CTD part II

Question 1

Why do you measure Ab 12 weeks apart for Dx APS

Answer 1

because can be elevated during infecitous disease

Question 2

what Ig is associated with clinical events in APS

Answer 2

IgG > IgM

Question 3

what has best correlation with thrombotic events in APS

Answer 3

anti La- SS-B

Question 4

what can induce La formation

Answer 4

heparin and other anticoagulants

Question 5

confirmatory test for APS

Answer 5

russel viper venom test

Question 6

What are the types of vascular thrombosis assoc wtih APS

Answer 6

TIA, CVA, venous sinus thrombosis, DVT, PE, budd chiari, digital infarction, thrombocytopenia, MI, heart valve disease, retinal thrombosis, adrenal infarction, avasc necrosis

Question 7

what is asherson syndrome

Answer 7

catastrophic APS

Question 8

What are pathophys causes arterial and venous clots

Answer 8

defects in clot inhibitors defects in clot lysis hyperviscosity defects in vascular endothelium

Question 9

diseases assoc with arterial and venous clots!!!

Answer 9

TTP HIT DIC APS (La, AcL, antiB2GP1) PNH infective endocarditis vasculitis

Question 10

what causes a reticulated rash, giraffe spot like

Answer 10

spasm deeper arterioles that supply superficial horizontal plexus with pooling of blood

Question 11

causes of livedo reticularis

Answer 11

SLE and PAN RA PSS dermatomyositis

Question 12

obstructive causes livedo reticularis

Answer 12

cryoglobulins, cold agglutinins APS ateriosclerosis (cholesterol emboli) DIC TTP hyperCa polycythemia rubra vera infections- parvo and syphilis

Question 13

key feature of scleroderma

Answer 13

tightening of skin and edema then fibrosis

Question 14

morphea

Answer 14

rash seen in PSS

Question 15

signs PSS

Answer 15

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

Question 16

unctonrolled HTN despite therapy. weight loss, dyspnea, dysphagia and polyarthralgias tendon friction rubs forearms and shins 2+ pitting edema

Answer 16

PSS fibroses

Question 17

what cause PSS

Answer 17

fibroblast metabolic defect, inc collagen in ECM endothelial dysfunction autoimmunity silica, solvents paraneoplastic

Question 18

autoimmune HLA assoc with PSS

Answer 18

DPB1 and DPB2

Question 19

Diagnostic criteria scleroderma

Answer 19

1 major and 2-3 minor

Question 20

major findings scleroderma

Answer 20

symmetric thickening, tightening and induration of skin proximal to MCP or MTP joints

Question 21

minor criteria findings scleroderma

Answer 21

sclerodactyly digital pitting bi basilar pulmonary fibrosis

Question 22

most important minor finding in scleroderma

Answer 22

bibasilar pulmonary fibrosis

Question 23

“high probability scleroderma”

Answer 23

3/5 CREST features or definite raynaud with nail fold changes and scleroderma specific Ab

Question 24

first sign PSS

Answer 24

raynauds

Question 25

skin findings in PSS

Answer 25

nonpitting edema with itching subcut fat loss and hair loss subcut calcification finger tip ulceration telangiectasia

Question 26

Triphasic color of raynauds

Answer 26

pallor cyanosis rubor

Question 27

what can casue raynauds

Answer 27

cold, Rx emotional stress

Question 28

digital ulcer association

Answer 28

anti-topoisomerase

Question 29

finger loss association

Answer 29

anti-centromere

Question 30

primary raynauds

Answer 30

age \<30 symmetrical normal SR, no nail bed capilary changes

Question 31

secondary Raynauds

Answer 31

CT disease\*\*Scleroderma with nail changes and serologic changes Drugs structural arterial disease occupational -vibration hermorrheologic-- PV cyroglobulins other pheochromo or hypothyroid

Question 32

drugs that can cause raynauds

Answer 32

ergotamine bleomycin vinblastine caffeine nicotine

Question 33

raynaud Tx

Answer 33

lifestyle medications: dihydroCCBs, phosphodiesterase inhibitors (viagra), infusion prostacyclin extreme- sympathectomy

Question 34

What is used to Tx finger tip ulcers assoc with raynauds

Answer 34

endothelin R antagonist or statin

Question 35

what are the skin and MSK involvement with scleroderma

Answer 35

tendon friction rubs, joint stiffness, restricted ROM

Question 36

Tx of scleroderma

Answer 36

mycophenolate

Question 37

visceral changes in scleroderma

Answer 37

skin thickening: visceral thickening heart, lungs, GI kidneys

Question 38

what can occur in GI with PSS

Answer 38

hypomotile bowels leading to bacterial overgrowth

Question 39

death from PSS

Answer 39

used to be kidney failure secondary to HTN, but have ACEI now from Lung involvement

Question 40

how can scleroderma present

Answer 40

HTN encephalopathy sudden dec in GFR, RBC or protein in urine grade 3-4 retinopathy microangiopathic hemolytic anemia

Question 41

Risk factors pointing to renal disease

Answer 41

rapid skin progression anemia anti RNA polymerase III recent intesification of lgucocorticoids

Question 42

Tx of scleroderma

Answer 42

ACEI

Question 43

how to Dx scleroderma CV disease

Answer 43

NT pro-BNP and doppler yearly

Question 44

scleroderma in lung

Answer 44

interstitial lung disease pulmonary HTN

Question 45

risk factors lung involvement scleroderma, specifically interstitial lung disease

Answer 45

afroamerican anti-topoisomerase AntiScl-70 dec FVC fibrosis on HRCT

Question 46

risk factors for pulmonary HTN with scleroderma

Answer 46

telangectasia late age onset decreased diffusion capacity elevated N proBNP anti centromere Ab, Anti B23

Question 47

what is this rash

Answer 47

livedo reticularis

Question 48

identify associations

Answer 48

raynauds ulcerations PSS anti-topoisomerase

Question 49

what is this

Answer 49

first presentation in PSS called morphea