scleroderma Flashcards
definition
fibrosis/hardening of the skin
types
localized or generalized
localized
morphed, linear (coup de sabre), no systemic involvement, not big deal
generalized other names
cutaneous scleroderma, systemic sclerosis
path
fibrous tissue, inflammation/ autoimmunity» vascular damage
epi
RARE, but runs in rheum families, high ANA, D, chock tow Indians, twins, environ
ABs
ANA 1:160 SEN, NPV, anticentromere SPEC, PPV, anti scl70 SPEC, PPV
skin clinical
dermis fibrosis. collagen» reticular dermis, T cells infiltrate, surround blood vessels. peripheral» proximal
CREST
old, non applicable nemonic. does NOT equal limited
vascular clinical
ALL pts have raunauds
raynauds
digital intimal proliferation- collagen hyperplasia, no arterial lumen, BAD
pulm HTN
intimal fibrosis, late and limited
lung clinical
interstitial, ground glass» honeycomb
renal crisis triad
acute renal failure, abrupt HTN, micro angio apthic hemolytic anemia
renal crisis treatment
ACE inhibitor- deal with high renin