Overview
Vasculitis Emergency
Topic Overview
Summary
Vasculitis emergencies occur when inflammation of blood vessels causes acute organ damage. ANCA-associated vasculitides (GPA, MPA, EGPA) are the most common causes of life-threatening vasculitis. Key emergencies include pulmonary-renal syndrome (alveolar haemorrhage + rapidly progressive glomerulonephritis), isolated renal crisis, and mononeuritis multiplex. Treatment is urgent immunosuppression (high-dose corticosteroids ± cyclophosphamide or rituximab) and plasma exchange in severe cases.
Key Facts
- ANCA vasculitides: GPA (Wegener's), MPA, EGPA (Churg-Strauss)
- Life-threatening: Pulmonary haemorrhage, RPGN, multi-organ failure
- Key test: ANCA (PR3, MPO), urinalysis (blood, protein, casts)
- Treatment: High-dose steroids + cyclophosphamide or rituximab
- Plasma exchange: For pulmonary haemorrhage or severe renal disease
Clinical Pearls
Pulmonary-renal syndrome = lungs + kidneys = think ANCA vasculitis or Goodpasture's
Urinalysis showing blood and protein with red cell casts = glomerulonephritis — urgent
Do NOT wait for biopsy to start treatment if clinical picture is clear
Why This Matters Clinically
Vasculitis emergencies can cause irreversible organ damage or death within days. Early recognition and aggressive immunosuppression are life-saving.
Visual Summary
Visual assets to be added:
- Vasculitis classification by vessel size
- ANCA vasculitis organ involvement diagram
- Pulmonary-renal syndrome flowchart
- Vasculitis emergency management algorithm
Epidemiology
Incidence
- ANCA-associated vasculitis: 20-25 per million/year
- GPA most common in Northern Europe
- MPA more common in Southern Europe and Asia
Demographics
- Peak age: 50-70 years
- Equal sex distribution (slight male predominance in GPA)
Types of Vasculitis by Vessel Size
| Size | Examples |
|---|---|
| Large vessel | Giant cell arteritis, Takayasu |
| Medium vessel | Polyarteritis nodosa, Kawasaki |
| Small vessel (ANCA) | GPA, MPA, EGPA |
| Small vessel (immune complex) | IgA vasculitis, cryoglobulinaemic |
Pathophysiology
ANCA-Associated Vasculitis
- ANCA antibodies (PR3 or MPO) activate neutrophils
- Neutrophils adhere to endothelium
- Release of reactive oxygen species and enzymes
- Endothelial damage → necrotising vasculitis
- Organ damage (kidneys, lungs, nerves)
Key ANCA Patterns
| Vasculitis | ANCA | Antigen |
|---|---|---|
| GPA | c-ANCA | PR3 (90%) |
| MPA | p-ANCA | MPO (60-70%) |
| EGPA | p-ANCA | MPO (40-60%) |
Pulmonary-Renal Syndrome
- Alveolar haemorrhage (capillaritis)
- Rapidly progressive glomerulonephritis (crescentic GN)
- Most commonly ANCA vasculitis or anti-GBM disease
Clinical Presentation
Constitutional Symptoms
Organ Involvement
| Organ | Features |
|---|---|
| Kidneys | RPGN: rising creatinine, haematuria, proteinuria, red cell casts |
| Lungs | Haemoptysis, infiltrates, respiratory failure (alveolar haemorrhage) |
| ENT | Sinusitis, epistaxis, saddle nose (GPA) |
| Skin | Purpura, ulcers, necrosis |
| Nervous system | Mononeuritis multiplex, peripheral neuropathy |
| Eyes | Scleritis, uveitis |
GPA (Granulomatosis with Polyangiitis) — Classic Triad
MPA (Microscopic Polyangiitis)
EGPA (Eosinophilic Granulomatosis with Polyangiitis)
Red Flags
| Finding | Significance |
|---|---|
| Haemoptysis + rising creatinine | Pulmonary-renal syndrome |
| Creatinine doubling in days | RPGN — urgent |
| New foot/wrist drop | Mononeuritis multiplex |
| Massive haemoptysis | Alveolar haemorrhage |
Fever
Common presentation.
Weight loss
Common presentation.
Fatigue
Common presentation.
Myalgia, arthralgia
Common presentation.
Clinical Examination
General
- Fever
- Weight loss
- Pallor
Respiratory
- Crackles (haemorrhage)
- Hypoxia
Skin
- Palpable purpura
- Ulcers, necrosis
Neurological
- Mononeuritis multiplex (asymmetric neuropathy)
ENT
- Nasal crusting
- Saddle nose deformity (GPA)
Investigations
Blood Tests
| Test | Finding |
|---|---|
| ANCA | PR3 (c-ANCA) or MPO (p-ANCA) |
| FBC | Anaemia, eosinophilia (EGPA) |
| CRP, ESR | Elevated |
| U&E, creatinine | Renal impairment |
| Anti-GBM | Exclude Goodpasture's |
| Complement (C3, C4) | Usually normal in ANCA vasculitis |
Urinalysis — CRITICAL
- Blood (haematuria)
- Protein
- Red cell casts (glomerulonephritis)
Imaging
| Modality | Findings |
|---|---|
| CXR/CT chest | Pulmonary infiltrates, nodules, haemorrhage |
| CT sinuses | Sinusitis, bony erosion (GPA) |
Biopsy
- Renal biopsy: Pauci-immune crescentic GN
- Skin, lung, or nerve biopsy if needed
Bronchoscopy
- Alveolar haemorrhage: Progressively bloodier lavage
Classification & Staging
By ANCA
| Type | ANCA | Antigen |
|---|---|---|
| GPA | c-ANCA | PR3 |
| MPA | p-ANCA | MPO |
| EGPA | p-ANCA | MPO (variable) |
By Severity
- Limited (localised disease)
- Severe (organ-threatening: renal, pulmonary)
Management
Immediate — Life-Threatening Vasculitis
| Action | Details |
|---|---|
| High-dose IV methylprednisolone | 500-1000 mg IV daily for 3 days |
| Then oral prednisolone | 1 mg/kg (max 60 mg) |
| Cyclophosphamide or rituximab | Induction immunosuppression |
| Plasma exchange | For severe pulmonary haemorrhage or dialysis-dependent renal failure |
Induction Therapy
| Agent | Regimen |
|---|---|
| Cyclophosphamide | IV pulses (15 mg/kg every 2 weeks x3, then every 3 weeks) or oral daily |
| Rituximab | 375 mg/m² weekly x4 OR 1g x2 (2 weeks apart) |
Maintenance Therapy
- Azathioprine or rituximab
- Low-dose prednisolone
- Duration: 2+ years
Supportive Care
- Oxygen
- Blood transfusion (if haemorrhage)
- Dialysis (if needed)
- PJP prophylaxis (co-trimoxazole)
Plasma Exchange (PLEX)
- Indicated for severe alveolar haemorrhage
- May consider for dialysis-dependent RPGN
Complications
Of Vasculitis
- End-stage renal disease
- Respiratory failure
- Stroke
- Cardiac involvement
- Death
Of Treatment
- Infection (immunosuppression)
- Infertility (cyclophosphamide)
- Malignancy (long-term)
- Steroid side effects
Prognosis & Outcomes
Prognosis
- Untreated: High mortality (over 80% at 1 year)
- With treatment: 5-year survival over 80%
Relapse
- 50% relapse within 5 years
- GPA relapses more than MPA
- Long-term monitoring essential
Evidence & Guidelines
Key Guidelines
- BSR Guideline on ANCA-Associated Vasculitis
- EULAR/ERA-EDTA Recommendations
Key Evidence
- Rituximab non-inferior to cyclophosphamide for induction (RAVE trial)
- Plasma exchange improves outcomes in severe disease (MEPEX)
Patient & Family Information
What is Vasculitis?
Vasculitis is inflammation of blood vessels. It can affect different organs including the kidneys, lungs, and nerves.
Symptoms
- Feeling very unwell
- Fever, weight loss
- Coughing blood
- Kidney problems
- Rash, skin ulcers
Treatment
- Strong medications to suppress the immune system
- Steroids and other immunosuppressants
- Sometimes plasma exchange
Resources
References
Primary Guidelines
- Ntatsaki E, et al. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology. 2014;53(12):2306-2309. PMID: 24729399
Key Trials
- Stone JH, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis (RAVE trial). N Engl J Med. 2010;363(3):221-232. PMID: 20647199
- Jayne DR, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone (MEPEX). J Am Soc Nephrol. 2007;18(7):2180-2188. PMID: 17582159