sjorgren's syndrome

Question 1

definition of sjogren syndrome

Answer 1

Sjogren syndrome is a systemic auto-immune disorder[1] characterised by the presence of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) as a consequence of lymphocytic infiltration into the lacrimal and salivary glands. Additional symptoms can include dryness of skin, nose, throat, vagina; arthralgias and myalgias; peripheral neuropathies; pulmonary, thyroid, and renal disorders; and lymphoma.[2] Sjogren syndrome can occur alone (primary Sjogren syndrome) or along with another auto-immune disease (secondary Sjogren syndrome) such as lupus, rheumatoid arthritis, and systemic sclerosis. treatment is mainly symptomatic.

Question 2

epidemiology

Answer 2

Possibly the most common of all systemic auto-immune rheumatic diseases, Sjogren syndrome is a systemic auto-immune disorder with a female-to-male ratio of 9:1 and a population prevalence of between 0.5% and 1.56%. About 60% have the disease secondary to rheumatoid arthritis, systemic lupus erythematosus (SLE), or systemic sclerosis. Two age peaks have been identified, the first after menarche (20s to 30s) and the second following the menopause (mid-50s)

Question 3

points for initial diagnosis

Answer 3

Initial attempt to establish diagnosis would target the following 4 criteria:

Eye symptoms: subjective dry eyes persisting more than 3 months
Oral symptoms: dry mouth waking from sleep to drink, persisting more than 3 months
Objective eye sign: Schirmer’s test decreased tear production (<5 mm in 5 minutes)
Auto-antibodies: anti-Ro and/or anti-La positive.

Question 4

symptoms and signs

Answer 4

• fatigue
• dry eyes
• dry mouth
• dental caries
• vasculitis

other signs

• burning mouth syndrome
• arthritis
• arthralgia
• myalgia
• increased oral fungal and bacterial infections
• kidney disease
• peripheral neuropathy
• corneal ulceration

Question 5

risk factors

Answer 5

female
SLE
RA
systemic sclerosis (scleroderma)
HLA class 2 markers

Question 6

investigations

Answer 6

1st investigations:

• schrimer’s test => positive (quantitative measurement of tears in eyes)
• anti-60 kD (SS-A) and anti-La (SS-B)