Lec 10 Scleroderma Flashcards
About 20% of patients with scleroderma have what antibody?
Anti endothelial cell cytotoxic factors
There is evidence scleroderma may be due to ____ that causes a graft vs host type disease
microchimerism
vaso_____ are important in the pathogenesis of scleroderma
constrictors
The ____ ____ may be a primary target tissue as evidenced by intimal sclerosis, fibrinoid change, and necrosis.
vascular endothelium
The is atrophy of skin ____ and loss of ____ ____ and ____ ____
appendages; hair follicles, sweat glands
Thinning of the ____ is also seen, with accumulation of excess ____ bundles and loss of ____ ____
epidermis; collagen; blood vessels
Changes in skin include puffy ____ and ____
face, hands
Skin changes over time:
____–>stiff with altered _____ –>atrophic
edematous; pigmentation
A characteristic skin finding is loss of _____
wrinkles
Raynouds phenomenon is often seen. In severe cases, these leads to ____ of the fingers and digital _____
infarction; pitting
____ dysmotility occurs in 90% of cases. Involvement of the ____ and ____ can lead to malabsorption
esophageal
duodenum, jejunum
In the colon, wide mouth ____ can occur
sacculations
In the kidney, slowly progressive ____ lesions with ____ proliferation may be seen
arterial; intimal
In the kidney, _____ ____ causes a gradual reduction in GFR
adventitial fibrosis
_____ ____ ____ is a sudden collapse of GFR that leads to severe volume depletion
scleroderma renal crisis
In contrast to SLE, renal damage is not ____ and there are no ____ ____
inflammatory; immune complexes
What kind of ischemia can lead to cardiac damage
microvascular
What acronym is used to describe symptoms of limited scleroderma?
CREST
What do each of the letters in CREST stand for?
calcinosis, raynaud, esophageal dysmotility, sclerodactyly, telangiectasia
Limited scleroderma is associated with ______ antibody.
anticentromere
_____ scleroderma (en coup de sabre) is characterized by sclerotic induration and hyperpigmentation occuring on a single extremity or face
linear
____ is a patch or patches of sclerodermatosis skin damage
morphea
____ _____ syndrome is a syndrome of abrupt onset myalgia, fatigue, and peripheral eoisonphilia associated with ingestion of an L-tryptophan dietary supplement
eiosinophilia myalgia
can silicon breast implants cause scleroderma?
no
Diffuse scleroderma is associated with anti ___ antibody (anti-DNA topoisomerase 1)
Scl70
Smoking cessatation is a mainstay because nicotine is a potent _____
vasoconstrictor
a positive ____ is present in about 90% of cases. Anti-RNA _____ III may also be present
ANA; polymerase
