Lecture 102 -Scleroderma Flashcards
what is scleroderma
who is most susceptible, is there genetic association?
describe in general the pathogensis
Auto immune disease characterized by sclerosis of the skin and visceral organs
commonly in middle aged females (AA > Whites)
HLADR5, DR3, and DR2
Pathogenesis:
Auto immune damage to mesenchyme
Endothelial Dysfunction + Vasconstriction + PDGF and TGF Beta
Activation of Fibroblasts
Leading to Fibrosis –> Ischemia —> End organ damage
what are the two types of scleroderma; is terms of skin changes what is the rough differentaition
diffuse and limited
diffuse – skin thickening on the truck, face, proximal and distal extremities
limited - face neck and extremities DISTAL to elbows and knee
Mneumonic of manifestation of limited scleroderma
what other manifestation is more common in limited than diffuse
what is the assocciated antibodiy
CREST Syndrome C --Calcinosis, anti-Centromere R -Raynauds E - Esophageal dysmotility S - sclerodactyly -- tightening of the skin on the fingers T - Telangiectasia
Also: PAH, pulm fibrosis
Antibody – Anti-centromere
Diffuse disease manifestations –
associatd antibody
Anti-SCl70
SKin Thickening of trunk, face, hands
Constitutional symptoms: Renal Crisis Heart Involvoemnt Gut Involvement MSK involvement Lung involvment
what is a common early presentation of scleroderma
what subtle finding may the rheumatologist look at to identify vascular damage?
puffy, painful hands
Nail bed fold capillary loops; (dilated early in the course of disease, and drop out of capillaries later inthe course of disease)
what are some severe vascular manifestations
Digital ulcers
Gangrene
autonecrosis
what is the most common cause of death in sclerodermal patients
what are some other negative prognostic factors?
lung disease – interstial fibrosis, pulm HTN
High skin score
low DLCO –
evidence of renal and lung invovlemet
ESR (sed rate) - inflammatory marker
patient presents with what you think may be scleroderma – how should you proceed
§ Look aggressively for internal organ involvement
§ Organ directed therapy can reduce morbidity and Mortality
renal crisis is more likely to occur in _____ slcero
how do you treat it
diffuse scleroderma
Tx aggressively with ACE I
management of skin manifestations
sometimes they resolve with time
can use immosupprssion
UVA light therapise
Treatmnet of raynauds
CCB ARBs Nitrates Endothelin recpetor antagonists Sildenafil
what type of Gut manifestation can arise in scleroderma
how can they be managed
esophageal dysmotility – prokinetics
GERD – H2 blockres, PPIs, treat dysmotility
Malabsorption – diet, prokinetics, abx, TPN
GAVE – (watermelon stomach) – gastric antral vascular ectasia – catuerize
best treatment for insterstitial lung disaesae
Cyclophosphamide if there is active inflammation
treatment of PAH
◊ Epoprostenol – prostcyclin analogue
◊ Treprostinil – Prostacyclin analogue
◊ Bosentan – Endothelin receptor antagonist
◊ PDE - Sildenfil
