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
  • Most common cause of death
  • Causes HTN emergency
  • Treatment is strict BP control - ACEi drug of choice
• 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
• Acute heart failure
  • Can be from diastolic dysfunction, malignant HTN during renal crisis, and decompensated pulmonary HTN

Source

Tintinalli