CTD part II Flashcards

1
Q

Why do you measure Ab 12 weeks apart for Dx APS

A

because can be elevated during infecitous disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what Ig is associated with clinical events in APS

A

IgG > IgM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what has best correlation with thrombotic events in APS

A

anti La- SS-B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what can induce La formation

A

heparin and other anticoagulants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

confirmatory test for APS

A

russel viper venom test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the types of vascular thrombosis assoc wtih APS

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is asherson syndrome

A

catastrophic APS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are pathophys causes arterial and venous clots

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

diseases assoc with arterial and venous clots!!!

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what causes a reticulated rash, giraffe spot like

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

causes of livedo reticularis

A

SLE and PAN RA PSS dermatomyositis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

obstructive causes livedo reticularis

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

key feature of scleroderma

A

tightening of skin and edema then fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

morphea

A

rash seen in PSS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

signs PSS

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

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

A

PSS fibroses

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what cause PSS

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

autoimmune HLA assoc with PSS

A

DPB1 and DPB2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Diagnostic criteria scleroderma

A

1 major and 2-3 minor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

major findings scleroderma

A

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

21
Q

minor criteria findings scleroderma

A

sclerodactyly digital pitting bi basilar pulmonary fibrosis

22
Q

most important minor finding in scleroderma

A

bibasilar pulmonary fibrosis

23
Q

“high probability scleroderma”

A

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

24
Q

first sign PSS

A

raynauds

25
Q

skin findings in PSS

A

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

26
Q

Triphasic color of raynauds

A

pallor cyanosis rubor

27
Q

what can casue raynauds

A

cold, Rx emotional stress

28
Q

digital ulcer association

A

anti-topoisomerase

29
Q

finger loss association

A

anti-centromere

30
Q

primary raynauds

A

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

31
Q

secondary Raynauds

A

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

32
Q

drugs that can cause raynauds

A

ergotamine bleomycin vinblastine caffeine nicotine

33
Q

raynaud Tx

A

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

34
Q

What is used to Tx finger tip ulcers assoc with raynauds

A

endothelin R antagonist or statin

35
Q

what are the skin and MSK involvement with scleroderma

A

tendon friction rubs, joint stiffness, restricted ROM

36
Q

Tx of scleroderma

A

mycophenolate

37
Q

visceral changes in scleroderma

A

skin thickening: visceral thickening heart, lungs, GI kidneys

38
Q

what can occur in GI with PSS

A

hypomotile bowels leading to bacterial overgrowth

39
Q

death from PSS

A

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

40
Q

how can scleroderma present

A

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

41
Q

Risk factors pointing to renal disease

A

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

42
Q

Tx of scleroderma

A

ACEI

43
Q

how to Dx scleroderma CV disease

A

NT pro-BNP and doppler yearly

44
Q

scleroderma in lung

A

interstitial lung disease pulmonary HTN

45
Q

risk factors lung involvement scleroderma, specifically interstitial lung disease

A

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

46
Q

risk factors for pulmonary HTN with scleroderma

A

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

47
Q

what is this rash

A

livedo reticularis

48
Q

identify associations

A

raynauds

ulcerations

PSS

anti-topoisomerase

49
Q

what is this

A

first presentation in PSS

called morphea