Systemic Sclerosis and Sjogren's

Question 0

limited scleroderma affects

Answer 0

extremities + head

Question 1

classifications of scleroderma

Answer 1

morphea
limited scleroderma
diffuse scleroderma

Question 2

diffuse scleroderma affects

Answer 2

limited + trunk

Question 3

early skin changes in scleroderma

Answer 3

swelling of fingers/hands–>erythema, pruritis

Question 4

later skin changes in scleroderma

Answer 4

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

Question 5

scleroderma sine sclerosis

Answer 5

dx later in life

raynaud, GI issues, autoabs, telangiectasias

Question 6

limited scleroderma aka

Answer 6

CREST

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

Question 7

calcinosis is

Answer 7

only seen in limited (not diffuse)

Question 8

dilated nailfod capilaries important..

Answer 8

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

Question 9

sclerodactyly

Answer 9

resorption of tufts of terminal phalanges

Question 10

diffuse scelroderma involves (4)

Answer 10

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

Question 11

limited scleroderma

Answer 11

CREST
calcinosis
pulmonary HTN
centromere

Question 12

4 changes in pathophys of scleroderma

Answer 12

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

Question 13

characteristics of diffuse scleroderma

Answer 13

vasculopathy
autoimmunity
fibrosis
pulmonary ILD
GI**
MSK
Skeletal Muscle

Question 14

how does vasculopathy happen

Answer 14

not a product of inflammation

actually oblierate vessels via thickeninging of intima and dibrosis of adventitia

Question 15

fibrosis mech

Answer 15

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

Question 16

ILD in first two years

Answer 16

if nothing happens in first two years, patient is ok

Question 17

underlying caues of GI issues

Answer 17

fibrosis
muscular atrophy
vessel thickening

Question 18

issues in upper GI

Answer 18

eso hypomotility
incompettent LES
gastroparesis

Question 19

issues in small bowel

Answer 19

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

Question 20

issues in colon

Answer 20

constripation

Question 21

issues in MSK

Answer 21

arthralgia, stiffness
synovitis
TENDON FRICTION RUBS
joint contractures

Question 22

issues with skeletal muscle

Answer 22

bland myopathy in 20%

Question 23

MC death in scleroderma

Answer 23

lung dz

Question 24

tx scleroderma

Answer 24

treat by symptoms

Question 25

primary vs secondary sjogren's

Answer 25

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

Question 26

classification of sjogrens

Answer 26

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

Question 27

infiltrating t cells of sjogrens

Answer 27

more CD4 then CD8

Question 28

SSa

Answer 28

Ro

Question 29

SSb

Answer 29

La

Question 30

antibody that b cells produce in sjogrens

Answer 30

iga

Question 31

when sjogrens is left untreated

Answer 31

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

Question 32

three tests for sjogrens

Answer 32

rose bengal/lissamine stains schirmer test slit lamp exam

Question 33

oral sx

Answer 33

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

Question 34

patients with sjogrens have an increased risk of

Answer 34

lymphoma

Question 35

tx sjogrens

Answer 35

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