Sjögren's sydnrome

Question 1

Define Sjögren’s Syndrome

Answer 1

Systemic auto-immune disorder characterised by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia)

Question 2

Aetiology of Sjögren’s Syndrome

Answer 2

Due to lymphocytic infiltration into the lacrimal and salivary glands, as well as other exocrine glands therefore affecting the eyes, nose, mouth, vagina and larynx
Associated with Anti-Ro and anti-La
60% have the disease secondary the rheumatoid arthritis, SLE or systemic sclerosis

Question 3

Risk factors for Sjögren’s Syndrome

Answer 3

Female
SLE
Rheumatoid arthritis 
Systemic sclerosis 
HLA class II markers
Family history

Question 4

Symptoms of Sjögren’s Syndrome

Answer 4

=> Eyes
Dry eyes (recurrent sand/gravel sensation, itch, burning | >3 months | multiple episodes per week)
Eye redness
Sensitivity to light and wind 
Corneal ulceration

=> Mouth (xerostomia)
Burning mouth syndrome
Enlarged salivary glands with no saliva pool

Dry nose
Fatigue, sleep disturbance, reduced physical capacity 
Dry vagina -> dyspareunia
Dry cough
Dysphagia, heartburn 
Alopecia 
Memory and speech difficulties 
Numbness in extremities (peripheral neuropathy)
Arthralgia and myalgia

Question 5

Signs of Sjögren’s Syndrome on examination

Answer 5

Parotid or salivary gland enlargement
Dry eyes - redness, corneal ulceration
Dry mouth or tongue
Dental caries (destruction around the necks of the teeth and on labial and incisal surfaces)

Question 6

Investigations for Sjögren’s Syndrome

Answer 6

Schirmer’s test: Positive (<5mm of paper is wetted after 5 minutes)
Anti-Ro and Anti-La: positive
Minor (labial) salivary gland biopsy: lymphocytic infiltration/aggregation

Sailometry: Reduced
Fluorescein corneal staining: score of 3 or more
Urinalysis + electrolytes: ?renal tubular acidosis

Parotid sialography: gross distortion of normal pattern of parotid ductules
Salivary gland scintegraphy: decrease uptake and secretion

Skin biopsy: Vascultitis
Angiography: assess for large vessel involvement