Systemic Sclerosis Flashcards Preview

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Flashcards in Systemic Sclerosis Deck (14)
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1
Q

Epidemiology

A

women > men and AA > whites

2
Q

Susceptibility genes

A

HLA, PTN22, IRF5, STAT4, BANK1, TNFSF4. T-bet

3
Q

Environmental predisposition

A

silica, heavy metals, mercury, organic chemicals,

drugs: bleomycin, cocaine, taxol

4
Q

SSc manifests as __ + ___ => _____

A

immune dysregualtion + microangiopathy → systemic fibrosis

5
Q

Pathogenesis of immune dysregualtion

A
  • Inciting event (drug, infection, oxidative damage, etc.)
  • Activation of TLR (by autoAg) on dendritic cell which produce INF-gamma
  • INF-gamma activate Th2 which activate B cells
  • B cells make AutoAbs against the AutoAg
  • Immune complexes form → more INF-gamma released along with profibrotic cytokines (TGF-beta)

**INF-gamma!!!!!!!

6
Q

Pathogenesis of vasculopathy

A

insult –> endothelial dysfunction –>
•Inc apoptosis and ICAM-expression on endothelial cells
•Dec NO, PGI
•Inc endothelin-1 and thrombin
•ROS generation
–> vasoconstrition and ischemia
+ luminal narrowing/remodeling de to shear stress, platelet aggregation, and thrombosis
–> T cells come in a release cytokine that elicit inflammation and recruit fibroblasts
–> fibrosis and vascular obliteration

7
Q

Clinical features of SSc

A

CREST
AutoAb (Scl70, U3-RNP, RNA pol III)
widening capillary loops

crest = Calcinosis, Raynauds, Esophageal dysmotitiy, Sclerodacyly, Telangiectasias

8
Q

Abs assc with limited sclerosis
diffuse sclerosis
both

A
limited = centromere, Th/To,  cardiolipin, B2-GP1
diffuse = Scl70 (topo1), RNA pol III
both = U3-RNP and B23
9
Q

How is early limited scleroderma diff from early diffuse scleroderma

A

Early Limited / Early Diffuse

Raynauds is 1st symptom / Raynaud’s is delayed

Rarely have general symptoms / Acute onset of many generalized symptoms

Minimal arthralgias / Arthralgias

Puffy fingers / Puffy hands

Limited skin thickening / More skin thickening

Anti-centromere Ab/ Anti-Scl 70 and Anti-RNA pol III Abs

10
Q
histo findings in..
vasculature
skin
lung
renal
A

vasculature: onion skin
skin: loss of hair follicles and sebaceuos glands
lung: ILD/fibrosis (NSIP pattern)
renal: fibinoid necrosis and thrombosis

11
Q

Describe effect of SSc on lungs

A

PSIP pattern
PAH
restrictive PFTs and dec DLCO
ground glass on CT

12
Q

Acute, sever HTN →HF or seizures
Grade II retinopathy
Presence of anti-RNA polymerase I and III antibody

Treated with ACEi

A

sclerodermal renal crisis”

13
Q

kid with isolated circular patch of thickened skin

A

plaque morphea

14
Q

multiple lesions involving extensive areas of skin and can occasionally coalesce, mimicking skin changes of systemic sclerosis

A

generalized morphea