Systemic Sclerosis and Sjogren's Flashcards
limited scleroderma affects
extremities + head
classifications of scleroderma
morphea
limited scleroderma
diffuse scleroderma
diffuse scleroderma affects
limited + trunk
early skin changes in scleroderma
swelling of fingers/hands–>erythema, pruritis
later skin changes in scleroderma
shiny, tight, thick pigment changes (hypo and hyperpigmented areas), sclerodactyly, joint contractures, digital ulcers, pitting of fingertips
scleroderma sine sclerosis
dx later in life
raynaud, GI issues, autoabs, telangiectasias
limited scleroderma aka
CREST
calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telengectasia
calcinosis is
only seen in limited (not diffuse)
dilated nailfod capilaries important..
to tell primary raynaud’s from secondary which helps predict whether the patient will develop CT abnormalities or not
sclerodactyly
resorption of tufts of terminal phalanges
diffuse scelroderma involves (4)
early organ involvment
renal crisis
pulmonary fibrosis
topo 1 (scl-70)
limited scleroderma
CREST
calcinosis
pulmonary HTN
centromere
4 changes in pathophys of scleroderma
fibroblasts–>tissue fibrosis
endothelial cells–>vasculopathy
b cells–>produce autoabs
t cells–>cytokine produx
characteristics of diffuse scleroderma
vasculopathy autoimmunity fibrosis pulmonary ILD GI** MSK Skeletal Muscle
how does vasculopathy happen
not a product of inflammation
actually oblierate vessels via thickeninging of intima and dibrosis of adventitia
fibrosis mech
profibriotic cytokines–>fibroblasts–>deposit type 1 and 3 collagen–> typical skin changes, pulm fibrosis, and GI manifestations
ILD in first two years
if nothing happens in first two years, patient is ok
underlying caues of GI issues
fibrosis
muscular atrophy
vessel thickening
issues in upper GI
eso hypomotility
incompettent LES
gastroparesis
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
issues in colon
constripation
issues in MSK
arthralgia, stiffness
synovitis
TENDON FRICTION RUBS
joint contractures
issues with skeletal muscle
bland myopathy in 20%
MC death in scleroderma
lung dz
tx scleroderma
treat by symptoms
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)
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
infiltrating t cells of sjogrens
more CD4 then CD8
SSa
Ro
SSb
La
antibody that b cells produce in sjogrens
iga
when sjogrens is left untreated
patients develop filamentary keratitis (sloughign of corneal epithelium) and corneal scarring+ infection
three tests for sjogrens
rose bengal/lissamine stains
schirmer test
slit lamp exam
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
patients with sjogrens have an increased risk of
lymphoma
tx sjogrens
moisture replacement
stimulation of endogenous secretion–>+ muscarinic (pilocarpine, cevimeline)
immunosuppression
oral disease
