Scleroderma Systemic sclerosis

• Facts:
  Inflammation that leads to progressive tissue fibrosis through excessive deposition of type I and type III collagen
  Commonly manifests as CREST syndrome, but can also be diffuse
• History / PE:
  Symmetric thickening of skin of face, distal extremities
  
  CREST syndrome
  
  C alcinosis (calcium in soft tissue)
  R aynaud's phenomenon
  E sophageal dysmotility
  S clerodactyly
  T elangiectasias
  
  Diffuse sclerosis
  Pulmoary fibrosis, cor pulmonale , ARF, malignant HTN
  
  
• Diagnosis:
  Positive RF and ANA
  Anticentromere antibodies (CREST)
  Anti-Scl-70 / anti-topoisomerase-1 antibodies (diffuse)
• Treatment:
  Corticosteroids (acute flares)
  Penicillamine (skin changes)
  Calcium channel blockers (Raynaud's)
  ACEIs (renal disease)
• Complications:
  Pulmonary HTN
• Differential Diagnosis:
  Mixed connective tissue disease (anti nRNP)
  Sjogren's syndrome