Autoimmune condition that affects the exocrine glands, characterised by lymphocytic infiltrates
Aetiology
Higher prevalence in females (9:1), occurs mainly in middle aged women
Can be a primary condition or can occur secondary to other autoimmune conditions (RA, SLE)
Pathophysiology
As the immune system mainly attacks lacrimal and salivary glands, patients typically present with dry eyes and mouth
Clinical presentation
Symptoms
Dry eyes - gritty feeling
Dry mouth
May lead to fissured tongue
Dry throat
Vaginal dryness
Joint pains
Fatigue
Signs
Bilateral parotid gland enlargement
Unexplained increase in dental caries
Investigations
Schirmers test - ocular dryness
Bloods - positive anti-Ro and Anti-La antibodies
May also have raised IgG and raised plasma viscosity/ESR
Lymphocytic infiltrates on a lip gland biopsy
Management
Symptom control
Tear and salivary replacement for dryness
Analgesia, exercise for pain and fatigue
Hydroxychloroquine can sometimes help with arthralgia and fatigue
Other immunosuppression would usually only be used in the context of organ involvement e.g. interstitial lung disease
Complications
Increased risk of lymphoma
Peripheral neuropathy and interstitial lung disease can occur but Sjorgen's is less commonly associated with organ complications than other connective tissue diseases - surveillance not performed
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