vasculitis

Question 1

secondary cause of vasculitis, anme a drug

Answer 1

levamisole

Question 2

secondary cause of vasculitis, name CTD and granulomatous case

Answer 2

SLE
RA
Sjogren
sarcoid

Question 3

what does cogan involve ?

Answer 3

keratitis + hearing loss

Question 4

buerger’s disease is what t?

Answer 4

thromboangitis obliterans

Question 5

most common stroke in GCA ?

Answer 5

posterior stoke

Question 6

S&S cranial manifestation of GCA ?

Answer 6

• Headache (76% sensitive)
• Jaw claudication (LR +4.2)
• Diplopia (LR +3.4)
• Scalp tenderness
• Amaurosis Fugax/Vision loss

Question 7

Extra cranial/ Systemic manifestation of GCA

Answer 7

• Limb claudication (upper or lower extremity)
• Constitutional symptoms

Question 8

eye finding ( fundoscopy ) in GCA

Answer 8

• acute ischemic optic neuritis
• pale optic disc
• visual acuity issue

Question 9

absolute GCA criteria

Answer 9

above age 50 !!!!!!!

Question 10

type of headache seen in GCA ?

Answer 10

temporal headache

Question 11

vessels that could be involved n your GCA criteria

Answer 11

temporal
axillary
Aorta

Question 12

a sign seen in GCA US of temporal artery

Answer 12

halo sign

Question 13

timeline of obtaining bx when started steroids ?

Answer 13

14 days

Question 14

after gca dx confirmed, what else should you do ?

Answer 14

cta/mra of neck chest abdomen and pelvis to r/o large vessel involvement

Question 15

for takayasu, prefered imaging modality and alternative?

Answer 15

mri
pet/US as alterantive

Question 16

if visual sx or critical cranial ischemia for gca, tx ?

Answer 16

IV pulse steroid 1g x 3 days then pred 1mg/kg + tocilizumab.

Question 17

if no visual sx or loss or critical cranial ischemia

Answer 17

pred 1mg/kg DIE + tocilizumab

Question 18

extracranial GCA ?

Answer 18

pred 1mg/kg die + tcz/mtx

Question 19

when to give ASA in GCA ?

Answer 19

only if critical/flow limiting lesion of carotid/vertebral arteries

Question 20

specific biomarkers for takayasu ?

Answer 20

no

Question 21

takayasu tx

Answer 21

GC + (MTX/AZA/TNFi)

Question 22

required criteria for PMR
tx

Answer 22

• age >50
• bilateral shoulder ache +/- hip pain, absence of other joint pain
• elevated ESR/CRP

tx : prednisone + taper and if relapse ( increase to prerelapse dose and decreased +/- dmardd mtx)

Question 23

livedo reticularis , test pain, mylagia, aneurysm of mesentric hepatic artery, we think of what ?

Answer 23

PAN

Question 24

can PAN have positive seorlogy

Answer 24

yes

Question 25

role for plex in severe disease PAN ?

Answer 25

no

Question 26

which anca associated small vessel vasculittis do you nsal crusting, sinusitis ?

Answer 26

more common in GPA

Question 27

pANCA causes ( other than vasculitis)

Answer 27

* Crohn’s disease or UC * Drug induced (ex. PTU, Cocaine) * CTD * Malignancies * Infections (Hep B, Hep C, HIV)

Question 28

GPA - types of sx - lab

Answer 28

- pulmo renal & ENT - c ANCA >>

Question 29

MPA -type of sx - labs

Answer 29

- pulmo renal - pANCA > C ANCA

Question 30

EGPA - type sx - labs

Answer 30

- asthma, allergic rhinitis, peripheral EO, peripherla neuropathy *rare but severe : CNS and GI *cardiac : 15-50% ( cond recommend echo at time of dx) - pANCA >> ( 40%)

Question 31

GPA/MPA management - severe disease - non severe

Answer 31

Severe - induction : gluco + ritux - maintenance : ritux or dmard ( AZA) *continue tx for at least 18M non severe - induction : gluco + mtx/mmf/aza

Question 32

what's avacopan

Answer 32

new c5a receptor agent that couldd be used in GPA/MPA only

Question 33

Organ?life threatening disease in AAV

Answer 33

1. gn 2. pulmo hmrg 3. reorbital disease 4. cns involvement 5. cardiac involvement 6. moneuritis multiplex 7. mesenteric involvement

Question 34

in what case is plex agreed upon to be used in GPA/MPA ? why ?

Answer 34

if concomitant anti GBM disease ( or if very severe refractory pulmo hmrg or hgih risk of ESRD) risk for infection

Question 35

EGPA severe disease - induction - maintenance non severe disease - induction - maintenance

Answer 35

SEVERE - induction : cortico + ritux/cyclophosmide - maintenance : cortico + mtx/aza/mf/ritux/mepo NON SEVEE - induction: glucocorticoid + mepo> ( mtx/aza/mmf/cyclo/ritux) - maintenance : mepolizumab

Question 36

type 1 monoclonal cryo vasc - type of IG - conditions - tx

Answer 36

- Monoclonal IgM - heme conditions - tx underlying heme prob + PLEX

Question 37

type 2 cryo - type of IG - conditions - tx

Answer 37

- polyclonal IgG, mono IG - 2/2 : Can be secondary to chronic infection (Hep C, Hep B, HIV, IE), connective tissue diseases or lympho-proliferative disorders - tx : 1)HCV Negative: Treat underlying disease Non-Severe: GC +/- colchicine Severe: GC +/- Ritux Life-Threatening: GC + Ritux + PLEX 2)HCV Positive: ALL patients get anti-viral therapy! -Moderate/Severe Disease: may add GC +/- Ritux -Life-Threatening: GC + Ritux + PLEX

Question 38

type 3 cryoglobulinemic vasculitis seen where ?

Answer 38

polyclonal IgM RF against polyclommnal immunoglobulins

Question 39

what's the clinical p[rsentation of actual cryoglobul;inemic vasculitis especially in type 2 or type 3

Answer 39

serum cryo + w/ end organ damage

Question 40

mildmod cryo presentation

Answer 40

Non-ulcerating skin lesions (purpura, acrocyanosis, livedo reticularis) non- debilitating peripheral neuropathy arthralgias/arthritis

Question 41

severe cryo vasculitis

Answer 41

Cutaneous ulcers progressive/debilitating neuropathy GN with renal failure or nephrotic syndrome

Question 42

life threatening

Answer 42

Rapidly progressive GN, CNS involvement, intestinal ischemia, DAH

Question 43

what reason would you delay TJA

Answer 43

- glucose conntrol - smoking cessation/reduction

Question 44

OA vs RA , difference in hand distribution

Answer 44

no DIP in RA

Question 45

avoid IA injections where ?

Answer 45

hand and hip

Question 46

BP target SLE

Answer 46

<130/80