Overview
Definition
An autoimmune disorder characterized by lymphocytic infiltration and destruction of the exocrine glands, primarily the lacrimal (tear) and salivary glands.
Epidemiology
Predominantly affects women (9:1 ratio), usually between 40-60 years old.
Etiology & Risk Factors
- Autoimmune destruction of exocrine glands. Can be primary (standing alone) or secondary (associated with RA or SLE).
Clinical Symptoms
- Xerophthalmia (severe dry eyes) - feeling of 'sand in the eyes'
- Xerostomia (severe dry mouth) - difficulty swallowing dry foods, severe dental caries (cavities)
- Bilateral parotid gland enlargement
- Extraglandular: Joint pain, fatigue, Raynaud's
Clinical Approach
Diagnosis
- Antibodies: Anti-Ro (SSA) and Anti-La (SSB) are highly characteristic
- Schirmer Test: Measures tear production using filter paper (< 5mm of wetting in 5 mins is positive)
- Lip/Salivary Gland Biopsy (Gold Standard): Shows focal lymphocytic sialadenitis
Management
- Symptomatic relief: Artificial tears, frequent water sips, sugar-free gum
- Cholinergic agonists (Pilocarpine, Cevimeline): Stimulate residual glandular function to increase saliva and tears
- Good dental hygiene (high risk of rampant cavities)
Complications
- Corneal ulceration (from severe dryness)
- MALT Lymphoma (Mucosa-Associated Lymphoid Tissue Lymphoma) - patients have a massively increased risk (up to 40x) of B-cell lymphoma