Systemic Sclerosis Flashcards
1
Q
What is systemic sclerosis
-what are the consequences
A
Excessive rapid production and accumulation of collagen that involves internal organs
Proximal cutaneous involvement
Early renal, GI, ILD
Pulmonary HTN
2
Q
What are the skin symptoms
A
Hardening and scarring => tight red, scaly skin
Nailfold capillaries
Digital ulcers
3
Q
What are the lung symptoms
A
Pulmonary fibrosis (honeycombing) Pulmonary HTN => dilated pulmonary vessels
4
Q
What are the Gi symptoms
A
Esophageal stricturing => relfux esophagitis
Decreased motility
5
Q
What are the renal symptoms
A
Poor prognostic factor, cause of death
- increased collagen in renal vessels => narrowing, leads to decreased blood flow
- hyperreninnemia (to increase blood flow)
- malignant HTN => renal damage
- kidney failure with accumulation of waste products in blood
- microangiopathic hemolytic anemia due to RBC trauma
6
Q
Describe limited cutaneous systemic sclerosis
A
CREST syndrome
- calcinosis
- Raynaud’s
- esophageal involvement
- sclerodactyly, distal involvement
- telangiectasia
7
Q
How would you treat systemic sclerosis
A
Early treatment => low does steroids (high dose may trigger renal crisis), immunosuppresion
Late treatment => symptomatic tretament, BP control
