14- SCLERODERMA

• Scleroderma


  A chronic connective tissue disorder in which cytokines stimulate fibroblasts à abnormally high amount of normal collagen deposition à widespread fibrosis
• Scleroderma


  • More common in young women
  • Two types: Diffuse (20%) and limited (80%)
• Diffuse scleroderma


  • Widespread skin involvement (trunk, all extremities)
  • Rapid onset of symptoms
  • Worse prognosis
  • Anti-topoisomerase I Ab (anti-SCL70) more common
  • Significant visceral involvements — lung, heart, GI and kidney
  • Pulmonary fibrosis (may then à pulmonary hypertension)
• Limited scleroderma


  • Skin involvement limited to distal extremities — sparing the trunk, only distal to elbows & knees
  • Delayed onset
  • Better prognosis
  • Anticentromere Ab more common
  • Visceral involvements occur late
  • Pulmonary hypertension (with normal lungs)
• CREST


  • Calcinosis of the digits
  • Raynaud phenomenon
  • Esophageal immobility
  • Sclerodactyly
  • Telangiectasias
• Raynaud phenomenon


  • In almost all patients, due to vasospasm and thickening of vessel walls, may lead to digital ischemia with ulceration and gangrene
• Cutaneous fibrosis


  • Tightening of the face and extremities
• Sclerodactyly


  • Claw-like hand à contractures, disability, disfigurement
• Dysphagia/reflux


  • From esophageal immobility (may à strictures)
• Gastrointestinal


  • Grave stomach (vascular phenomenon)
  • Delayed gastric emptying, abdominal distention and pseudo-obstruction
  • Large diverticula of small & large bowel à bacterial overgrowth à diarrhea
• Pulmonary


  • Pulmonary fibrosis and/or pulmonary HTN, most common cause of death
• Cardiac


  • RVH, pericardial effusions, myocardial fibrosis, pericarditis à CHF, arrhythmias (heart block)
• Renal crisis


  • Rapid malignant HTN
• Positive ANA


  Sensitive but not specific
• Autoantibodies


  • Anticentromere Ab
  • Anti-topoisomerase I (anti-scleroderma 70) Ab
• Diagnostic tests


  1. Barium swallow

  2. Pulmonary function test
• Purpose of diagnostic tests

  Detect complications
• Symptomatic treatments


  • NSAIDs for musculoskeletal pain
  • H2 blockers or PPIs for esophageal reflux
  • Avoiding cold & smoking
  • Use of CCB for Raynaud
• ACE inhibitors


  Used to prevent and treat renal hypertensive crisis
• Methotrexate


  Slows underlying disease process of limited scleroderma (CREST)
• Cyclophosphamide


  Improves dyspnea & PFTs in pulmonary fibrosis
• Treatments for pulmonary hypertension


  • Bosentan
  • Ambrisentan
  • Sildenafil
  • Prostacyclin analogs