Scleroderma: Difference between revisions
(Created page with "== Basics == *Autoimmune disease, aka '''Systemic Sclerosis''' *Inappropriate and excessive accumulation of collagen and matrix in various tissues *Widespread vascular lesion...") |
No edit summary |
||
| Line 38: | Line 38: | ||
Tintinalli | Tintinalli | ||
[[Category: | [[Category:Rheum]] | ||
Revision as of 01:37, 22 November 2014
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
Presentation
- 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