Sjorgens

Question 1

Define Sjorgens syndrome

Answer 1

Inflamation (AID) and destruction of exocrine glands (saliva, tears, lacrimal etc)
Lymphocyte infiltrate the glands, hyperreactivity of the B cells to nuclear Ab, but different from SLE
Few relations with genetics it seems

Risk factors:
HLA DR3
Female
SLE/RA

Question 2

Signs and Sx of Sjorgens syndrome

Answer 2

Swollen parotid glands,
dry eyes (very red, patients will say)
dry tongue (no saliva)
large submandibular mouths

dry eyes: keratoconjunctivitis sicca
dry mouth
vaginal dryness
arthralgia
Raynaud’s, myalgia
sensory polyneuropathy
recurrent episodes of parotitis
renal tubular acidosis (usually subclinical)

Question 3

Investigations of Sjorgens syndrome

Answer 3

Positive to ANA - especially anti-Ro and anti-La
also Rheumathoid factor positive

Lip gland biopsy-foci of lymphocyte infiltrates

anti-La (SSB) antibodies in 30% of patients with PSS
Schirmer’s test: filter paper near conjunctival sac to measure tear formation
histology: focal lymphocytic infiltration
also: hypergammaglobulinaemia, low C4

Question 4

Management of Sjorgens syndrome

Answer 4

Not really imminosupressive
tear substitute,
saliva substitute,
rarely pilocarpine

complications:
risk of increased lymphoma