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
• recurrent episodes of parotitis
• renal tubular acidosis (usually subclinical)

Investigation

• rheumatoid factor (RF) positive in nearly 50% 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