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Scleroderma
Joaquin
masoud
C.
masoud,
DMD,MScD
Part
1:
introduc:on,
clinical
feature,
pathogenesis
Deni&on
1. Systemic
sclerosis
(scleroderma)
-
a
mul:system
disorder
characterized
by
1)
func:onal
and
structural
abnormali:es
of
blood
vessels
2)
brosis
of
the
skin
and
internal
organs
3)
immune
system
ac:va:on
4)
autoimmunity
2.
Localized
scleroderma
-
morphea,
linear
scleroderma
Epidemiology
It
is
Rare
in
children
Its
Peak
is
between
age
35-65
More
women
aected
then
men
Family
history
of
other
auto
immune
diseases
Ethnic
background
inuence
survival
and
disease
manifesta:on.
(
in
iran,
the
prevalence
is
more
between
caspian
people)
2. Limited cutaneous systemic sclerosis 1) symmetric restricted fibrosis - affecting the distal extremities and face/neck 2) prolonged delay in appearance of distinctive internal manifestation 3) prominence of calcinosis and telangiectasia 4) good prognosis * CREST syndrome - calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia
Clinical
features
Raynaud s Typical skin changes Esophageal and small bowel dysfunction Interstitial lung disease Pulmonary hypertension Renal crises
Causes
of
Raynaud s
Occlusive
arterial
disease
Rheuma:c
diseases:Scleroderma,CREST,MCTD,SLE,RA,
Myosi:s
Repe::ve
vascular
injury
Hyperviscosity
:Polycythemia,Cryoglobulinemi a
Thoracic
outlet
syndrome
Clinical
features
1. Vascular
abnormali:es
1)
Raynaud's
phenomenon
-
cold
hands
and
feet
with
reversible
skin
color
change
(white
to
blue
to
red)
-
induced
by
cold
temperature
or
emo:onal
stress
-
ini:al
complaint
in
3/4
of
pa:ents
-
90%
in
pa:ents
with
skin
change
(prevalence
in
the
general
popula:on:
4-15%)
2)
digital
ischemic
injury
Clinical
feature
2.skin involvement: 1) -edematous phase -indurative phase -atrophic phase 2) firm,thickened bound to underlying soft tissue 3) Decrease in range of motion , loss of facial expression, inability to open mouth fully
microstomia
Cold sensi:vity
Edematous phase
Skin Indura:on
Acrosclerosis
Skin microscopy
Acrolysis
Telangiectasia
Face
/
mucous
membrane
blanched
by
pressure
Clinical features
4. intes:nal
involvement
1)
esophagus:
hypomo:lity
and
retrosternal
pain,
reux
esophagi:s,
stricture
Abnormal mo:lity
Diver:cula
Barium
enema
study
-
mul:ple
wide-mouthed
diver:cula
of
colon
-
broad
base
and
neck
-
usually
asymptoma:c
Diver:cula
5. lungs 1) 2/3 of patients affected - leading cause of mortality and morbidity in later stage of systemic sclerosis 2) pathology - interstitial fibrosis - intimal thickening of pulmonary arterioles (pulmonary hypertension)
Pulmonary brosis
7.kidney 1) diffuse scleroderma in association with rapid progression of skin involvement 2) pathology - intimal hyperplasia of the interlobular artery - fibrinoid necrosis of afferent arterioles - glomerulosclerosis 3) proteinuria, abnormal sediment, azotemia, microangiopathic hemolytic anemia, renal failure
Kidney arteriogram
Pathogenesis
1. Vasculopathy of small artery and capillary - endothelial cell injury - adhesion and activation of platelet - PDGF, thromboxane A2 release - vasoconstriction & growth of endothelial cell and fibroblast - narrowing or obliteration, increased permeability 2. Fibrosis - aberrant regulation of fibroblast cell growth - increased production of extracellular matrix (collagen, fibronectin, and glycosaminoglycan) - thickening of the skin & fibrosis of internal organs
Pathogenesis
Pathogenesis
3. Immunologic mechanism 1) cell mediated immunity - skin: cellular infiltrates in perivascular region and dermis (T cell, Langerhans cell, plasma cell, macrophage) 2) humoral immunity - hypergammaglobulinemia - autoantibody production antinuclear antibody (+) > 95%
Pathogenesis
4. Environmental factors 1) silica dust 2) organic solvents 3) biogenic amines 4) urea formaldehyde 5) polyvinyl chloride 6) rapeseed oil 7) bleomycin 8) L-tryptophan 9) silicone implant
Thank you