📋 Key Information Summary
- Classic PAN is a systemic necrotising vasculitis of medium-sized muscular arteries — ANCA-negative and does NOT cause glomerulonephritis or pulmonary capillaritis.
- Approximately 30% of cases are associated with hepatitis B virus (HBV) infection via immune complex deposition; idiopathic cases account for the remainder.
- Hallmark pathology: transmural fibrinoid necrosis with neutrophilic infiltrate at arterial bifurcations, progressing to segmental aneurysms visible on conventional angiography.
- Classic organ involvement includes renal (infarcts, renovascular hypertension — no glomerulonephritis), peripheral nerves (mononeuritis multiplex), mesenteric vasculature (ischaemic colitis), and testes.
- Diagnosis rests on clinical features plus either histological confirmation (biopsy of symptomatic site) or characteristic angiographic aneurysms — ACR 1990 classification criteria require ≥3 of 10 features.
- Five-factor score (FFS 2009) stratifies prognosis and guides treatment intensity: age >65, cardiac involvement, GI involvement, renal insufficiency (Cr >140 µmol/L), ear/nose/throat involvement.
- Idiopathic PAN: treat with combination cyclophosphamide + high-dose prednisolone for remission induction; azathioprine or methotrexate for maintenance.
- HBV-associated PAN: antiviral therapy (entecavir or tenofovir) is the cornerstone; short-course corticosteroids plus plasma exchange — do NOT give prolonged cyclophosphamide.
- Prednisolone taper typically extends over 12–18 months; abrupt discontinuation risks relapse.
- Pulmonary involvement is ABSENT in classic PAN — if present, consider ANCA-associated vasculitis (e.g., GPA, MPA, EGPA) instead.
- Aboriginal and Torres Strait Islander peoples have higher HBV prevalence; screen all PAN patients for HBV and tailor therapy accordingly.
- Relapse occurs in 10–20% of idiopathic PAN; monitor for recurrent neuropathy, skin lesions, or rising inflammatory markers after remission.
Introduction & Australian Epidemiology
Classic polyarteritis nodosa (PAN) is a systemic necrotising vasculitis predominantly affecting medium-sized muscular arteries, causing transmural inflammation, segmental necrosis, and subsequent ischaemic injury to visceral organs and peripheral nerves. Crucially, PAN spares the pulmonary circulation and does not produce glomerulonephritis — features that distinguish it from ANCA-associated vasculitides.
In Australia, PAN is rare, with an estimated incidence of 2–9 per million population per year. The disease shows a male predominance (M:F ratio ~2:1) and a peak incidence in the fifth to sixth decade of life. Approximately 30% of cases are associated with hepatitis B virus (HBV) infection, an important consideration given the higher prevalence of chronic HBV in Aboriginal and Torres Strait Islander communities and among migrants from endemic regions (Southeast Asia, Sub-Saharan Africa, Pacific Islands).
The distinction between HBV-associated and idiopathic PAN is therapeutically critical: HBV-associated PAN responds best to antiviral therapy with short-course corticosteroids and plasma exchange, whereas idiopathic PAN requires immunosuppressive induction with cyclophosphamide. Accurate classification therefore directly impacts treatment outcomes.
Do not confuse with ANCA-associated vasculitis: Classic PAN is ANCA-negative. A positive c-ANCA or p-ANCA should prompt consideration of granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic granulomatosis with polyangiitis (EGPA) — which involve small vessels, may cause glomerulonephritis, and have different treatment algorithms.
Pathophysiology & Classification
Pathogenesis
Classic PAN is characterised by ANCA-negative necrotising arteritis of medium-sized muscular arteries at branch points. The pathological hallmark is transmural fibrinoid necrosis with neutrophil infiltration of the vessel wall, progressing to mononuclear cell infiltrates, intimal proliferation, and eventual fibrosis. Segmental aneurysms (up to 1 cm) form at arterial bifurcations and are visible on conventional catheter-based angiography.
Two distinct aetiological pathways are recognised:
- HBV-associated PAN (~30% of cases): Immune complexes containing HBV surface antigen (HBsAg) and antibody deposit in arterial walls, activating complement and driving transmural inflammation. Circulating immune complexes correlate with disease activity. This form typically presents within six months of acute HBV infection and is more common in younger patients.
- Idiopathic PAN (~70% of cases): No identifiable infectious trigger. The immunopathogenesis is incompletely understood but involves T-cell–mediated vascular injury and macrophage activation. Some cases are associated with hairy-cell leukaemia, relapsing polychondritis, or familial Mediterranean fever (FMF).
Classification Criteria — ACR 1990
The American College of Rheumatology (ACR) 1990 classification criteria require ≥3 of the following 10 features for classifying a patient as having PAN:
| Criterion | Definition | Sensitivity / Specificity |
|---|---|---|
| 1. Weight loss ≥4 kg | Unintentional, since illness began, not attributable to dieting | 73% / 73% |
| 2. Livedo reticularis | Mottled, net-like violaceous skin discolouration | 16% / 96% |
| 3. Testicular pain or tenderness | On history or examination, not attributable to infection or trauma | 16% / 96% |
| 4. Myalgia, weakness, or leg tenderness | Diffuse myalgias or leg tenderness, or mild muscle weakness of intrinsic muscles | 65% / 75% |
| 5. Mononeuropathy or polyneuropathy | Development of mononeuritis multiplex or peripheral polyneuropathy | 58% / 74% |
| 6. Diastolic BP >90 mmHg | New-onset or worsening hypertension | 53% / 73% |
| 7. Elevated BUN or creatinine | BUN >40 mg/dL (14.3 mmol/L) or creatinine >1.5 mg/dL (133 µmol/L) — due to renal infarction, NOT glomerulonephritis | 37% / 88% |
| 8. HBV seropositivity | Positive anti-HBsAg antibody or HBsAg | 19% / 89% |
| 9. Arteriographic abnormality | Aneurysms or occlusions of visceral arteries (not due to atherosclerosis, fibromuscular dysplasia, or other causes) | 45% / 91% |
| 10. Biopsy of small or medium artery containing PMN | Histological evidence of granulocytes or granulocytes and mononuclear cells in the artery wall | 22% / 91% |
The 1990 ACR criteria have a reported sensitivity of 82.2% and specificity of 86.6% for distinguishing PAN from other vasculitides. These are classification criteria (designed for research), not diagnostic criteria — clinical judgement remains essential.
Chapel Hill Consensus (2012): PAN is defined as "necrotising arteritis of medium or small arteries without vasculitis in arterioles, capillaries, or venules, and not associated with ANCA." This emphasises the medium-vessel and ANCA-negative nature of the disease.
Clinical Features
PAN is a multisystem disease with protean manifestations driven by ischaemia and infarction of medium-sized arteries. Constitutional symptoms (fever, malaise, weight loss) are present in >50% of patients at diagnosis. Organ-specific features depend on the vascular bed involved:
| System | Manifestations | Frequency |
|---|---|---|
| Peripheral nerves | Mononeuritis multiplex (foot/wrist drop); painful sensory neuropathy; asymmetric polyneuropathy | 50–70% |
| Renal | Renovascular hypertension (renal artery branch involvement); renal infarction (flank pain, haematuria); progressive renal impairment — NO glomerulonephritis | 40–60% |
| Gastrointestinal | Mesenteric ischaemia (postprandial abdominal pain, weight loss, GI haemorrhage, bowel perforation); hepatic artery involvement | 30–40% |
| Skin | Livedo reticularis; subcutaneous nodules (classic "nodosa"); purpura; digital gangrene; ulcers | 30–40% |
| Musculoskeletal | Myalgia; arthralgia (non-erosive, non-deforming); leg tenderness | 50–65% |
| Testicular | Testicular pain/tenderness (highly specific but low sensitivity) | 16% |
| Cardiac | Coronary arteritis (angina, MI); pericarditis; cardiomyopathy — contributes to mortality | 10–30% |
| Central nervous system | Cerebral infarction; seizures; encephalopathy (less common than peripheral neuropathy) | 5–10% |
Key diagnostic clues: The combination of new-onset hypertension + mononeuritis multiplex + abdominal pain + constitutional symptoms — with active urine sediment absent (no dysmorphic RBCs or RBC casts) — should strongly suggest PAN. ANCA testing should be negative. Fever of unknown origin with neuropathy is another classic presentation.
Features That EXCLUDE Classic PAN
- Pulmonary involvement (haemoptysis, nodules, cavitation) → consider GPA, MPA, or EGPA
- Glomerulonephritis (dysmorphic red cells, RBC casts, rapidly declining GFR) → consider ANCA-associated vasculitis or IgA vasculitis
- Positive ANCA (c-ANCA/PR3 or p-ANCA/MPO) → not classic PAN
- Small-vessel involvement on biopsy (capillaritis, venulitis) → not classic PAN
Investigations
Laboratory Tests
Essential
Full blood count (FBC)
MBS Item 65070. Typically shows normocytic anaemia, leucocytosis, thrombocytosis — reactive changes.
Essential
ESR and CRP
MBS Item 65070 / 65081. Almost always markedly elevated at presentation (ESR often >60 mm/hr). Useful for monitoring disease activity.
Essential
ANCA panel (c-ANCA/PR3, p-ANCA/MPO)
MBS Item 65103. MUST be NEGATIVE in classic PAN. A positive result redirects diagnosis to ANCA-associated vasculitis.
Essential
Hepatitis B serology (HBsAg, anti-HBs, anti-HBc, HBV DNA)
MBS Item 69483 / 69484. Mandatory — determines HBV-associated vs idiopathic PAN and directly guides treatment. HBV DNA quantitative if HBsAg positive.
Essential
Serum creatinine, eGFR, urea, electrolytes
MBS Item 65070. Renal impairment from infarction (NOT glomerulonephritis). Urinalysis should show bland sediment (no RBC casts).
Available
Hepatitis C, HIV serology
MBS Item 69484. Exclude HCV and HIV co-infection, especially in HBV-associated PAN.
Available
LFTs, serum albumin
MBS Item 65070. Hepatic artery involvement may elevate transaminases. Hypoalbuminaemia reflects systemic inflammation or malnutrition.
Available
Complement levels (C3, C4, CH50)
MBS Item 65093. May be low in HBV-associated PAN (immune complex consumption). Usually normal in idiopathic PAN.
Available
Serum cryoglobulins
MBS Item 65093. Type III cryoglobulinaemia can mimic PAN; sample must be kept warm during transport (37°C).
Imaging
Essential
Conventional catheter-based angiography
Gold standard — demonstrates microaneurysms (up to 1 cm) at renal, hepatic, mesenteric, and coeliac artery bifurcations. Also shows segmental stenoses and occlusions. MBS Item 13200 series. CTA/MRA are less sensitive for small aneurysms but may be used as initial screening.
Available
CT angiography (CTA) abdomen/pelvis
MBS Item 56800. Non-invasive screening for larger visceral artery aneurysms. Less sensitive than catheter angiography for small branch-vessel disease.
Available
MR angiography (MRA)
MBS Item 63001 series. Alternative non-invasive modality; useful if contrast allergy precludes CTA. Limited spatial resolution for small aneurysms.
Available
Nerve conduction studies / EMG
MBS Item 11702 / 11703. Confirms mononeuritis multiplex (asymmetric, axonal pattern) — supports diagnosis when clinical features are equivocal.
Histopathology
Essential if biopsy target available
Tissue biopsy (nerve + muscle, skin, kidney, testis)
Highest diagnostic yield from sural nerve biopsy combined with peroneus brevis muscle (yield ~60–70%). Skin biopsy of a symptomatic lesion (nodule, purpura) has moderate yield. Renal biopsy shows interlobular/arcuate arteritis — NOT glomerulonephritis. Testicular biopsy rarely needed unless clinically indicated.
Bland urinary sediment is expected: In classic PAN, the urinalysis should show NO dysmorphic red blood cells and NO red blood cell casts. If present, consider ANCA-associated vasculitis or IgA nephropathy. Mild proteinuria and haematuria from renal infarction may occur but should not produce an active sediment.
Risk Stratification & Severity Scoring
The Five-Factor Score (FFS), revised in 2009 by the French Vasculitis Study Group (FVSG), is used to predict mortality and guide treatment intensity in systemic vasculitides including PAN:
Low Risk
FFS = 0
No adverse prognostic factors present. Five-year survival >90%.
Setting: Treat with glucocorticoids alone in idiopathic PAN; antivirals + short-course steroids in HBV-PAN
Intermediate Risk
FFS = 1
One adverse prognostic factor. Five-year survival ~80–85%.
Setting: Add immunosuppressive agent (cyclophosphamide) to induction regimen
High Risk
FFS ≥ 2
Two or more adverse factors. Five-year survival ~55–70%.
Setting: Intensive combination therapy; consider plasma exchange; ICU-level monitoring if visceral crisis
Five-Factor Score (FFS 2009) — Adverse Prognostic Factors
| Factor | Definition |
|---|---|
| Age >65 years | At time of diagnosis |
| Cardiac symptoms | Cardiomyopathy, heart failure, or pericarditis attributable to vasculitis |
| GI involvement | Mesenteric ischaemia, perforation, haemorrhage, pancreatitis, or biliary involvement |
| Renal insufficiency | Serum creatinine >140 µmol/L (≥1.58 mg/dL) |
| ENT involvement | Absent in classic PAN (only scored in AAV) — effectively only 4 factors apply to PAN |
Note on FFS and PAN: The ENT factor is scored as 0 in PAN by definition (PAN does not affect the respiratory tract). For PAN, only age >65, cardiac, GI, and renal factors apply. GI involvement carries the worst prognosis and is the strongest single predictor of mortality.
Treatment
Treatment of PAN depends critically on whether the disease is idiopathic or HBV-associated. The two forms require fundamentally different therapeutic strategies.
Idiopathic PAN — Remission Induction
Prednisolone
Solone® · Panafcortelone® · Glucocorticoid
Adult dose
1 mg/kg/day PO (max 60 mg) for 2–4 weeks, then taper by 5–10 mg every 1–2 weeks to 20 mg, then by 2.5 mg every 2–4 weeks; total taper 12–18 months
Paediatric dose
1–2 mg/kg/day PO (max 60 mg); taper guided by paediatric rheumatologist
Route
Oral (IV methylprednisolone pulse 500 mg–1 g for severe/fulminant disease × 3 days)
Renal adjustment
No dose adjustment; monitor glucose, potassium, and BP closely
PBS status
✔ PBS General Benefit
Cyclophosphamide
Endoxan® · Alkylating agent
Adult dose — oral
2 mg/kg/day PO (max 150 mg/day) for 3–6 months, combined with prednisolone
Adult dose — IV pulse
15 mg/kg IV pulse every 2–3 weeks for 3–6 pulses; lower cumulative dose, fewer side effects — preferred in many Australian centres
Paediatric dose
2 mg/kg/day PO or 10–15 mg/kg IV pulse; specialist supervision mandatory
Renal adjustment
eGFR 10–50: reduce dose by 25%; eGFR <10: reduce by 50%; dialysed patient: give after dialysis
Key monitoring
FBC fortnightly (neutropenia risk); urinalysis (haemorrhagic cystitis); mesna co-administration with IV pulses; lifetime bladder cancer risk
PBS status
✔ PBS General Benefit
Idiopathic PAN — Remission Maintenance
Azathioprine
Imuran® · Purine analogue
Adult dose
2 mg/kg/day PO (typically 100–200 mg/day) after cyclophosphamide induction; continue for ≥2 years
Paediatric dose
2 mg/kg/day PO; specialist guidance required
Key note
Check TPMT/NUDT15 genotype before commencing; FBC monitoring fortnightly × 8 weeks then monthly
PBS status
✔ PBS General Benefit
Methotrexate
Methoblastin® · Antifolate
Adult dose
15–25 mg PO/SC weekly with folic acid 5 mg the day after methotrexate
Renal adjustment
eGFR <30: avoid; eGFR 30–50: reduce dose by 50%; use with extreme caution
PBS status
✔ PBS General Benefit
HBV-Associated PAN — Specific Treatment
Do NOT give prolonged immunosuppression in HBV-PAN: Cyclophosphamide and sustained high-dose corticosteroids promote HBV replication and risk fatal hepatic flare. Antiviral therapy is the cornerstone of treatment.
Entecavir
Baraclude® · Nucleoside analogue
Adult dose
0.5 mg PO daily (1 mg if lamivudine-resistant); first-line antiviral for HBV-PAN
Duration
Continue until HBsAg seroconversion or indefinite (long-term suppressive therapy)
Renal adjustment
eGFR 30–49: 0.25 mg daily; eGFR 10–29: 0.15 mg daily; dialysis: 0.05 mg after dialysis
PBS status
⚠ PBS Authority Required
Tenofovir alafenamide (TAF)
Vemlidy® · Nucleotide analogue
Adult dose
25 mg PO daily; alternative first-line antiviral with less nephrotoxicity than tenofovir disoproxil (TDF)
Renal adjustment
eGFR <15: not recommended; preferred over TDF in CKD
PBS status
⚠ PBS Authority Required
Short-course corticosteroids in HBV-PAN: Prednisolone 1 mg/kg/day for 2 weeks, then rapid taper over 2–4 weeks (total duration ≤6 weeks). Only enough to control acute vasculitis while antivirals take effect.
Plasma exchange in HBV-PAN: 3–7 sessions (60 mL/kg per session) in the first 2 weeks to remove circulating immune complexes. Indicated for severe disease (FFS ≥1) or visceral crisis. Available at major tertiary centres.
Supportive Therapies
| Intervention | Indication | Details |
|---|---|---|
| Antihypertensive therapy | Renovascular hypertension | ACE inhibitor or ARB first-line; target BP <130/80 mmHg |
| Pneumocystis jirovecii prophylaxis | All patients on cyclophosphamide or ≥20 mg prednisolone >4 weeks | Trimethoprim 160 mg / sulfamethoxazole 800 mg PO daily (or 3 times/week) — PBS General Benefit |
| Osteoporosis prophylaxis | Prednisolone ≥7.5 mg for ≥3 months | Calcium 1000 mg + vitamin D 1000 IU daily; consider bisphosphonate if age >65 or prior fracture |
| Gastric protection | Prednisolone + NSAID or GI risk factors | Pantoprazole 20–40 mg daily — PBS General Benefit |
| Analgesia | Neuropathic pain, myalgia | Gabapentin, pregabalin, or duloxetine for neuropathic pain; paracetamol for myalgia |
Monitoring
Disease Activity Monitoring
Monthly
ESR and CRP during active treatment and tapering; clinical review for new symptoms (neuropathy, skin lesions, abdominal pain).
Every 3 months
FBC, renal function, LFTs (cyclophosphamide/azathioprine toxicity); urinalysis (bland sediment expected); BP monitoring.
Every 6 months
HBV DNA quantitative (in HBV-PAN — should become undetectable); HBsAg status.
After remission
Clinical review every 3–6 months for ≥5 years; relapse rate ~10–20% in idiopathic PAN.
Relapse Surveillance
- Watch for recurrent mononeuritis multiplex, new skin nodules/ulcers, rising ESR/CRP, worsening hypertension, or abdominal symptoms.
- Relapse is more common during steroid taper — reduce taper rate if relapse occurs.
- HBV-PAN relapse usually indicates antiviral failure or non-adherence — check HBV DNA and antiviral resistance.
Cyclophosphamide Toxicity Monitoring
| Toxicity | Monitoring | Action |
|---|---|---|
| Neutropenia | FBC every 2 weeks during oral therapy; day 10–14 after IV pulse | Hold if ANC <1.5 × 10⁹/L; G-CSF if clinically indicated |
| Haemorrhagic cystitis | Urinalysis each visit; maintain hydration ≥2 L/day | Mesna co-administration with IV pulses; switch to azathioprine if recurrent |
| Gonadal toxicity | Counselling at baseline | Consider GnRH agonist (goserelin) or sperm/egg cryopreservation before treatment |
| Bladder malignancy | Lifelong risk; annual urinalysis for haematuria | Urological referral if persistent haematuria after cumulative dose >30 g |
Special Populations
Pregnancy
Cyclophosphamide: Contraindicated — teratogenic (FDA Category D). Avoid conception during and for 3 months after treatment.
Prednisolone: Relatively safe in pregnancy; use lowest effective dose. Monitor for gestational diabetes.
Azathioprine: Can be continued in pregnancy if needed for maintenance (no significant teratogenic risk at standard doses).
Methotrexate: Contraindicated — abortifacient and teratogenic; discontinue ≥3 months before conception.
Entecavir/TAF: Limited human data; switch to tenofovir disoproxil (TDF) which has more established safety data in pregnancy.
Ideally, achieve remission before pregnancy. Manage with obstetrician and rheumatologist co-care.
Paediatrics
PAN in children is rare and more frequently linked to streptococcal infection than HBV in Australia.
Cutaneous PAN (limited to skin and musculoskeletal) is more common in children and may respond to corticosteroids alone or colchicine.
Cyclophosphamide: 2 mg/kg/day or 10–15 mg/kg IV pulse; dose-limiting side effects are more significant in children.
Mycophenolate: Used as steroid-sparing agent in some paediatric centres; PBS available with authority.
Growth monitoring essential on long-term steroids. Bone age assessment if prolonged glucocorticoid use.
Elderly (>65 years)
Age >65 is an independent adverse prognostic factor in the FFS.
Higher risk of cyclophosphamide toxicity (myelosuppression, infection). Prefer IV pulse over daily oral.
Glucocorticoid complications more common: diabetes, osteoporosis, cataracts, psychosis.
Consider rituximab off-label as cyclophosphamide-sparing agent if significant comorbidities (discuss with rheumatology).
Aggressive osteoporosis prophylaxis; monitor glucose closely; lower threshold for PJP prophylaxis.
Renal Impairment
Renal impairment in PAN is from infarction, not glomerulonephritis — renal function often does not improve with immunosuppression.
Cyclophosphamide: Dose reduce: eGFR 10–50: −25%; eGFR <10: −50%.
Methotrexate: eGFR <30: avoid. eGFR 30–50: reduce by 50%.
Nephrotoxic agents (NSAIDs, gentamicin) should be avoided.
Reno-protective strategies (ACEi/ARB) important. Monitor for accelerated atherosclerosis from renovascular disease.
Hepatic Impairment
Hepatic artery vasculitis may cause abnormal LFTs — do not assume drug toxicity.
Azathioprine: Hepatotoxic; reduce dose or avoid in significant hepatic impairment.
Methotrexate: Avoid in significant hepatic impairment (hepatotoxic; alcohol abstinence essential).
In HBV-PAN: monitor for hepatic flare during immunosuppression; antiviral therapy should be initiated BEFORE corticosteroids.
Hepatology referral for HBV-PAN with significant liver involvement.
Immunocompromised
Cyclophosphamide + corticosteroids carry a high risk of opportunistic infection.
PJP prophylaxis is mandatory (co-trimoxazole).
Screen for latent TB (IGRA) before starting immunosuppression.
Avoid live vaccines during immunosuppression; ensure influenza and pneumococcal vaccination up to date.
If recurrent infections on cyclophosphamide, switch to azathioprine or rituximab early.
Aboriginal and Torres Strait Islander Health Considerations
Aboriginal and Torres Strait Islander Health
Quick Reference — Treatment Summary
Idiopathic PAN (FFS = 0)
Prednisolone alone
Taper over 12–18 months
Monitor for relapse during taper
Idiopathic PAN (FFS ≥ 1)
Cyclophosphamide + Prednisolone
CYC 3–6 months → Azathioprine ≥2 years
PJP prophylaxis; FBC monitoring
HBV-Associated PAN
Entecavir 0.5 mg daily + short-course prednisolone (≤6 weeks) ± plasma exchange
Antiviral until HBsAg seroconversion (often indefinite)
NO prolonged cyclophosphamide; monitor HBV DNA
📚 References
- 1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11.
- 2. Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990;33(8):1088–1093.
- 3. Guillevin L, Pagnoux C, Seror R, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19–27.
- 4. Pagnoux C, Seror R, Henegar C, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62(2):616–626.
- 5. Guillevin L, Mahr A, Callard P, et al. Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore). 2005;84(5):313–322.
- 6. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68(3):310–317.
- 7. De Virgilio A, Greco A, Magliulo G, et al. Polyarteritis nodosa: a contemporary overview. Autoimmun Rev. 2016;15(6):564–570.
- 8. Australian Government Department of Health. National Hepatitis B Strategy 2018–2022. Canberra: Commonwealth of Australia; 2018.
- 9. Australian Institute of Health and Welfare (AIHW). Aboriginal and Torres Strait Islander Health Performance Framework 2020 summary report. Canberra: AIHW; 2020.
- 10. Kidney Health Australia. Caring for Australasians with Renal Impairment (CARI) Guidelines — Renal Vasculitis. Melbourne: KHA; 2020.
- 11. RACGP. Guidelines for preventive activities in general practice (Red Book). 10th edn. East Melbourne: RACGP; 2018.
- 12. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook. Australian Government Department of Health, Canberra. Updated 2023. Available at: immunisationhandbook.health.gov.au.
- 13. Robson JC, Dawson J, Cronholm PF, et al. Patient-reported outcomes in vasculitis. Curr Opin Rheumatol. 2018;30(1):48–54.