Sjogren's syndrome.
Sjogren's syndrome is an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces. It may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders, where it usually develops around 10 years after the initial onset. Sjogren's syndrome is much more common in females (ratio 9:1). There is a marked increased risk of lymphoid malignancy (40-60 fold)
Features
dry eyes: keratoconjunctivitis sicca
dry mouth
vaginal dryness
arthralgia
Raynaud's, myalgia
sensory polyneuropathy
renal tubular acidosis (usually subclinical)
Investigation
rheumatoid factor (RF) positive in nearly 100% of patients
ANA positive in 70%
anti-Ro (SSA) antibodies in 70% of patients with PSS
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
Management
artificial saliva and tears
pilocarpine may stimulate saliva production.
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