Vasculitis

Question 1

Define vasculitis.

Answer 1

Inflammation and necrosis of vessel walls causing ischaemia and organ damage.

Question 2

How are vasculitides classified?

Answer 2

By vessel size: large (GCA, Takayasu), medium (PAN), small (GPA, MPA, EGPA, immune-complex).

Question 3

Main pathogenic mechanisms?

Answer 3

ANCA-mediated neutrophil activation or immune-complex deposition (↓C3/C4).

Question 4

Key ANCA antigens.

Answer 4

PR3 (c-ANCA) → GPA; MPO (p-ANCA) → MPA/EGPA.

Question 5

Drugs causing ANCA vasculitis.

Answer 5

Hydralazine, PTU, minocycline, cocaine-levamisole.

Question 6

Large-vessel examples.

Answer 6

GCA, Takayasu arteritis.

Question 7

Medium-vessel example.

Answer 7

Polyarteritis nodosa (PAN).

Question 8

Small-vessel examples.

Answer 8

GPA, MPA, EGPA, cryoglobulinaemic, IgA vasculitis.

Question 9

Classic features of GCA.

Answer 9

Age >50, headache, jaw claudication, vision loss, PMR overlap.

Question 10

Immediate treatment of GCA.

Answer 10

High-dose prednisolone 40–60 mg/day before biopsy.

Question 11

Steroid-sparing drug for GCA.

Answer 11

Tocilizumab (IL-6 inhibitor).

Question 12

Takayasu hallmark.

Answer 12

Young women, pulseless limbs, BP difference >10 mmHg, aortic thickening on CTA/MRA.

Question 13

PAN key feature.

Answer 13

Medium-vessel necrotising arteritis sparing lungs; HBsAg positive in 30%.

Question 14

PAN angiogram finding.

Answer 14

Microaneurysms – ‘string of beads’ appearance.

Question 15

GPA key triad.

Answer 15

ENT, lung, and renal granulomatous disease.

Question 16

ANCA pattern in GPA.

Answer 16

c-ANCA (PR3 positive).

Question 17

MPA hallmark.

Answer 17

No granulomas, alveolar haemorrhage + GN, p-ANCA (MPO).

Question 18

EGPA triad.

Answer 18

Asthma + eosinophilia + neuropathy.

Question 19

Main cause of death in EGPA.

Answer 19

Cardiac involvement (myocarditis, restrictive cardiomyopathy).

Question 20

Mepolizumab use.

Answer 20

IL-5 blocker for refractory EGPA (PBS 2025).

Question 21

Cryoglobulinaemic vasculitis cause.

Answer 21

HCV infection; low complement; purpura + neuropathy.

Question 22

Induction therapy for AAV.

Answer 22

High-dose steroids + rituximab or cyclophosphamide.

Question 23

Maintenance therapy for AAV.

Answer 23

Azathioprine, MMF, MTX, or rituximab; pred ≤7.5 mg by 3 mo.

Question 24

Avacopan mechanism.

Answer 24

C5a receptor blocker – steroid-sparing agent (TGA 2023).

Question 25

When is plasma exchange indicated?

Answer 25

No routine use (PEXIVAS 2020: no mortality/ESRD benefit).

Question 26

PBS-listed biologics.

Answer 26

Rituximab (AAV), Tocilizumab (GCA), Mepolizumab (EGPA).

Question 27

Investigations for vasculitis.

Answer 27

ESR/CRP, urinalysis, ANCA, complement, HBV/HCV serology, biopsy, imaging.

Question 28

GCA biopsy note.

Answer 28

Skip lesions possible – negative result doesn’t exclude diagnosis.

Question 29

Pulmonary–renal syndrome causes.

Answer 29

AAV > anti-GBM > SLE.

Question 30

Key differentiator: PAN vs MPA.

Answer 30

PAN = lung spared, ANCA negative; MPA = lung + renal, ANCA positive.

Question 31

Most relapse-prone AAV type.

Answer 31

PR3-positive (GPA) more relapse than MPO (MPA/EGPA).

Question 32

GCA imaging clue.

Answer 32

PET-CT uptake in aortic branches confirms large-vessel involvement.

Question 33

RAVE/RITUXVAS trial finding.

Answer 33

Rituximab non-inferior to cyclophosphamide for AAV induction.

Question 34

GiACTA trial finding.

Answer 34

Tocilizumab reduced GCA relapse by >50% (NNT≈8).

Question 35

ADVOCATE trial finding.

Answer 35

Avacopan non-inferior to prednisone taper for AAV induction.

Question 36

PEXIVAS finding.

Answer 36

Plasma exchange not routinely beneficial in AAV.

Question 37

REMAP-EGPA finding.

Answer 37

Mepolizumab increased relapse-free survival.

Question 38

Exam pearl: GCA treatment timing.

Answer 38

Start steroids immediately—don’t wait for biopsy.

Question 39

Exam pearl: PAN clue.

Answer 39

HBV+, microaneurysms, lung sparing.

Question 40

Exam pearl: EGPA clue.

Answer 40

Asthma, eosinophilia, neuropathy.

Question 41

Exam pearl: GPA clue.

Answer 41

ENT + lung + kidney + c-ANCA (PR3).

Question 42

Exam pearl: MPA clue.

Answer 42

Lung + renal + p-ANCA (MPO).

Question 43

Exam pearl: Cryoglobulinaemia.

Answer 43

HCV + low C4 + purpura + neuropathy.

Question 44

Important safety step pre-biologic.

Answer 44

Screen for HBV, HCV, HIV, TB; vaccinate (pneumo, influenza, hep B).

Question 45

PMR overlap importance.

Answer 45

Half of GCA patients have PMR symptoms.

Question 46

Nerve biopsy yield in PAN.

Answer 46

≈50% diagnostic yield.

Question 47

AAV relapse monitoring.

Answer 47

Rising ANCA, ESR/CRP, haematuria indicate flare.

Question 48

Most lethal complication of GCA.

Answer 48

Irreversible blindness if steroid delayed >24 h.

Question 49

EBM stat: GiACTA remission.

Answer 49

56% steroid-free with tocilizumab vs 14% placebo.

Question 50

EBM stat: Rituximab relapse-free gain.

Answer 50

≈12% better vs cyclophosphamide (RAVE).

Question 51

EBM stat: Tocilizumab NNT.

Answer 51

≈8 to prevent one GCA relapse.

Question 52

Mnemonic for differentiation.

Answer 52

Giant old, Takayasu young, PAN no lung, GPA c-ANCA, MPA p-ANCA, EGPA asthma.

Question 53

Typical demographic and first-line management of Takayasu arteritis?

Answer 53

Young Asian women <40 y (“pulseless disease”). Treat with high-dose corticosteroids ± methotrexate or azathioprine. Refractory cases → TNF inhibitor or tocilizumab. Mnemonic: “TAK = Teen/Adult + Aorta + Kinky pulses.”

Question 54

Which vasculitis is linked to hepatitis B and spares the lungs?

Answer 54

Polyarteritis nodosa (PAN). Medium-vessel necrotising arteritis → neuropathy, livedo, HTN, renal infarcts. HBsAg + in ~30 %. Mnemonic: “PAN’s Hep B plan: Peripheral nerves, Arteries, No lung.”

Question 55

Which biologic is PBS-listed for refractory EGPA in 2025?

Answer 55

Mepolizumab (IL-5 inhibitor). Improves remission and reduces steroid need. Mnemonic: “EGPA breathes easier with ME-polizumab.”

Question 56

Which ANCA subtype is more relapse-prone in AAV?

Answer 56

PR3 / c-ANCA (typical of GPA). Relapses > MPO / p-ANCA (renal-dominant). Mnemonic: “PR3 = Persistent Relapses × 3.”