Vasculitis

This is a term used to describe inflammation of the blood vessel walls.

It leads to generalised inflammatory symptoms (fever, fatigue, weight loss and myalgia) as well as symptoms according to which organ or tissue is affected secondary to the regional ischaemia.

Large Vessel Vasculitis

These conditions involve inflammation of the large arteries like the aorta and its major branches.

Temporal (giant cell) Arteritis

This is a granulomatous vasculitis involving branches of the carotid artery.

This leads to patches of inflammation which “skips” parts of the affected artery.

It is the most common vasculitis in adults older than the age of 60 and is more prevalent in females. 

Symptoms

Headache, over the temple area

Scalp tenderness

Jaw claudication

Inflammatory symptoms (lethargy, fever, anorexia, night sweats)

Risk of irreversible vision loss

Complications include aortic aneurysms and large vessel stenoses

Key tests

Blood tests show raised inflammatory markers (ESR and CRP)

Rapid-access vascular ultrasonography of the temporal and axillary artery

Temporal artery biopsy is diagnostic but can still be negative in the disease

Creatinine kinase (CK) and EMG are normal as it is not a muscular disorder

Management

High dose prednisolone – this is started while awaiting confirmation of diagnosis

If visual symptoms are present, this needs an urgent referral to ophthalmol

Takayasu Arteritis

This is a granulomatous vasculitis involving the branch points of the aortic arch.

It presents in younger adults such as young Asian females

It is called the “pulseless disease” and the vascular inflammation can cause stenosis, occlusion and aneurysm formation.

Symptoms

Unequal blood pressure in the upper limbs

Weak/absent pulse in the upper extremities

Abnormal flow produces a carotid bruit with intermittent claudication

Can also lead to renal artery stenosis

Inflammatory symptoms – fever, weight loss, malaise, lethargy

Key tests

Blood tests show raised inflammatory markers (CRP and ESR)

CT/MRA angiography is diagnostic – shows segmental narrowing or occlusion

Management

Steroids

Medium Vessel Vasculitis

These conditions involve the medium sized muscular arteries that supply organs

Polyarteritis Nodosa

This is a necrotizing vasculitis that involves multiple organs. It leads to aneurysm and thrombus formation in arteries causing infarction of the affected organs and tissues.

It is usually seen in middle-aged men and associated with hepatitis B infection.

Symptoms

Inflammation – fever, weight loss, anorexia, arthralgia

Haematuria, renal failure (due to renal artery involvement)

Motor weakness and sensory loss in peripheral nerves

Mononeuritis multiplex, hypertension

Livedo reticularis on legs

Key tests

Blood tests show raised inflammatory markers (WCC, ESR, CRP)

Check for hepatitis B serology

Renal artery biopsy or angiography – shows small aneurysms strung like beads

Management

For HBV related PAN, usually high dose steroids followed by combination of antiviral therapy and plasma exchange.

For non-HBV related PAN, treatment involves immunosuppression e.g., steroids and/ or cyclophosphamide

Kawasaki Disease

This is a vasculitis classically affecting children around the age of 3

Symptoms

Fever which lasts  > 5 days which is not responsive to antipyretics

Conjunctivitis and large lymph nodes

Red lips and strawberry tongue

Erythema of palms and soles which later peel

Key tests

It is a clinical diagnosis and there is no specific diagnostic test.

Management

High dose aspirin (usually not given to children but exception made in this disease)

IV Immunoglobin (IVIG)

N.B. One of the biggest complications is a coronary artery aneurysm. Therefore, children should be given a screening echocardiogram to check for this.

Buerger’s Disease (thromboangiitis obliterans)

This is a necrotizing, non-atherosclerotic vasculitis which is associated with smoking.

It causes inflammation of the blood vessels supplying the fingers and toes which can lead to ischaemia of the tissue and necrosis.

Symptoms

Ulceration, gangrene and autoamputation of the fingers and toes

Superficial thrombophlebitis 

Management

Smoking cessation is first-line.

Patients may also require surgical debridement of any gangrenous tissue

ANCA-associated Vasculitis

This is an umbrella term which encompasses a group of multi-system autoimmune small vessel vasculitides. It is very rare and can be seen in patients at any age.

The conditions are characterised by the formation of granulomas and inflammation of small vessels, causing them to rupture (causing alveolar haemorrhage) or become occluded (causing infarction), resulting in a variety of clinical symptoms.

The most common areas affected are the lungs, kidneys and peripheral nerves.

Microscopic Polyangiitis

A necrotizing vasculitis without evidence of necrotizing granulomatous inflammation.

It is associated with p-ANCA antibodies with MPO specificity

Symptoms

Rapidly progressing glomerulonephritis – gives hematuria and hypertension

Pulmonary haemorrhage – leads to hemoptysis, lung infiltrates, cough

 Inflammatory symptoms –> Lethargy, weight loss, fever

Key tests

Positive for pANCA antibodies with MPO specificity

Management

Corticosteroids and Cyclophosphamide (but relapses are common)

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

A necrotizing vasculitis characterised by granuloma formation in organs including the kidney, lung and nasopharynx, associated with cANCA directed towards PR3.

In addition to renal involvement, it classically causes nasal symptoms including nosebleeds, stuffy nose and crustiness of nasal secretions.

Symptoms

Nasopharyngeal – epistaxis, sinusitis, nasal crusting + saddle-shape nose deformity

Respiratory tract – dyspnea, haemoptysis

Kidney – Rapidly progressive glomerulonephritis

Vasculitis rash

Key tests

Serology – associated with raised levels of cANCA

CXR shows cavitating lesions

Renal biopsy shows epithelial crescents in Bowman’s capsule

Management

Immunosuppression

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

This is a rare necrotizing vasculitis seen in patients with a history of allergic airway hypersensitivity, characterised by a raised eosinophil count.

It gives a triad of adult-onset asthma, peripheral eosinophilia and paranasal sinusitis.

It is associated with p-ANCA antibodies directed against MPO.

Symptoms

Triad of adult onset Asthma, peripheral eosinophilia and paranasal sinusitis

Unlike Wegener’s, there is less involvement of the kidneys and nasopharynx.

The key is the raised eosinophilia which gives more asthma-type symptoms

Key tests

Associated with raised levels of pANCA

Management

Steroids and immunosuppression