Vasculitis

Question 1

What is vasculitis?

Answer 1

Inflammation of the blood vessels. Often with ischaemia, necrosis and organ involvement.

Question 2

Which blood vessels does vasculitis affect?

Answer 2

Can affect any of them.

Question 3

Why is diagnosing vasculitis difficult?

Answer 3

The clinical manifestations are diverse and can affect any organ/system. Therefore pin pointing this is tricky.

Question 4

Primary vasculitis

Answer 4

Results from an inflammatory response that targets the vessel walls- no known cause.
Sometimes this can be autoimmune.

Question 5

Secondary vasculitis

Answer 5

May be triggered by an infection, a drug, a toxin or may occur as part of another inflammatory disorder or cancer.

Question 6

Pathogenesis of vasculitis

Answer 6

Activated dendritic cells release inflammatory mediators that promote the activation of T cells and cause inflammation, granuloma formation and macrophage activation.

Activated macrophages cause progressive endothelial damage, disruption of the internal elastic lamina and intimal hyperplasia.

Question 7

Classification of vasculitis

Answer 7

International Chapel Hill consensus classification system
Could be large vessel e.g. Giant cell arteritis and Takayasu
Could be medium vessel e.g. Polyarteritis nodosa and Kawasaki disease
Or small vessel which are made up of immune complex associated vasculitis or ANCA associated vasculitis.

Question 8

Large vessel vasculitis

Answer 8

Primary vasculitis causing chronic granulomatous formation primarily affecting the aorta and its major branches.

Question 9

Categories of large cell vasculitis

Answer 9

Giant cell arteritis and takayasu arteritis.

Question 10

Takayasu arteritis aetiology

Answer 10

Generally affects people of less than 40 years old and is much commoner in females. More common in Asian populations.

Question 11

Giant cell arteritis aetiology

Answer 11

Generally affects those over 50.

Question 12

Similarities between Takayasu arteritis and giant cell arteritis

Answer 12

They are both characterised by granulomatous infiltration in the walls of large vessels.

Question 13

Presenting features of large cell vasculitis

Answer 13

Bruit- most commonly in the carotid artery
Blood pressure difference in the extremities
Claudication
Vessel tenderness or carotodynia (unilateral tenderness of the carotid artery)
Hypertension

Question 14

Temporal arteritis association with other diseases

Answer 14

Associated strongly with polymyalgia rheumatica. About 50% of people with GCA have PMR.

Question 15

Temporal arteritis symptoms

Answer 15

Unilateral headache
Scalp tenderness
Jaw claudication
Temporal arteries may be prominent with reduced pulsation. 
Risk of blindness.

Question 16

Investigations into large cell vasculitis

Answer 16

Inflammatory markers (ESR, CRP, Plasma viscocity) usually raised.
If there are symptoms of temporal arteritis- then temporal artery biopsy is needed.
Imaging such as MRI and pet scan may show vessel wall thickening/stenosis/aneurysm

Question 17

Treatment of large cell vasculitis

Answer 17

Steroids- 40-60mg of prednisolone

Steroid sparing agents such as methotrexate could be considered.

Question 18

Kawasaki disease aetiology

Answer 18

Seen in children- usually under 5 years.

Question 19

Where does Kawasaki’s disease affect?

Answer 19

Can affect many vessels but predominantly the coronary arteries. This is where aneurysms can develop.

Question 20

What is polyarteritis nodosa

Answer 20

Necrotising inflammatory lesions that arteries at vessel bifurcations resulting in microaneurysmal formations and aneurysm formations.

Question 21

What is polyarteritis nodosa associated with?

Answer 21

Hepatitis B. It can also affect the skin, gut and kidneys.

Question 22

Divisions in small vessel vasculitis

Answer 22

ANCA positive and ANCA negative.

Question 23

New name for Wegners granulomatosis

Answer 23

Granulomatosis with polyangitis

Question 24

New name for Churg Strauss syndrome

Answer 24

Eosinophillic granulomatosis with polyangitis

Question 25

Pathology of eosinophillic granulomatosis with polyangitis

Answer 25

Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels. Associated with asthma

Question 26

Pathology of granulomatosis with polyangitis

Answer 26

Granulomatous inflammation of respiratory tract, small and medium vessels. Necrotising glomerulonephritis common.

Question 27

Pathology of microscopic polyangitis

Answer 27

Necrotising vasculitis with few immune deposits. Necrotising glomerulonephritis very common

Question 28

Aetiology of granulomatosis with polyangitis (GPA)

Answer 28

GPA is more common in individuals of northern European descent (approximately 90%)
It has a male-to-female ratio of 1.5:1
The onset of GPA may occur at any age, although typically presents at age 35-55 years.
Constitutional symptoms and arthralgia are common.

Question 29

ENT symptoms of GPA

Answer 29

Sinusitis
Nasal crusting
Epistaxis
Mouth ulcers
Sensorineural deafness
Otitis media and deafness
“Saddle nose” due to cartilage ischaemia

Question 30

Respiratory symptoms of GPA

Answer 30

Pulmonary infiltrates 
Cough 
Hemoptysis 
Diffuse alveolar hemorrhage
Cavitating nodules on CXR

Question 31

Cutaneous feature of GPA

Answer 31

Palpable purpura

Cutaneous ulcers

Question 32

Renal features of GPA

Answer 32

Necrotising glomerulonephritis

Manifests as blood and protein in urine

Question 33

Nervous features of GPA

Answer 33

Mononeuritis multiplex
Sensorimotor polyneuropathy
Cranial nerve palsies

Question 34

Ocular features of GPA

Answer 34

Conjunctivitis 
Episcleritis 
Uveitis 
Optic nerve vasculitis 
Retinal artery occlusion  
Proptosis

Question 35

Features of EGPA

Answer 35

The same as GPA however there is late onset asthma and a high eosinophil count.

Question 36

What are ANCA’s?

Answer 36

Anti-neutrophil cytoplasmic antibodies- they are autoantibodies that attack the cytoplasm of neutrophillic granulocytes.

Question 37

How is ANCA detected?

Answer 37

Immunoflourescence.

This can differentiate between pANCA and cANCA.

Question 38

GPA is associated with which type of antibodies?

Answer 38

cANCA and PR3

Question 39

EGPA is associated with which type of antibodies?

Answer 39

pANCA and MPO

Question 40

Management of autoimmune vasculitis

Answer 40

Management depends on degree of involvement.
Localised/early systemic- methotrexate and steroids
Generalised/systemic- Cyclophosphamide + steroids (1st line)
Rituximab + Steroids (alternative)
Plasma exchange if creatinine > 500
Refractory- IV Immunoglobulins
Rituximab

Question 41

Henoch Schonlein Purpura

Answer 41

Acute IgA mediated disorder.
Generalized 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)
Approximately 75% of cases occur in children aged 2-11 years; HSP is rare in infants

Question 42

How does Henoch schonlein purpura develop?

Answer 42

More than 75% of patients have had preceding URTI, pharyngeal infection, or GI infection
Most common is group A streptococcus
Preceding illness usually predates HSP by 1-3 weeks

Question 43

Presentation of HSP

Answer 43

Purpuric rash typically over buttocks and lower limbs
Colicky abdominal pain
Bloody diarrhoea
Joint pain +/- swelling
Renal involvement (50%)

Question 44

Management of HSP

Answer 44

Usually self limiting
Symptoms tend to resolve within 8 weeks.
Relapses may occur for months to years.
Urinalysis needs to be performed to screen for renal involvement.