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cutaneous systemic sclerosis
Clinical manifestations:
- limited cutaneous systemic sclerosis with involvement restricted to face & extremities distal to the elbow
- tightness, thickening & swelling of digits extending proximal to the metacarpophalangeal joints
- sclerodactyly
- digital telangiectasias
- terminal digital pitting or ulceration
- Raynaud's phenomenon
- internal organ fibrosis generally not a feature
- patients may display features of CREST syndrome
- calinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias)
Laboratory:
- antinuclear antibody generally positive (see scleroderma)
- anti-centromere antibody (30%); 90% with CREST syndrome
Special laboratory:
- see scleroderma
Radiology:
- chest X-ray
- basilar interstitial pulmonary fibrosis
- barium swallow if dysphagia (see scleroderma)
Complications:
- pulmonary hypertension
- scleroderma renal crisis
- also see scleroderma
Management:
- see scleroderma
General
systemic sclerosis
References
- Medical Knowledge Self Assessment Program (MKSAP) 16,17
American College of Physicians, Philadelphia 2012,2015