Selected vasculitides

Question 1

Giant cell arteritis clinical features?

Answer 1

changes in vision (amaurosis fugax, must treat to prevent permanent vision loss)
HA (most common)
scalp tenderness
asymmetric enlargement, tenderness, or beading of temporal arteries (pulse may be decreased or absent)
fatigue
fever
jaw claudication
Labs: ESR and CRP
Imaging: CTA/MRA, temporal artery US

Question 2

Polymyalgia rheumatica clinical features?

Answer 2

pain, aching, or stiffness or all three in selected muscle groups (e.g., shoulders or pelvic girdle), with symptoms most pronounced in the morning
fatigue
anorexia
weight loss
Labs: r/o RA with anti CCP and RF, ESR and CRP

Question 3

Diagnosis of giant cell arteritis?

Answer 3

temporal artery bx showing disruption of the internal elastic lamina, thinning of the media, scarring of the intima
3 criteria to meet:
age >50
new HA
abnormal temporal artery bx/temporal artery in general
ESR >50

Question 4

Diagnosis of polymyaglia rheumatica?

Answer 4

mandatory: age >50, aching shoulders, abnormal ESR/CRP
additional: morning stiffness >45 min, hip pain or reduced ROM, - RF and CCP, absence of peripheral synovitis, US findings of synovitis or tenosynovitis in shoulders

Question 5

GCA treatment?

Answer 5

prednisone or prednisolone 1 mg/kg daily and ASA for 2-4 w, taper by 10-20%/week, add back for flares
Tocilizumab if problems tapering or high risk for steroid related adverse outcomes

Question 6

PMR treatment?

Answer 6

prednisone 15 to 20 mg/kg daily for 1-2 months, taper 20%/month

Question 7

ANCA associated vasculitis includes which diseases?

Answer 7

microscopic polyangiitis
granulomatosis with polyangiitis
eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome)

Question 8

Microscopic polyangiitis and granulomatosis with polyangiitis clinical features?

Answer 8

petechial/purpuric rash
inflammatory arthritis (symmetrical small joints primarily)
sinus congestion
sensorineural hearing impairment
bloody nasal crusting/epistaxis
pulmonary hemorrhage
interstitial fibrosis
mononeuritis multiplex
glomerulonephritis with acute kidney injury

Question 9

Ab seen in microscopic polyangiitis vs granulomatosis with polyangiitis vs eosinophilic granulomatosis with polyangiitis

Answer 9

GPA: anti PR3 and cANCA

MPA and EGPA: anti MPO and p ANCA

Question 10

eosinophilic granulomatosis with polyangiitis clinical features?

Answer 10

difficult to control asthma (steroid dempendent)

hypereosinophilic syndrome with associated organ dysfunction from eosinophil infiltration

Question 11

Kidney bx and non kidney bx findings in GPA, MPA and EGPA?

Answer 11

kidney bx: pauci-immune glomerulonephritis
granulomatous inflammation with vasculitis and the absence of microorganisms is supportive of GPA and EGPA
same histopathology without granulomata is supportive of MPA

Question 12

Treatment for ANCA associated vasculitis?

Answer 12

Life threatening: induction therapy with high-dose glucocorticoids and another immunosuppressive agent (cyclophosphamide or rituximab).
Maintenance therapy: combination of tapering glucocorticoids and another agent (azathioprine or mycophenolate)
Non-life threatening: weekly methotrexate with adjunctive glucocorticoid
can use tocilizumab with prednisone taper in GCA
can relapse easily

Question 13

Which ANCA Ab is more risk for relapse?

Answer 13

PR3-ANCA

Question 14

IV pulse steroids to vs prednisolone taper for GCA?

Answer 14

IV pulse steroids led to more-rapid tapering of prednisone and higher frequency of sustained remission than standard