Gastrointestinal system: Lack of saliva (xerostomia) causes difficulty in chewing and swallowing. There may be oral ulcers, dental caries and firm non-tender enlargement of the parotid gland.

Other manifestations include arthralgia, Raynaud’s phenomenon and an association with other organ specific autoimmune disorders in primary Sjogren’s¨ syndrome, e.g. thyroid disease. Occasionally there are systemic features including vasculitis and renal tubu-lar defects.

Investigations

Anaemia of chronic disease, leucopenia and high ESR Rheumatoid factor (RhF) is positive in 80–100%, ANA positive in 60–80%, anti-Ro (SSa) antibodies are seen in primary Sjogren’s¨ syndrome and may cause neona

tal heart block in offspring of affected women.

Schirmer’s test for keratoconjunctivitis sicca measures tear production. An edge of a strip of filter paper is placed in the lower eyelid and the length that becomes wet is measured.

Management

Artificial tears and saliva replacement solutions provide symptomatic relief.