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