Systemic sclerosis: Difference between revisions
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== |
== Clinical Manifestations == |
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=== Syndromes === |
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* Scleroderma (only skin involvement) |
* [[Scleroderma]] (only skin involvement) |
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* Limited cutaneous systemic sclerosis (lcSSc) |
* Limited cutaneous systemic sclerosis (lcSSc) |
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** CREST syndrome |
** [[CREST syndrome]] |
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* Diffuse cutaneous systemic sclerosis (dcSSc) |
* Diffuse cutaneous systemic sclerosis (dcSSc) |
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* SSc sine scleroderma (only internal organ involvement) |
* SSc sine scleroderma (only internal organ involvement) |
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* Overlap syndromes |
* Overlap syndromes |
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=== Limited |
=== Limited Cutaneous Systemic Sclerosis (lcSSC) === |
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* Sclerosis often restricted to hands, face, and neck |
* Sclerosis often restricted to hands, face, and neck |
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** Telangiectasia |
** Telangiectasia |
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=== Diffuse |
=== Diffuse Cutaneous Systemic Sclerosis (dcSSc) === |
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* Extensive skin sclerosis, including proximal to wrists, including proximal limbs and trunk |
* Extensive skin sclerosis, including proximal to wrists, including proximal limbs and trunk |
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== Management == |
== Management == |
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=== Raynaud |
=== Raynaud Phenomenon === |
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* Non-pharmacologic: smoking cessation, cold avoidance |
* Non-pharmacologic: smoking cessation, cold avoidance |
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Latest revision as of 17:14, 3 March 2026
Clinical Manifestations
Syndromes
- Scleroderma (only skin involvement)
- Limited cutaneous systemic sclerosis (lcSSc)
- 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.
