Systemic sclerosis Flashcards
What are the 3 types of sclerosis of the skin that exist?
Isolated Raynaud’s
Localised scleroderma
Systemic sclerosis
What is Raynaud’s?
Innapropriate vasospasm in response to cold
In which conditions is Raynaud’s a symptom?
Rheumatoid arthritis
Systemic sclerosis
MCTD
Systemic Lupus Erythematosus
How can you differentiate between idiosynchratic Raynaud’s and Systemic sclerosis?
Auto-antibody testing
Microscopy imaging of the capillaries in the nail beds
Why is SSc such a complicated disease?
Multi-systemic disease
What are the different forms of SSc?
Limited
Diffused
Overlap
What trait determines survivability of a SSc patient?
Involvement of internal organs
What are complications of SSc present in all patients?
Raynaud’s
GI involvement
What are complications of SSc present in diffuse stages of the condition?
Lung fibrosis
Pulmonary hypertension
Renal and heart involvement
How does SSc affect the GI tract?
Causes fibrosis of the gut wall
What is a symptom of the GI tract that indicates Raynaud’s is a symptom of SSc?
Acid reflux
How does SSc affect esophageal mobility?
No normal peristaltic waves causes the acid of the stomach to form acid reflux
Damage of the esophagus and acid aspiration
How can we measure esophageal mobility?
Manometry
What are the consequences of esophageal immobility?
Acid aspiration
Acid reflux
What are the two vascular pathologies triggered in SSc?
Pulmonary hypertension
Scleroderma renal crisis
How can we treat scleroderma renal crisis?
ACE inhibitors
50% present with chronic kidney disease and need dialysis
How can we treat pulmonary hypertension?
Endothelin antagonists
What are current therapies for SSc?
Esophageal reflux disease - reduce volume of stomach acid
Intestinal bacterial overgrowth - antibiotics
Lung fibrosis - immunosuppression
What are complications of SSc which have no treatment?
Digital ulcers GI disease - malnutrition Severe pain Sexual dysfunction Physiological and emotional impact
