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:
Other diseases e.g. Lupus, RA
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).
Giant cell arteritis
Primary CNS vasculitis
May include the large vessel conditions
Henoch-Schönlein purpura (IgA 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)
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.
There may also be dissections or aneurysms that develop.
Mental state changes
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:
Clear duration/timescale of symptoms
Recent new medications/vaccinations
Full systematic review - this should include a detailed systems examination
These will be undertaken to:
Diagnose a vasculitis (and hopefully specific type)
Identify potential triggers
They will include
U&Es - assess renal dysfunction
FBC - plts
Dip and microscopy
Full confirmation may require biopsy of an affected organ. Angiography may also provide additional diagnostic information.
There are a few conditions that can present similarly to a vasculitis:
Anti-GBM antibody disease (Goodpasture’s disease)
Buerger’s disease (thromboangiitis obliterans)
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:
Removal of trigger and observation
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.
These can relate to the end organ effects of the disease, but the medical therapy may also cause complications:
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.
ENT (about 90-100% of patients)
Respiratory (about 90% of patients)
Renal (about 80% of patients)
Progressive deterioration in renal function
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
Cyclophosphamide (methotrexate in some cases)
In severe and rapidly progressing cases, plasmapheresis/plasma exchange or high dose corticosteroids (methylpred) may be needed.
Supportive care may be needed in significant cases.
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.
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:
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)
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.