Sjögren's syndrome

Question 1

Define Sjögren’s syndrome

Answer 1

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

Question 2

Aetiology of Sjögren’s syndrome

Answer 2

Exact unknown
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
HLA-A1, -B8, or -DR3/DQ2 thought to be linked
Associated with Anti-Ro and anti-La

Secondary: 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

Epidemiology of Sjögren’s syndrome

Answer 4

Most common of all systemic auto-immune rheumatic diseases
Female:male ratio 9:1
Age peak after first menarche (20-30) and second after menopause (mid-50s)

Question 5

Symptoms of Sjögren’s syndrome

Answer 5

Dry eyes:
- sensation of sand/gravel/itch/burning
- >3 months, multiple episodes a week
- Sensitivity to light and wind
Dry mouth: burning, enlarged salivary glands but no saliva
Dry nose
Fatigue: sleep disturbance, reduced physical capacity
Dry vagina → dyspareunia
Dry cough, dysphagia, heartburn

Vasculitis - skin rash, symptoms of polyarteritis nodosa
Dental caries (destruction around the necks of the teeth and on labial and incisal surfaces)
Alopecia
Memory and speech difficulties
Numbness in extremities (peripheral neuropathy)
Arthralgia and myalgia

Question 6

Signs of Sjögren’s syndrome on examination

Answer 6

Dry eyes, corneal ulceration
Dry mouth, tongue
Enlarged salivary/parotid glands (bilateral)

Question 7

Investigations for Sjögren’s syndrome

Answer 7

Schirmer’s test: positive (<5mm wet after 5 mins)
Urinalysis
Sialometry: decreased

Abs: anti-Ro(70%) and Anti-La (30%), rheumatoid factor (50%)
U&Es

Fluorescein corneal staining: 3 or more
Parotid sialography: gross distortion of normal parotid ductules + retention of contrast
Salivary gland scintigraphy: decreased uptake + secretion
Minor salivary gland biopsy: Focus score 1 or greater (increased cellular infiltrates)
Labial gland biopsy: Lymphocytic infiltration/aggregation (gold standard)
Skin biopsy: ? vasculitis
Angiography: ? vessel involvement

Question 8

Management for Sjögren’s syndrome

Answer 8

Tear substitutes
Saliva substitutes
Pilocarpine (cholinergic agonist) to stimulate saliva
Analgesia: NSAIDs
Hydroxychloroquine and prednisolone (immunosuppression)

Requires specialist O&G management as Abs can cross placenta → heart block

Question 9

Complications of Sjögren’s syndrome

Answer 9

Increased risk of Lymphoma (MALT)
Autoimmune thyroid disease, haemolytic anaemia, primary biliary cirrhosis
Impacts WOL: visual impairment, driving, reading, working, sleep
Anxiety and depression
Punctate epithelial erosions
Corneal scarring, thinning, ulceration, neovascularisation
Corneal infection or perforation

Question 10

Prognosis for Sjögren’s syndrome

Answer 10

lifespan is generally not shortened
Dry eye disease is usually not curable but symptoms can generally be improved with treatment and modification of underlying causes