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classifications of scleroderma

morphea
limited scleroderma
diffuse scleroderma

1

limited scleroderma affects

extremities + head

2

diffuse scleroderma affects

limited + trunk

3

early skin changes in scleroderma

swelling of fingers/hands-->erythema, pruritis

4

later skin changes in scleroderma

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

5

scleroderma sine sclerosis

dx later in life
raynaud, GI issues, autoabs, telangiectasias

6

limited scleroderma aka

CREST
calcinosis, raynaud's, esophageal dysmotility, sclerodactyly, telengectasia

7

calcinosis is

only seen in limited (not diffuse)

8

dilated nailfod capilaries important..

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

9

sclerodactyly

resorption of tufts of terminal phalanges

10

diffuse scelroderma involves (4)

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

11

limited scleroderma

CREST
calcinosis
pulmonary HTN
centromere

12

4 changes in pathophys of scleroderma

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

13

characteristics of diffuse scleroderma

vasculopathy
autoimmunity
fibrosis
pulmonary ILD
GI**
MSK
Skeletal Muscle

14

how does vasculopathy happen

not a product of inflammation
actually oblierate vessels via thickeninging of intima and dibrosis of adventitia

15

fibrosis mech

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

16

ILD in first two years

if nothing happens in first two years, patient is ok

17

underlying caues of GI issues

fibrosis
muscular atrophy
vessel thickening

18

issues in upper GI

eso hypomotility
incompettent LES
gastroparesis

19

issues in small bowel

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

20

issues in colon

constripation

21

issues in MSK

arthralgia, stiffness
synovitis
TENDON FRICTION RUBS
joint contractures

22

issues with skeletal muscle

bland myopathy in 20%

23

MC death in scleroderma

lung dz

24

tx scleroderma

treat by symptoms

25

primary vs secondary sjogren's

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

26

classification of sjogrens

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

27

infiltrating t cells of sjogrens

more CD4 then CD8

28

SSa

Ro

29

SSb

La

30

antibody that b cells produce in sjogrens

iga

31

when sjogrens is left untreated

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

32

three tests for sjogrens

rose bengal/lissamine stains
schirmer test
slit lamp exam

33

oral sx

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

patients with sjogrens have an increased risk of

lymphoma

35

tx sjogrens

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