Scleroderma Flashcards
20-30% of scleroderma patients have these
Anti endothelial cell cytotoxic factors
Potent vasoconstrictors that are an important factor in scleroderma
Endothelins
NO
EDCF (endothelium derived constricting factors)
Microvascular change in Sclerodema
intimal sclerosis, fibrinoid change and necrosis
causes vessel diameter decrease, local ischemia and FIBROSIS
Skin thickness change in SD
Reduced skin thickness, Increased collagen bundles and ECM constituents
Stereotypical SD patient
- Female
- 30-40
- Pale/cyanotic
- Raynauds
- puffy face in morning
- telangiectasias
- Esoph. dysmotility & Pulmonary fxn issues
Skin stages in SD
- Edema
- Stiff/adhered
- atrophic
- soft/pliable
___ in 90% of PSS patients
Esophageal dysmotility
Other GI stuff with PSS
Colon sacculations
Primary biliary cirrhosis
Pulmonary Sx with PSS
Interstsitial fibrosis
Ace inhibitors in PSS have helped reduce the severity of __________
sclerodermal renal crisis
(sudden glomerular collapse)
Difference between PSS nephritis and lupus nephritis
PSS nephritis is not inflammatory
Heart Involvement in PSS
pericardial effusion and COR PULMONALE
Linear scleroderma =
Morphea =
- en coup de sabre
- band of sclerotic induration and hyperpig.
Morphea = Patch or patches of sclerodermatosis skin change
CREST is associated with _______
anti-centromere pattern ANA (on HEP2 substrate)
Scleroderma is 50% associated with _______
Diabetes
