(Ch24) Vasculitis

Question 1

Risk factors for systemic sx in vasculitits

Answer 1

1. fever
2. Paresthesias
3. Absence of pain

Question 2

predictors of chronicity (>4w) in vasculitits

Answer 2

1. Arthralgia
2. cryoglobulinemia
3. Absence of fever

Question 3

Risk factors for nephritis in HSP/IgA vasculitis

Answer 3

Risk for nephritis:
1. Male >10y at onset
2. Severe GI sx
3. Joint sx
4. persistent or recurrent purpura
5. Labs (↑Wbc,↑plt,↑ASO,↓C3)
6. HLA-B35
7. Path: ↓eosino in adults, PV C3 not IgA + papillary edema

Question 4

Predictors of relapse in HSP

Answer 4

1. Age >30 Yrs
2. Underlying systemic disorder
3. Persistent purpura >1 month
4. Abdominal pain
5. Hematuria
6. Absence of IgM of DIF

Question 5

Predictors of Poor prognosis in HSP/IgA vasculitis

Answer 5

1. Adult onset
2. Renal failure at Dx
3. Nephrotic syndrome
4. HTN
5. ↓Factor XIII

Question 6

which gene mutation As/w ↓GI Sx in HSP

Answer 6

✗ ICAM-1469 K/E variant

Question 7

Hypocomplementemic urticarial vasculitis syndrome diagnostic criteria

Answer 7

2 Majors + 2 minors

Majors:
1. Urticaria for 6 months
2. Hypocomplements

Minors:
1. Bx proved vasculitis
2. Arthralgia or arthritis 50%
3. ⚑ Uveitis or episcleritis
4. Glomerulonephritis 10%
5. Recurrent abdominal pain
6. +ve C1q precipitin test & ↓C1q

Question 8

HUVS vs SLE

Answer 8

HUVS:
Eye Sx
COPD
angioedema
Lack anti-dsDNA & anti-sm

Question 9

Labs for Urticarial vasculitis

Answer 9

C3/C4
ESR,
ANA (rare dsDNA, Sm)
anti-C1q precipitin
C1q levels

Question 10

Erythema elevated diutinum Classic histopath finding for late stage

Answer 10

Intracellular lipidosis

Question 11

Erythema elevated diutinum As/w

Answer 11

HIV
Hairy cell leukaemia
IgA gammopathy

Extremely rare except ocular finidings
mainstay rx Dapsone

Question 12

Mixed cryglubinemia mutation As/w Hep C

Answer 12

t(14;18)

Consider adding HCV tx to Rx

Question 13

MC Lymphoproliferative disorder As/w Mixed cryglubinemia

Answer 13

B-cell NHL

Question 14

Predictors of relapse in non-infectious mixed cryoglubinemia

Answer 14

Purpura,
Necrosis
Articular involvement

Question 15

Ddx for Lymphocytic vasculitis

Answer 15

Pernio
PLEVA
Panniculitis
Lupus
Bechet

Question 16

If biopsy done on lesions >72h what would be +ve in DIF

Answer 16

only C3 will be detected in lesions present for >72 hours (in 30% of cases).

Question 17

DIF pattern in vasculitis

Answer 17

granular pattern

Question 18

which vasculitis As/w infective endocarditis

Answer 18

Microscopic Polyangiitis
MPA As/w >90% renal involvement
no granuloma

Question 19

medium vessel vasculitis with highest relapse rate

Answer 19

Wegener > MPA >PAN

Question 20

Vasculitis with Staph A induced neutrophils priming

Answer 20

Wegener
sx: Strawberry gums and PG-like ulcers
↓Activity of 𝜶-1 antitrypsin

Question 21

European criteria for Wegener

Answer 21

European criteria (3 of 6):
1. URT inflammation
2. Typical radiologic features on CXR or chest CT
3. Abnormal urine analysis
4. Biopsy-proven granulomatous inflammation
5. Airway stenosis esp in children
6. Serology e.g. anti-PR3 ANCAs.

Question 22

Triggers for Churg–Strauss

Answer 22

Leukotriene inhibitor & omalizumab

Question 23

Bx granuloma of Churg-Strauss vs Wegener

Answer 23

Wegener: palisading neutrophilic Dermatitis w granulomatous basophilic necrobiosis

Churg-struss: palisading Eoisn Dermatitis w granulomatous Eosinophilic necrobiosis

Question 24

Churg strauss +ve ANAC As/w

Answer 24

1. ↑cardiac Sx
2. Pleural effusions
3. Fever
4. Livedo reticularis

Question 25

Churg strauss -ve ANAC As/w

Answer 25

1. purpura
2. renal
3. sinus
4. neuro Sx

Question 26

Phases of Churg strauss

Answer 26

1st prodromal phase
2nd eosinophilic phase:
3rd Vasculitic phase:

Question 27

PAN classic vs skin limited associated infections

Answer 27

Classic PAN: HBV>HCV
spares lung

Cutaneous PAN: Strept esp kids, parvo B19 & HIV

Question 28

APL associated vasculitis and vasculopathy

Answer 28

Vasculopathy: Sneddon
Vasculitis: PAN

Both can be associated with ADA2 deficiency/mutations

Question 29

Which initial labs ordered for vasculitis and to be repeated with flares

Answer 29

CBC+ diff
ESR /CRP
Hepatic panel
Renal + UA
Stool guaiac

Question 30

which vasculitis can present as inflammatory or non-inflammatory retiform purpura, and need to be ruled out in any patient presenting with retiform purpura

Answer 30

ANCA vasculitis and PAN