Systemic Sclerosis and Sjogren's Flashcards

0
Q

limited scleroderma affects

A

extremities + head

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1
Q

classifications of scleroderma

A

morphea
limited scleroderma
diffuse scleroderma

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2
Q

diffuse scleroderma affects

A

limited + trunk

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3
Q

early skin changes in scleroderma

A

swelling of fingers/hands–>erythema, pruritis

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4
Q

later skin changes in scleroderma

A
shiny, tight, thick
pigment changes (hypo and hyperpigmented areas), sclerodactyly, joint contractures, digital ulcers, pitting of fingertips
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5
Q

scleroderma sine sclerosis

A

dx later in life

raynaud, GI issues, autoabs, telangiectasias

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6
Q

limited scleroderma aka

A

CREST

calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telengectasia

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7
Q

calcinosis is

A

only seen in limited (not diffuse)

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8
Q

dilated nailfod capilaries important..

A

to tell primary raynaud’s from secondary which helps predict whether the patient will develop CT abnormalities or not

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9
Q

sclerodactyly

A

resorption of tufts of terminal phalanges

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10
Q

diffuse scelroderma involves (4)

A

early organ involvment
renal crisis
pulmonary fibrosis
topo 1 (scl-70)

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11
Q

limited scleroderma

A

CREST
calcinosis
pulmonary HTN
centromere

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12
Q

4 changes in pathophys of scleroderma

A

fibroblasts–>tissue fibrosis
endothelial cells–>vasculopathy
b cells–>produce autoabs
t cells–>cytokine produx

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13
Q

characteristics of diffuse scleroderma

A
vasculopathy
autoimmunity
fibrosis
pulmonary ILD
GI**
MSK
Skeletal Muscle
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14
Q

how does vasculopathy happen

A

not a product of inflammation

actually oblierate vessels via thickeninging of intima and dibrosis of adventitia

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15
Q

fibrosis mech

A

profibriotic cytokines–>fibroblasts–>deposit type 1 and 3 collagen–> typical skin changes, pulm fibrosis, and GI manifestations

16
Q

ILD in first two years

A

if nothing happens in first two years, patient is ok

17
Q

underlying caues of GI issues

A

fibrosis
muscular atrophy
vessel thickening

18
Q

issues in upper GI

A

eso hypomotility
incompettent LES
gastroparesis

19
Q

issues in small bowel

A

hypomotility–>bacterial overgrown, pseudoobstruction, malabsorption
diverticula
Gastric antral vascular ectasia- dilated and thrombic in antrum, bleeding into intestine–>chronic GI bleeding; watermelon stomach

20
Q

issues in colon

A

constripation

21
Q

issues in MSK

A

arthralgia, stiffness
synovitis
TENDON FRICTION RUBS
joint contractures

22
Q

issues with skeletal muscle

A

bland myopathy in 20%

23
Q

MC death in scleroderma

A

lung dz

24
Q

tx scleroderma

A

treat by symptoms

25
Q

primary vs secondary sjogren’s

A

primary- dry eyes and mouth secondary to autoimmune dysfunction
secondary- dry eyes and mouth in presence of other autoimmune CTD disorders (like RA)

26
Q

classification of sjogrens

A

need 2/3
- SS-A or SS-B and + RF w/ ANA
salivary gland bx with infilitration
objective signs of dry eyes

27
Q

infiltrating t cells of sjogrens

A

more CD4 then CD8

28
Q

SSa

A

Ro

29
Q

SSb

A

La

30
Q

antibody that b cells produce in sjogrens

A

iga

31
Q

when sjogrens is left untreated

A

patients develop filamentary keratitis (sloughign of corneal epithelium) and corneal scarring+ infection

32
Q

three tests for sjogrens

A

rose bengal/lissamine stains
schirmer test
slit lamp exam

33
Q

oral sx

A

water bottle sign
loss of salvia, cracked lips and tongue, rampant dnetal caries and dental loss
oral candidasis
parotid/subM enlargement with increased risk for calculi and parotitis abscessformation

34
Q

patients with sjogrens have an increased risk of

A

lymphoma

35
Q

tx sjogrens

A

moisture replacement
stimulation of endogenous secretion–>+ muscarinic (pilocarpine, cevimeline)
immunosuppression
oral disease