Scleroderma and gout Flashcards
Reynaud’s
and when is it serious
Raynaud’s is usually triggered by cold temperatures, anxiety or stress. The condition occurs because your blood vessels go into a temporary spasm, which blocks the flow of blood. This causes the affected area to change colour to white, then blue and then red, as the bloodflow returns
common in young women
if + ulcers, -> underlying autoimmune condition
old age/men -> concerning
primary Reynaud’s
no underlying autoimmune condition
no ulcers
symptoms of cold hands and feet and painful attacks with changes in temperature
Secondary Reynaud’s
a sign of scleroderma, and is caused by another, potentially serious health condition, including lupus or vasculitis as well as scleroderma
can severely restrict the blood supply, so it carries a higher risk of complications, such as ulcers, scarring and even tissue death (gangrene) in the most serious cases.
diagnosing Reynaud’s
symptoms
thermal threshold test (The water test shows the temperature response (by thermography) to a cold challenge - one minute immersion of hands in cold water at 15 degrees C.)
blood tests
types of scleroderma
limited ssc (systemic sclerosis)
affects limited parts of body: feet, hands, knees…
diffuse ssc
skin involvement
scleroderma and trigger
systemic autoimmune disease
auto-antibody mediated
triggered by event: infection, environmental toxin in someone with genetic susceptibility
scleroderma symptoms
progressive thickening and fibrosis of the skin
(skin tightness)
excessive collagen deposition
fibrosis of internal organs
vascular dysfunction and abnormalities
organs affected by ssc
gastrointestinal:
heart burn
esophageal dysmotility
pulmonary:
fibrosis
pulmonary arterial hypertension
cardiac:
scleroderma heart
MSK (musculoskeletal):
inflammatory joints
Raynaud’s with digital ulceration
renal:
renal crisis
connective tissue homeostasis
connective tissue acts as a scaffold
main cell fibroblasts
essential for wound healing
can degrade ECM or maintain ECM
during tissue injury fibroblasts are overactivated,
pathophysiology of scleroderma
increased ECM deposition by activated fibroblasts
leading to pathological scarring
expressive production of fibroblasts
fibroblasts promote pro-fibrotic microenvironment secreting GFs, chemokines and cytokines leading to more fibroblast production
myositis
Myositis is the name for a group of rare conditions. The main symptoms are weak, painful or aching muscles. This usually gets worse, slowly over time. You may also trip or fall a lot, and be very tired after walking or standing
inflammation of muscles
renal crisis
Scleroderma renal crisis is a life-threatening complication of scleroderma and presents with the abrupt onset of severe hypertension accompanied by rapidly progressive renal failure, hypertensive encephalopathy, congestive heart failure, and/or microangiopathic hemolytic anemia.
high bp and seizures
treating renal crisis
reduce BP
ace inhibitors
renal transplant
hypertensive crisis complications
cardiac:
heart attack
pulmonary oedema
renal:
AKI
CNS:
Ocular: burst blood vessels at back of eye
blindness
predicting scleroderma disease phenotype
test for antibodies:
anti- centromere: hypertension
topoisomerase: pulmonary fibrosis
polymerase: scleroderma renal crisis
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