CTD part II Flashcards
Why do you measure Ab 12 weeks apart for Dx APS
because can be elevated during infecitous disease
what Ig is associated with clinical events in APS
IgG > IgM
what has best correlation with thrombotic events in APS
anti La- SS-B
what can induce La formation
heparin and other anticoagulants
confirmatory test for APS
russel viper venom test
What are the types of vascular thrombosis assoc wtih APS
TIA, CVA, venous sinus thrombosis, DVT, PE, budd chiari, digital infarction, thrombocytopenia, MI, heart valve disease, retinal thrombosis, adrenal infarction, avasc necrosis
what is asherson syndrome
catastrophic APS
What are pathophys causes arterial and venous clots
defects in clot inhibitors defects in clot lysis hyperviscosity defects in vascular endothelium
diseases assoc with arterial and venous clots!!!
TTP HIT DIC APS (La, AcL, antiB2GP1) PNH infective endocarditis vasculitis
what causes a reticulated rash, giraffe spot like
spasm deeper arterioles that supply superficial horizontal plexus with pooling of blood
causes of livedo reticularis
SLE and PAN RA PSS dermatomyositis
obstructive causes livedo reticularis
cryoglobulins, cold agglutinins APS ateriosclerosis (cholesterol emboli) DIC TTP hyperCa polycythemia rubra vera infections- parvo and syphilis
key feature of scleroderma
tightening of skin and edema then fibrosis
morphea
rash seen in PSS
signs PSS
raynauds cutaneous, subcut calcification, pigment and depigment, telangiectasias cardiac: pericarditis fibrosis, MI, heart block pulm: restrictive, dec CO diffusion, pulm HTN GI: GERD, sticking below sternum(dysphagia low)!!!, hypomotility with bacterial overgrowth renal: onion skinning!!!! and HTN
unctonrolled HTN despite therapy. weight loss, dyspnea, dysphagia and polyarthralgias tendon friction rubs forearms and shins 2+ pitting edema
PSS fibroses
what cause PSS
fibroblast metabolic defect, inc collagen in ECM endothelial dysfunction autoimmunity silica, solvents paraneoplastic
autoimmune HLA assoc with PSS
DPB1 and DPB2
Diagnostic criteria scleroderma
1 major and 2-3 minor
major findings scleroderma
symmetric thickening, tightening and induration of skin proximal to MCP or MTP joints
minor criteria findings scleroderma
sclerodactyly digital pitting bi basilar pulmonary fibrosis
most important minor finding in scleroderma
bibasilar pulmonary fibrosis
“high probability scleroderma”
3/5 CREST features or definite raynaud with nail fold changes and scleroderma specific Ab
first sign PSS
raynauds
skin findings in PSS
nonpitting edema with itching subcut fat loss and hair loss subcut calcification finger tip ulceration telangiectasia
Triphasic color of raynauds
pallor cyanosis rubor
what can casue raynauds
cold, Rx emotional stress
digital ulcer association
anti-topoisomerase
finger loss association
anti-centromere
primary raynauds
age <30 symmetrical normal SR, no nail bed capilary changes
secondary Raynauds
CT disease**Scleroderma with nail changes and serologic changes Drugs structural arterial disease occupational -vibration hermorrheologic– PV cyroglobulins other pheochromo or hypothyroid
drugs that can cause raynauds
ergotamine bleomycin vinblastine caffeine nicotine
raynaud Tx
lifestyle medications: dihydroCCBs, phosphodiesterase inhibitors (viagra), infusion prostacyclin extreme- sympathectomy
What is used to Tx finger tip ulcers assoc with raynauds
endothelin R antagonist or statin
what are the skin and MSK involvement with scleroderma
tendon friction rubs, joint stiffness, restricted ROM
Tx of scleroderma
mycophenolate
visceral changes in scleroderma
skin thickening: visceral thickening heart, lungs, GI kidneys
what can occur in GI with PSS
hypomotile bowels leading to bacterial overgrowth
death from PSS
used to be kidney failure secondary to HTN, but have ACEI now from Lung involvement
how can scleroderma present
HTN encephalopathy sudden dec in GFR, RBC or protein in urine grade 3-4 retinopathy microangiopathic hemolytic anemia
Risk factors pointing to renal disease
rapid skin progression anemia anti RNA polymerase III recent intesification of lgucocorticoids
Tx of scleroderma
ACEI
how to Dx scleroderma CV disease
NT pro-BNP and doppler yearly
scleroderma in lung
interstitial lung disease pulmonary HTN
risk factors lung involvement scleroderma, specifically interstitial lung disease
afroamerican anti-topoisomerase AntiScl-70 dec FVC fibrosis on HRCT
risk factors for pulmonary HTN with scleroderma
telangectasia late age onset decreased diffusion capacity elevated N proBNP anti centromere Ab, Anti B23
what is this rash
livedo reticularis
identify associations
raynauds
ulcerations
PSS
anti-topoisomerase
what is this
first presentation in PSS
called morphea
