A family of autoimmune, multi-system, connective tissue d/o
Can be limited to the skin alone (localized scleroderma); or have more diffuse symptoms and findings→ systemic sclerosis
Systemic sclerosis (SS) broken into limited cutaneous (lcSSc) or diffuse cutaneous forms (dcSSc)
SS can have overlap with lupus, rheumatoid arthritis, polymyositis, or Sjögren's
Risk Factors
Women > men
< 50
Known disease
Symptoms
Skin tightening
Sub-cu calcium
Telangiectasias
Raynauds
GERD due to atrophy of esophageal smooth muscle, leading to impaired peristalsis and decreased LES tone, leading to Barretts (increased risk distal esophageal cancer), scarring, dysphagia
If systemic→ can affect lung (DPLD), pericardium, joints, etc
Additional symptoms based on organ systems involved: SOB, chest pain, joint pain/swelling
Physical Exam Findings
Skin tightening
Raynauds → fingers blanch/hurt when exposed to cold temp