Vasculitis

Question 1

What is vasculitis - how is it classified?

Answer 1

Vasculitis is defined as inflammation of blood vessels. It may result in vessel wall thickening, stenosis, and occlusion with subsequent ischemia.

The clinical presentation varies according to the histologic type of inflammation, the size of the involved blood vessel segment, and the distribution of the involved vessels.

The classification is according to the International Chapel Hill Consensus.

Question 2

What are the different types of vasculitides?

Answer 2

• large vessel vasculitis
• medium vessel vasculitis
• immune complex small-vessel vasculitis
• ANCA-assoc. small vessel vasculitis

Question 3

What is large vessel vasculitis? What are the two main large vessel vasculitides? how do these differ in age affected?

Answer 3

The term ‘large vessel vasculitis’ applies to primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches

Temporal (giant cell) arteritis and Takayasu arteritis (TA). These arteritides differ in the age of onset, with the former rarely occurring before the age of 50 years and the latter rarely after 50 years.

TA - young women in 20-30’s

Question 4

What are the early features of large vessel vasculitides?

Answer 4

Early features include non specific features such as low-grade fever, malaise, night sweats, weight loss, arthralgia and fatigue. Following this patients can experience claudicant symptoms in both the upper and lower limbs.

Question 5

What can happen if large vessel vasculitis goes untreated?

Answer 5

vascular stenosis and aneursyms can occur. This results in reduced pulses and bruits.

Question 6

What is seen on blood tests in large vessel vasculitis?

Answer 6

ESR, PV and CRP and elevated.

Question 7

What can be seen on imaging in large vessel vasculitis?

Answer 7

Imaging such as MR angiography can detect thickened vessel walls and stenosis.

Question 8

what is the mainstay of treatment of large vessel vasculitis?

Answer 8

The mainstay of treatment is corticosteroids, starting at 40-60mg prednisolone and gradually reducing. Steroid sparing agents such as methotrexate and azathioprine may be added.

Question 9

How can small to medium vessel vasculitis be classified?

Answer 9

This group can be divided into ANCA positive and negative conditions.

Question 10

Small to medium vessel vasculitis:

-what features are similar?

Answer 10

Fever and weight loss

A raised, non blanching purpuric rash

Arthralgia/arthritis

Mononeuritis multiplex

Glomerulonephritis

Lung opacities on x-ray

Question 11

What are the four types of vasculitis assoc. with ANCA?

Answer 11

Granulomatosis with polyangitis (wegners):

• respiratory tract
• necrotising glomerulonephritis common

Microscopic polyangitis:

• small vessels
• necrotising glomerulonephritis
• pulmonary capilaritis often occurs

Renal limited vasculitis:
-pauci-immune necrotising glomerulonephritis

Eosinophilic Granulomatosis with Polyangiitis (Chrug-strauss) syndrome:
-eosinophil rich and granulomatous inflammation involving respiratory tract

Question 12

What symptoms are seen in granulomatosis with polyangitis?

Answer 12

ENT symptoms are common in GPA and include nose bleeds, deafness, recurrent sinusitis and nasal crusting (over time there can be collapse of the nose). Respiratory symptoms such as haemoptysis and cavitating lesions on x-ray are frequently seen.

Question 13

What symptoms are seen in eosinophilic granulomatosis with polyangitis?

Answer 13

This condition is characterised by late onset asthma, rhinitis and a raised peripheral blood eosinophil count.

Neurological symptoms such as mono neuritis multiplex are common.

Question 14

What symptoms are seen with microscopic polyangitis?

Answer 14

The most important complication is glomerulonephritis, which occurs in up to 90% of patients.

Question 15

what is included in the investigation of vasculitis?

Answer 15

ESR, PV and CRP and raised

Anaemia of chronic disease is common.

U+E looking for renal involvement

Anti-neutrophil cytoplasmic antibody (ANCA)

Urinalysis (looking for renal vasculitis)

CXR

Biopsy of an affected area e.g. skin or kidney is often helpful in confirming the diagnosis

Question 16

What is the management of vasculitis?

Answer 16

Most cases of ANCA associated vasculitis require treatment with IV steroids and cyclophosphamide due to their aggressive disease course.

There are some situations e.g. GPA localised to the nose, where other options would be considered.

Question 17

What is henoch-schonlein purpura?

Answer 17

Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalised vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS).

It commonly affects children. Commonly a history of an upper respiratory tract infection predates the symptoms by a few weeks.

Common symptoms are a purpuric rash over the buttocks and lower limbs, abdominal pain and vomiting and joint pain.

Henoch schonlein purpura tends to be a self limiting condition not requiring specific treatment. It usually settles over the course of weeks to months.