Introduction
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).
Epidemiology
- Incidence: 5.00 cases per 100,000 person-years
- Peak incidence: 30-40 years
- Sex ratio: more common in females 10:1
| Condition | Relative incidence |
|---|---|
| Salivary gland stones | 1.20 |
| Sjogren's syndrome | 1 |
| Systemic lupus erythematosus | 1.00 |
| <1 | 1-5 | 6+ | 16+ | 30+ | 40+ | 50+ | 60+ | 70+ | 80+ |
Clinical features
Features
- dry eyes: keratoconjunctivitis sicca
- dry mouth
- vaginal dryness
- arthralgia
- Raynaud's, myalgia
- sensory polyneuropathy
- recurrent episodes of parotitis
- renal tubular acidosis (usually subclinical)
Investigations
Investigation
- rheumatoid factor (RF) positive in around 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
Management
Management
- artificial saliva and tears
- pilocarpine may stimulate saliva production
Complications
There is a marked increased risk of lymphoid malignancy (40-60 fold).