Introduction

Vasculitis is a broad category that refers to inflammation in blood vessels. It is frequently brought on by the body’s immune system, but the reason why the body decides to attack its own blood vessels is not known.

There are many conditions that can lead to vasculitis. Rheumatologists are the medical specialty that diagnose and manage vasculitis, which often medicine that blocks immune system overactivity

Vascular surgeons are involved when the arteries develop problems that require surgical correction, or when the diagnosis of vasculitis requires a sample of an artery to be looked at under a microscope.

Different forms of vasculitis

Vascular surgeons generally divide vasculitis into three kinds

1. Large vessel vasculitis
2. Medium sized vessel vasculitis
3. Small vessel vasculitis

Some kinds of vasculitis can affect all three sizes.

Presentation features of vasculitis

Large vessel vasculitis

• Limb claudication (painful calves when walking a particular distance, due to lack of oxygenated blood supply to that limb)
• Bruits (noisy blood flow through a narrowing in the artery when listening with a stethoscope)
• Asymmetrical blood pressures (due to narrowing of arteries to the arm)
• Absence of pulses (due to narrowing of arteries to the arm)

Medium sized vessel vasculitis

• Cutaneous nodules and ulcers (identified on the skin)
• Livedo reticularis – a purple star-like fanning of skin colour (see picture)
• Digital gangrene (black toes)
• Mononeuritis multiplex
• Renovascular hypertension

Small vessel vasculitis

• Palpable purpura (Red, raised blotches, sometimes with mixed-in brown and grey colours
• Pulmonary-renal syndromes (issues with the lungs and kidneys due to damage to the small vessels within these organs)
• Urticarial skin rashes (itchy skin)
• Scleritis (red, inflamed vessels in the white of the eye)
• Damage to the toes from lack of blood, sometimes resulting in gangrene

Large vessel vasculitis

The large vessel vasculitis tends to affect the big artery that comes off of the heart, the aorta. The most common kind of large vessel vasculitis is giant cell arteritis, or temporal arteritis. Diagnosis of this condition is achieved through:

1. Blood tests
2. Temporal artery ultrasound
3. Nuclear medicine (PET) scan
4. Biopsy of the temporal artery

Symptoms of temporal arteritis (giant cell arteritis)

• Headache
• Pain in the jaw, particularly when chewing
• Vision changes, particularly double vision
• Fevers

Temporal artery biopsy

Biopsy is typically performed in the operating theatre, under either general or local anaesthetic. The artery is marked using an ultrasound and a cut is made behind the hairline, in front of and above the ear. A segment of artery is removed and it is looked at under a microscopy. This is the only way to be sure if you have this condition.

Your doctor will often commence treatment with steroids before the biopsy is done. This is to prevent the condition from causing permanent damage, such as blindness. To ensure it is safe to come off the steroids, you will need a biopsy proving there is no inflammation affecting the artery.

Small vessel vasculitis

There are multiple named conditions that are considered small vessel vasculitis. They fall into two broad categories, based on the presence of a particular antibody*

*Note: ‘antibody’ are immune molecules that your body produces, which are the body’s immune soldiers that wage war against whatever they think is the enemy)

Categories of small vessel vasculitis and the named conditions that fall into each include:

1. Anti-neutrophil cytoplasmic antibody (ANCA) positive
   a. Granulomatosis with polyangiitis (aka Wegener’s granulomatosis)
   b. Microscopic polyangiitis (MPA)
   c. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
2. Anti-neutropgil cytoplasmic antibody (ANCA) negative
   a. Cyoglobulinaemic vasculitis
   b. IgA vasculitis (Henoch-Schonlein purpura)
   c. Hypocomplement urticarial vasculitis (anti-C1q vasculitis)

What does small vessel vasculitis do to the body?

Small vessel vasculitis, as the name suggests, affect small blood vessels. This demonstrates itself most commonly in three locations

1. The lungs – The small vessels that are responsible for gas exchange in the lungs are affected.
2. The kidneys – The small vessels that are responsible for filtering blood in the kidneys are affected
3. The toes – The small vessels in the toes which supply the skin of the toes with blood are affected, causing ulcers and gangrene (death of the tissue)

Conclusions

Vasculitis is a serious medical condition, with many different types and effects on the body. It needs to be managed by a general practitioner in combination with a rheumatologist, sometimes a kidney specialist, sometimes a lung specialist and sometimes a vascular surgeon.