• Vasculitis is a histological term describing inflammation of the
• Characterized by widespread vasculitis leading to systemic
symptoms and signs, generally requiring treatment with
corticosteroids and/ or immunosuppressive drugs.
• Two main features are helpful in classifying these
– The size of the blood vessels involved
– The presence or absence of anti-neutrophil cytoplasmic antibodies (ANCA) in
PMR & GCA
• Polymyalgia rheumatica (PMR) and giant cell
(temporal) arteritis are systemic illnesses of the
• Both are associated with the finding of a giant cell
arteritis on temporal artery biopsy.
Polymyalgia rheumatica (PMR)
PMR causes a sudden onset of severe pain and stiffness of the
shoulders and neck, and of the hips and lumbar spine.
These symptoms are worse in the morning, lasting from 30
minutes to several hours.
Clinical history is usually diagnostic and the patient is always
over 50 years old.
Approximately one-third of patients develop systemic features
of tiredness, fever, weight loss, depression and occasionally
Investigation of PMR
A raised ESR and/or CRP is a hallmark of this condition.
Serum alkaline phosphatase and γ-glutamyltranspeptidase
may be raised as markers of the acute inflammation.
Anaemia often present.
Temporal artery biopsy shows giant cell arteritis in 10–30% of
cases, but is rarely performed unless GCA is also suspected.
Giant cell arteritis
GCA is inflammatory granulomatous arteritis of large
cerebral arteries which occurs in association with
The patient may have current PMR, a history of
recent PMR, or be on treatment for PMR.
Clinical features of GCA
It is extremely rare under 50 years of age.
Presenting symptoms include:
Tenderness of the scalp or of the temple
Claudication of the jaw when eating
Tenderness and swelling of one or more temporal or occipital arteries.
The most feared manifestation is sudden painless temporary or
permanent loss of vision in one eye due to involvement of the
Systemic manifestations of severe malaise, tiredness and fever
Investigation of GCA
• A temporal artery biopsy from the affected side is the
definitive diagnostic test. This should be taken before, or
within 7 days of starting, high doses of corticosteroids.
• Anemia may be present
• ESR is usually raised and the CRP very high.
• Liver biochemistry: Abnormalities occur, as in PMR
Histological features of GCA
The histological features of GCA are:
Cellular infiltrates of CD4+ T lymphocytes, macrophages and giant cells
in the vessel wall.
Granulomatous inflammation of the intima and media
Breaking up of the internal elastic lamina
Giant cells, lymphocytes and plasma cells in the internal elastic lamina.
Treatment of PMR or GCA
Corticosteroids produce a dramatic reduction of symptoms of
PMR within 24–48 hours of starting treatment, provided the
dose is adequate.
This treatment should reduce the risk of patients who have
PMR developing GCA.
In GCA, corticosteroids are obligatory because they
significantly reduce the risk of irreversible visual loss and
other focal ischaemic lesions, but much higher doses are
needed than in PMR.
Sometimes biopsy is not obtainable, so the treatment should
not be delayed, especially if there have already been episodes
of visual loss or stroke.
PMR: 10–15 mg prednisolone as a single dose in the morning.
GCA: 60–100 mg prednisolone, usually in divided doses.
Most patients will eventually be able to stop corticosteroids
after 12–18 months.
Calcium and vitamin D supplements and sometimes
bisphosphonates are necessary to prevent osteoporosis while
high-dose steroids are being used
This is rare, except in Japan.
It is of unknown aetiology and occurs in females.
There is a vasculitis involving the aortic arch as well as other major arteries.
There is also a systemic illness, with pain and tenderness over the affected
Absent peripheral pulses and hypertension are common.
Corticosteroids help relieve symptoms.
Treatment may require a surgical bypass to improve perfusion of the affected
Eventually heart failure and strokes may occur but most patients survive for at
least 5 years
Polyarteritis nodosa (PAN)
Classical PAN is a rare condition which usually occurs in
its occasional association with hepatitis B antigenaemia
suggests a vasculitis secondary to the deposition of immune
Pathologically, there is fibrinoid necrosis of vessel walls with
microaneurysm formation, thrombosis and infarction.
These include fever, malaise, weight loss and myalgia.
These initial symptoms are followed by dramatic acute
features that are due to organ infarction.
Neurological: mononeuritis multiplex is due to
arteritis of the vasa nervorum.
Abdominal: pain due to arterial involvement of the
abdominal viscera, mimicking acute cholecystitis,
pancreatitis or appendicitis. Gastrointestinal
haemorrhage occurs because of mucosal ulceration.
Renal: presents with haematuria and proteinuria.
Hypertension and acute/chronic kidney disease
Cardiac: coronary arteritis causes myocardial
infarction and heart failure. Pericarditis also
Skin: subcutaneous haemorrhage and gangrene
occur. A persistent livedo reticularis is seen in
chronic cases. Cutaneous and subcutaneous
palpable nodules occur, but are uncommon.
Lung: involvement is rare.
Blood count: Anaemia, leucocytosis and a raised ESR
Angiography: Demonstration of microaneurysms in
hepatic, intestinal or renal vessels if necessary.
Other investigations: as appropriate (e.g. ECG and
abdominal ultrasound), depending on the clinical
problem. ANCA is positive only rarely in classic PAN.
Is with corticosteroids, usually in combination
with immunosuppressive drugs such as
• This is an acute systemic vasculitis involving medium-sized
vessels, affecting mainly children under 5 years of age.
• It is very frequent in Japan, and an infective trigger is
• It occurs worldwide and is also seen in adults.
Fever lasting 5 days or more
Bilateral conjunctival congestion 2–4 days after onset
Dryness and redness of the lips and oral cavity 3 days after onset
Acute cervical lymphadenopathy accompanying the fever
Polymorphic rash involving any part of the body
Redness and oedema of the palms and soles 2–5 days after onset
The persistent fever plus at least 4/5 features should be present to make
the diagnosis, or < 4 if coronary aneurysms can be seen on two
dimensional echocardiography, MRI or angiography.
Cardiovascular changes in the acute stage include pancarditis and
coronary arteritis leading to aneurysms or dilatation.
Anti-endothelial cell autoantibodies are often detectable.
Other features include diarrhoea, albuminuria, aseptic meningitis and
arthralgia and, in most, there is a leucocytosis, thrombocytosis and a
• Is with a single dose of high-dose intravenous
immunoglobulin (2 g/kg), which prevents the
coronary artery disease, followed after the acute
phase by aspirin 200–300 mg daily.
• There is no evidence that steroid treatment improves
It is characterized by lesions involving the upper respiratory tract, lungs and kidneys.
It often starts with severe rhinorrhoea, with subsequent nasal mucosal ulceration
followed by cough, haemoptysis and pleuritic pain.
Etiology is unknown
Single or multiple nodular masses or pneumonic infiltrates with cavitation are seen
on chest X-ray.
The typical histological changes are usually best seen on renal biopsy, which shows
necrotizing microvascular glomerulonephritis.
This disease responds well to treatment with cyclophosphamide 150–200 mg daily.
Rituximab is also being used.
A variant of Wegener’s granulomatosis called ‘midline granuloma’ affects the nose
and paranasal sinuses and is particularly mutilating; it has a poor prognosis.
This condition classically occurs in males in their 4th decade, who present
with rhinitis and asthma, eosinophilia and systemic vasculitis.
The aetiology is uncertain.
Typically, it involves the lungs, peripheral nerves and skin, but renal
involvement is uncommon.
Transient patchy pneumonia-like shadows may occur.
Skin lesions include tender subcutaneous nodules as well as petechial or
ANCA is usually positive.
The disease responds well to corticosteroids.
Microscopic vasculitis (polyangiitis)
This involves the kidneys and the lungs where
it results in recurrent haemoptysis.
ANCA is usually positive.
• This clinical syndrome comprises a characteristic skin rash, abdominal
colic, joint pain and glomerulonephritis.
• Approximately 30–70% have clinical evidence of renal disease with
haematuria and/or proteinuria
• The renal lesion is a focal segmental proliferative glomerulonephritis,
sometimes with mesangial hypercellularity.
• In more severe cases, epithelial crescents may be present.
• Immunoglobulin deposition is mainly IgA in the glomerular mesangium
distribution, similar to IgA nephropathy.
• There is no treatment of proven benefit; steroid therapy is ineffective.
• Treatment is usually supportive but with crescentic GN aggressive
immunosuppression has been tried with variable outcome.
• (LCV) is the most common cutaneous vasculitis affecting small vessels.
• This usually appears on the lower legs as a symmetrical palpable purpura.
• It can be caused by drugs (15%), infection (15%), inflammatory disease
(10%) or malignant disease (<5%) but often no cause is found (55–60%).
• Investigations are only necessary with persistent lesions or associated
signs and symptoms.
• LCV often settles spontaneously, treatment with analgesia, support
stockings, dapsone or prednisolone may be needed to control the pain
and to heal up any ulceration.
• Kumar & Clark's clinical medicine 8th edition