Vasculitis
Last updated 21st September 2022. Tom Heaton
Vasculitis refers to inflammation of blood vessels, but it is usually the arteries that are affected.
The term vasculitides refers to the different types of vasculitis.
It is categorised as:
Primary vasculitis is not related to another disease process.
They are traditionally categorised based on the size of the vessels they involve, and hence to resulting features (although there is some overlap).
The term vasculitides refers to the different types of vasculitis.
It is categorised as:
- Primary
- Large vessel
- Medium vessel
- Small vessel
- Large vessel
- Secondary
- Drug
- Infection
- Malignancy
- Other diseases e.g. Lupus, RA
- Drug
Primary vasculitis is not related to another disease process.
They are traditionally categorised based on the size of the vessels they involve, and hence to resulting features (although there is some overlap).
- Large vessel
- Giant cell arteritis
- Takayusu’s arteritis
- Kawasaki disease
- Behçet’s disease
- Giant cell arteritis
- Medium vessel
- Wegener’s vasculitis
- Churg-Strauss syndrome
- Polyarteritis nodosa
- Primary CNS vasculitis
- May include the large vessel conditions
- Wegener’s vasculitis
- Small vessel
- Henoch-Schönlein purpura (IgA vasculitis)
- Cryoglobulinemic vasculitis
- Hypersensitivity vasculitis
- Wegener’s a.k.a. Granulomatosis with polyangiitis (ANCA-associated)
- Churg-Strauss a.k.a. Eosinophilic granulomatosis with polyangiitis (ANCA-associated)
- Microscopic polyangiitis (ANCA-associated)
- Henoch-Schönlein purpura (IgA vasculitis)
Presentation
As the disease affects blood vessels, all organs can be affected.
These tend to run in particular patterns, and again the size of the vessels involved can provide a useful guide to the symptoms and signs that may be expected.
Each type of vasculitis is unlikely to cause all the symptoms described though.
Large Vessel
Medium Vessel
Small Vessel
Some common disease patterns should also make you think about vasculitis as a cause:
Because of the overlap with a number of triggers, and because of the varying forms that it may take, a clear history is vital.
Key features include:
These tend to run in particular patterns, and again the size of the vessels involved can provide a useful guide to the symptoms and signs that may be expected.
Each type of vasculitis is unlikely to cause all the symptoms described though.
Large Vessel
- Skin
- Cyanosis
- Extremity discolouration
- Cyanosis
- GI
- Bowel infarction
- Bowel infarction
- Renal
- Hypertension
- Hypertension
- Muscles
- Claudication
- Claudication
- CNS
- CVAs
- CVAs
Medium Vessel
- Skin
- Erythema nodosum
- Livedo reticularis
- Ulcers
- Distal infarcts
- Erythema nodosum
- GIT
- Pain
- Perforation
- Pain
- Renal
- Haematuria
- RBC casts
- Hypertension
- Haematuria
- Muscles
- Myositis
- Myositis
- CNS
- Mental state changes
- CVA
- Mental state changes
Small Vessel
- Skin
- Palpable purpura
- Splinter haemorrhages
- Urticaria
- Palpable purpura
- GIT
- Mucositis
- Bleeding
- Mucositis
- Renal
- Haematuria
- Haematuria
- Muscles
- Pain
- Pain
- CNS
- Polyneuropathy
- Polyneuropathy
Some common disease patterns should also make you think about vasculitis as a cause:
- Mononeuritis multiplex
- Pulmonary-renal disease
- Palpable purpura
Because of the overlap with a number of triggers, and because of the varying forms that it may take, a clear history is vital.
Key features include:
- Clear duration/timescale of symptoms
- Recent illness
- Recent new medications/vaccinations
- Full systematic review - this should include a detailed systems examination
Investigations
These will be undertaken to:
They will include
Full confirmation may require biopsy of an affected organ.
Angiography may also provide additional diagnostic information.
- Diagnose a vasculitis (and hopefully specific type)
- Identify potential triggers
- Identify mimics/differentials
They will include
- Bloods
- U&Es - assess renal dysfunction
- FBC - plts
- Coagulation
- LFTS
- ANCAs
- ANA
- Cryoglobulins
- Complement levels
- Rheumatoid factor
- U&Es - assess renal dysfunction
- Urine
- Dip and microscopy
- Dip and microscopy
- Imaging
- CXR
- MRI
- CXR
Full confirmation may require biopsy of an affected organ.
Angiography may also provide additional diagnostic information.
Differential Diagnoses
There are a few conditions that can present similarly to a vasculitis:
- DIC
- Infective endocarditis
- Anti-GBM antibody disease (Goodpasture’s disease)
- Buerger’s disease (thromboangiitis obliterans)
- Amyloidosis
Management
The different forms of vasculitis have different treatment regimes.
A general structures approach could be summarised as the following, with increasing management for the increasingly severe forms:
IVIG and plasmapheresis may have a role in severe refractory disease.
There is also an increasing interest in biologic therapy.
Surgical intervention may be required to manage some of the complications of the disease e.g. vascular stenting.
A general structures approach could be summarised as the following, with increasing management for the increasingly severe forms:
- Removal of trigger and observation
- Steroids
- Cytotoxic agents e.g. cyclophosphamide, methotrexate
IVIG and plasmapheresis may have a role in severe refractory disease.
There is also an increasing interest in biologic therapy.
Surgical intervention may be required to manage some of the complications of the disease e.g. vascular stenting.
Complications
These can relate to the end organ effects of the disease, but the medical therapy may also cause complications:
- Renal injury/dysfunction
- Distal ischaemia
- Pulmonary haemorrhage
- CVAs
- VTE
- Subglottic stenosis
Specific Vasculitides
Wegener's Granulomatosis
Now known as granulomatosis with polyangiitis.
This is a necrotising vasculitis of small and medium sized vessels.
The key sites it affects are the upper and lower respiratory tract and kidneys.
Presentation
Assessment
A standard assessment as described above is appropriate initially.
More extensive respiratory assessment is likely to be needed given the likelihood of involvement.
This may include HRCT scan, but also potentially upper airway direct visualisation e.g. nasendoscopy.
Serum ANCA is a useful test in the diagnosis. c-ANCA is both sensitive and fairly specific to the disease (although can be negative in purely respiratory disease).
Biopsy of disease sites (granulomata) in the respiratory or renal tract may be indicated to aid diagnosis.
Management
This will be under the support of the respiratory and renal teams.
Immune modulation is the primary management strategy
Supportive care may be needed in significant cases.
This is a necrotising vasculitis of small and medium sized vessels.
The key sites it affects are the upper and lower respiratory tract and kidneys.
Presentation
- ENT (about 90-100% of patients)
- Nasal congestion
- Epistaxis
- Destructive sinusitis
- Respiratory (about 90% of patients)
- Haemoptysis
- Cough
- Dyspnoea
- Renal (about 80% of patients)
- Haematuria
- Proteinuria
- Progressive deterioration in renal function
- Other
- Fever
- Weight loss
- Vasculitic rash
- Joint inflammation
Assessment
A standard assessment as described above is appropriate initially.
More extensive respiratory assessment is likely to be needed given the likelihood of involvement.
This may include HRCT scan, but also potentially upper airway direct visualisation e.g. nasendoscopy.
Serum ANCA is a useful test in the diagnosis. c-ANCA is both sensitive and fairly specific to the disease (although can be negative in purely respiratory disease).
Biopsy of disease sites (granulomata) in the respiratory or renal tract may be indicated to aid diagnosis.
Management
This will be under the support of the respiratory and renal teams.
Immune modulation is the primary management strategy
- Corticosteroids
- Cyclophosphamide (methotrexate in some cases)
Supportive care may be needed in significant cases.
Microscopic Polyangiitis
This is very similar to Wegner’s and can be difficult to differentiate.
The kidneys are the main organ system affected and the respiratory tract is slightly less commonly involved (30-50%).
Assessment and management is much the same.
The kidneys are the main organ system affected and the respiratory tract is slightly less commonly involved (30-50%).
Assessment and management is much the same.
Kawasaki's Disease
This is an acute, self-limiting vasculitis.
The aetiology is unknown but it usually occurs in young children (6 months - 5 years).
There is an east asian predisposition which would appear to suggest a genetic susceptibility.
There is also seasonal variation which would suggest an infective trigger (or more than one), but none has been identified.
Presentation
The classical clinical presentation is:
It can mimic a general viral infection and be mistaken as such.
There is often general irritability, beyond what would be expected for the fever.
Assessment
There are no specific investigations for this condition and will be based upon the initial presentation.
Management
The mainstay of therapy is aspirin and IVIG.
The focus is to minimise the risk of coronary complications.
Additional immune modulating therapy may be indicated, especially in cases when there is an inadequate response to IVIG.
This may include corticosteroids initially, or anti-TNF therapies.
Complications
A major complication is coronary artery disease with aneurysm.
This can lead to a risk of MI, or ischaemic heart disease.
The risk is high in untreated disease (15-25%) dropping to <5% when treated.
The aetiology is unknown but it usually occurs in young children (6 months - 5 years).
There is an east asian predisposition which would appear to suggest a genetic susceptibility.
There is also seasonal variation which would suggest an infective trigger (or more than one), but none has been identified.
Presentation
The classical clinical presentation is:
- Persistent fever >5 days
- At least 4 of:
- Bilateral bulbar non-exudative conjunctivitis
- Cervical lymphadenopathy - usually unilateral and >1.5cm in diameter
- Change in lips/oral cavity
- Erythema, lip cracking, strawberry tongue, diffuse injection of mucosa
- Change in extremities
- Acute - erythema of palms/soles, oedema
- Subacute - periungual peeling (week 2-3)
- Polymorphous rash
It can mimic a general viral infection and be mistaken as such.
There is often general irritability, beyond what would be expected for the fever.
Assessment
There are no specific investigations for this condition and will be based upon the initial presentation.
Management
The mainstay of therapy is aspirin and IVIG.
The focus is to minimise the risk of coronary complications.
Additional immune modulating therapy may be indicated, especially in cases when there is an inadequate response to IVIG.
This may include corticosteroids initially, or anti-TNF therapies.
Complications
A major complication is coronary artery disease with aneurysm.
This can lead to a risk of MI, or ischaemic heart disease.
The risk is high in untreated disease (15-25%) dropping to <5% when treated.
Links & References
- Hasudungan, A. Vasculitis. Youtube. 2019. https://www.youtube.com/watch?v=D9h6-LPySPI
- Strong Medicine. Vasculitis - an overview. Youtube. 2014. https://www.youtube.com/watch?v=oqRiT6DcwBs
- Knott, L. Vasculitis. Patient.info. 2016. https://patient.info/doctor/vasculitis-pro
- Lin, M. Kawasaki disease. ALIEM. 2012. https://www.aliem.com/paucis-verbis-kawasaki-disease/
- Jarvis, S. Kawasaki Disease. Patient UK. 2020. https://patient.info/doctor/kawasaki-disease-pro
