Uncommon, specifically defined condition which features symptoms also seen in other connective tissue diseases
Clinical presentation
- Raynaud’s
- Arthralgia/arthritis
- Myositis
- Sclerodactyly
- Pulmonary hypertension
- ILD
Investigations
- Antibodies - associated with anti-RNP antibodies
Management
- Varies according to presentation
- CCBs may be helpful for Raynaud’s
- If there is significant muscle or lung disease immunosuppression may be required
- Monitoring - patients should be monitored for pulmonary hypertension (anual echo) and ILD (PFTs)