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Systemic Sclerosis (Examination (Face (Beaked nose: “nasal skin tethering”…

- - - - Tight skin
      - Can you move skin between your fingers?
- - - - Mortality: respiratory failure and renal impairment
  - - - GN: proteinuria and haematuria
    - - Arrhythmias
      - RV strain
    - - FBC: anaemia
      - U+E: renal impairment
      - Abs
        
        ANA: +ve in 90%
        
        Centromere: limited (80%)
        
        Scl70 (topoisomerase): diffuse (70%)
    - - Hand radiographs: calcinosis
      - CXR: fibrosis
      - HRCT: fibrosis
      - Echo: pulmonary HTN
    - - Lung function tests: restrictive pattern
      - Ba swallow: dysmotility
  - - - Immunosuppression used for organ involvement or progressive skin disease.
    - - Gloves and hand-warmers
        
        CCBs: e.g. nifedipine
        
        Severe: prostacyclin infusion
    - - Aggressive blood pressure control
        
        ACEi
    - - PPI for reflux
    - - Sildenafil
      - Bosentan: dual endothelin receptor antagonist
- - - - Widespread cutaneous and early visceral involvement
        
        Rapid progression: months
    - - Distribution limited to below elbows and knees and face
        
        Slow progression: yrs
        
        Pulmonary HTN in ~15%
        
        CREST
        
        Calcinosis
        
        Raynaud’s phenomenon
        
        Esophageal dysmotility
        
        Sclerodactlyly
        
        Telangiectasia