sjögren’s syndrome Flashcards
What is Sjögren’s syndrome?
A chronic autoimmune disorder where the body’s immune system attacks its own salivary glands and lacrimal glands resulting in dry eyes and dry mouth
What is Sicca syndrome?
Dry eyes or mouth but not both
What is primary and secondary Sjögren’s?
Primary - no connective tissue disease
Secondary - connective tissue disease present such as SLE, rheumatoid arthritis or scleroderma
What is the indidence of SS?
0.2-1.2% of people affected
Female:male ratio is 10:1
Lifespan not affected
Describe the systemic involvement of SS
Can affect lungs, kidneys, liver, pancreas, blood vessels and nervous system
Sometimes causes general fatigue and chronic pain
Any features in lupus can also be skin in SS
What are the aetiological components of SS?
Genetic and environmental
Describe the genetic aetiology of SS
No specific inheritance
Association with Anti-Ro and Anti-La seems genetic
Low oestrogen gives risk of connective tissue disease
Describe the environmental aetiology of SS
EBV associated - weak evidence
EBV also associated with lymphoma
What are the consequences of SS?
Gradual loss of salivary/lacrimal gland tissue through inflammatory destruction
Enlargement of major salivary glands - usually symmetrical (sialosis)
Increase risk of any lymphoma (5%) and MALT lymphoma
Oral and ocular effects of loss of saliva and tears
What are the oral and ocular effects of loss of saliva and tears?
Caries risk
Oral infection risk
Loss of lubricating and taste function associated with saliva
Loss of lubrication of eyes from lack of tear production
How is SS diagnosed?
Uses ACR-EULAR (2016) - if pt scores ≥4, they can be diagnosed with SS
Histopathological findings (labial biopsy) - 3
Autoantibody findings (anti-la, anti-ro) - 3
Dry eyes/mouth through objective salivary flow and Schirmer test - 1
Ultrasound - 1
Ocular staining score - 1
What are the oral symptoms of SS?
Daily feeling of a dry mouth for >3 months
Recurrent swelling of salivary glands as an adult
Frequently drink liquid to aid swallowing dry foods
What are the ocular symptoms of SS?
Persistent troublesome dry eyes for >3 months
Recurrent sensation of sand/gravel in the eyes
Tear substitutes used >3 times a day
What are the ocular signs of SS?
Abnormal Schirmer test - <5mm wetting in 5 minutes
Fluorescein tear film assessment - green stain
What are the oral signs of SS?
Abnormal unstimulated whole salivary flow (UWS) - <1.5ml in 15 minutes