Sjorgren's Syndrome

Question 1

pathogenesis

Answer 1

autoimmune condition, lymphocytic infiltrates in exocrine organs

Question 2

who gets it?

Answer 2

primary

secondary to other AI conditions - RA, SLE

Question 3

how does it present?

Answer 3

dryness of the eyes and mouth - sicca symptoms
other symptoms: arthralgia, fatigue, vaginal dryness, parotid gland swelling
may occur: peripheral neuropathy and ILD, increased risk of lymphoma

Question 4

how is it diagnosed?

Answer 4

American-European consensus group classification criteria (4 of the following)

• ocular symptoms
• oral symptoms
• ocular signs
• oral signs
• positive minor salivary gland biopsy findings
• positive anti-SSA/anti-SSB (Ro or LA)

Question 5

ocular symptoms

Answer 5

dry eyes for >3 months
foreign-body sensation
use of tear substitutes >3x daily

Question 6

oral symptoms

Answer 6

dry mouth
recurrently swollen salivary glands
frequent use of liquids to aid swallowing

Question 7

ocular signs

Answer 7

Schimer test - performed without anaesthesia (<5mm in 5 min)

positive vital dye staining results

Question 8

oral signs

Answer 8

abnormal salivary scrintigraphy findings
abnormal parotid sialography findings
abnormal sailometry findings
unstimulated salivary flow <1.5 ml in 25 min

Question 9

what is the management?

Answer 9

symptomatic
lubricating eye drops
saliva replacement products
pilocarpine can stimulate saliva production (side effects such as flushing)
regular dental care - high risk of caries
hydroxychloroquine - arthralgia, fatigue
organ involvement (e.g. ILD) - other immunosuppression