Sjogren’s Syndrome

Sjogren’s Syndrome (Sicca syndrome, Mickulicz’s disease, Autoimmune exocrinopathy)

Chronic progressive systemic inflammatory autoimmune disease associated with some rheumatologoical disorders (rheumatoid arthritis, SLE, MCTD) that share certain autoimmune features primarily affecting exocrine glands. 90% are female. Most over age of 40. Characteristic is the sicca complex of dry eyes and dry mouth. Characterised by lymphocytic and plasma cell infiltration of the lacrimal and salivary glands  dry eyes and mouth. It is believed that most cases may be undiagnosed.

Can be primary or associated with other diseases (secondary) – generally up to 50% have rheumatoid arthritis; 5-8% scleroderma; 5-10% SLE; 2-4% polymyositis.

Associated with HLA-DR3, B8 and –DRw52 antigens. Underlying pathology results from the lymphocytic infiltration of glandular and nonglandular organs. Dysfunction of salivary and lacrimal glands is characteristic. The autoimmune response to a possible infectious agent (probably a retrovirus) is widely believed, but has had some critics .

Clinical features:
Dryness of mouth (xerostomia), keratoconjunctivitis sicca (dry eyes) and dryness of other mucous membranes (sicca symptoms). Often have a gritty sensation in eyes. 20-35% have Raynauds. Can have kidney, gastrointestinal and respiratory symptoms. Fatigue is common.
Joints symptoms – non-erosive form with similar distribution to rheumatoid arthritis (morning stiffness, intermittent synovitis and chronic polyarthritis).
Peripheral sensorimotor neuropathy can develop due to vasculitis (a subclinical neuropathy is very common)
Characteristic antibodies are produced – Ro(SS-A) and La(SS-B)

Differential diagnosis – DILS (diffuse infiltrative lymphocytosis syndrome)  features that are similar to Sjogren’s that occurs in those with HIV infection.

Treatment:
Hydroxychloroquine, methotrexate or hydrocortisone.
Main treatment is symptomatic management of eye (eye drops) and mouth dryness – especially good dental care. Arthritis generally responds well to NSAID’s.

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