Scleroderma

Background

• Autoimmune disease, aka Systemic Sclerosis
• Inappropriate and excessive accumulation of collagen and matrix in various tissues
• Widespread vascular lesions
  • Endothelial dysfunction
  • Vascular spasm
  • Vascular wall thickening
  • Narrowed lumen

Clinical Features

• Systemic complaints (fever, malaise, fatigue, weight loss, myalgias)
• Skin lesions (fingers, hands, face)
• Carpal tunnel
• Raynaud's
• Interstitial lung disease
• Renal impairment
• GI dysmotility
• GERD/aspiration
• Chronic esophagitis and stricture formation

Emergencies

• Scleroderma renal crisis
  • Causes HTN emergency
  • Treatment is strict BP control - ACEi drug of choice
    • Was the most common cause of death prior to ACEi usage
• Pulmonary
  • Respiratory failure
  • ARDS
  • Aspiration pneumonitis
  • Pulmonary HTN
  • Alveolar hemorrhage
    • Hemoptysis, infiltrates on CXR, anemia.
    • Emergent bronchoscopy
    • High dose glucocorticoids, cyclophosphamide, local vessel embolization or plasma exchange
• Cardiac
  • Acute heart failure
    • Can be from diastolic dysfunction, malignant HTN during renal crisis, and decompensated pulmonary HTN
  • Cardiopulmonary complications are the most common causes of scleroderma-related death, with cardiac causes and pulmonary fibrosis being the most common.

References

• Tintinalli