Systemic sclerosis
From IDWiki
Syndromes
- Scleroderma (only skin involvement)
- Limited cutaneous systemic sclerosis (lcSSc)
- CREST syndrome
- Diffuse cutaneous systemic sclerosis (dcSSc)
- SSc sine scleroderma (only internal organ involvement)
- Environmentally-induced scleroderma (vinyl chloride, epoxies, pesticides)
- Overlap syndromes
Limited cutaneous systemic sclerosis (lcSSC)
- Sclerosis often restricted to hands, face, and neck
- May have the CREST syndrome
- Calcinosis cutis
- Raynaud phenomenon
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
Diffuse cutaneous systemic sclerosis (dcSSc)
- Extensive skin sclerosis, including proximal to wrists, including proximal limbs and trunk
- Greater risk for internal organ involvement, including kidneys, lungs, and heart
Management
Raynaud phenomenon
- Non-pharmacologic: smoking cessation, cold avoidance
- First-line: nifedipine or other DHP calcium channel blocker
- Second-line: PDE-5 inhibitors, such as sildenafil, tadalafil, or vardenafil
- Non-guideline second-line: topical nitroglycerine
- Can consider fluoxetine
- Last-line: IV iloprost or other prostanoid
Further Reading
- Kowal-Bielecka O, Fransen J, Avouac J EUSTAR Coauthors, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Annals Rheumatic Diseases 2017;76:1327-1339.
