Sjögren syndrome
Description
- Chronic autoimmune exocrinopathy: progressive lymphocytic infiltration of salivary and lacrimal glands
- Classic triad: sicca (dry eyes + dry mouth), fatigue, pain
- Primary - isolated
- Secondary - alongside another CTD
- RA > SLE > systemic sclerosis (also dermatomyositis, PBC, autoimmune thyroid disease)
Two disease axes - they do not track together
- Glandular (sicca) - burdensome, rarely dangerous
- Extra-glandular / systemic - drives morbidity and mortality
- Sicca severity does not predict systemic disease
In children
- Recurrent parotitis is the commonest presentation, then sicca (dry mouth, halitosis, painful oral mucosa)
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