Diferencia entre revisiones de «Scleroderma»
Sin resumen de edición |
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| Línea 8: | Línea 8: | ||
**Narrowed lumen | **Narrowed lumen | ||
== | == Clinical Features == | ||
*Systemic complaints (fever, malaise, fatigue, weight loss, myalgias) | *Systemic complaints (fever, malaise, fatigue, weight loss, myalgias) | ||
*Skin lesions (fingers, hands, face) | *Skin lesions (fingers, hands, face) | ||
Revisión del 14:11 29 jun 2015
Basics
- 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