Vasculitis

Question 1

define vasculitis

Answer 1

• Vasculitis is inflammation of the blood vessels and there are different types which affect the different size of blood vessels.
• Categorized based on small vessels, medium vessels or large vessels.

Question 2

what are the 4 types of small vessel vasculitis ?

Answer 2

1) Henoch-Schonlein purpura
2) Eosinophilic granulomatosis with polyangiitis (Churg-Struass syndrome)
3) Microscopic polyangiitis
4) Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Question 3

what are the 3 types of medium vessel vasculitis?

Answer 3

1) Polyarteritis nodosa
2) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
3) Kawasaki Disease

Question 4

what are the 2 types of large vessel vasculitis?

Answer 4

Large vessel vasculitis

1) Giant cell arteritis
2) Takayasu’s arteritis

Question 5

what are the generic features you might see in a vasculitis?

Answer 5

1) Purpura
2) Joint and muscle pain
3) Peripheral neuropathy
4) Renal impairment
5) GI sx
6) Anterior uveitis and scleritis
7) HTN

Question 6

what are the systemic sx you might see in vasculitis ?

Answer 6

Systemic manifestations

1) Fatigue
2) Weight loss
3) Fever
4) Anorexia
5) Anaemia

Question 7

what investigations would you do in vasculitis?

Answer 7

1) Inflammatory markers (CRP and ESR raised in vasculitis)

2) Anti neutrophil cytoplasmic antibodies (ANCA)

Question 8

what are the 2 types of ANCA?

Answer 8

• Two types of ANCA tests: p-ANCA( aka anti-MPO) and c-ANCA (aka anti-PR3).
• P-ANCA = microscopic polyangiitis and Churg-Strauss
• C-ANCA = Wegener’s granulomatosis

Question 9

what is henoch-schonlein purpura?

Answer 9

• This is an IgA vasculitis that presents with a purpuric rash affecting the lower limbs or buttocks in children.
• Inflammation due to IgA deposits in blood vessels of affected organs such as skin, kidneys and GI tract.
• Usually triggered by upper aiway infection or GE.
• Common in children < 10 years.
• Rash caused by inflammation and leaking of blood vessels under skin  purpura.

Question 10

4 classic features of henoch-schonlein purpura?

Answer 10

1) Purpura
2) Joint pain
3) Abdominal pain
4) Renal involvement – can cause an IgA nephritis

Question 11

how do you manage henoch-schonlein purpura?

Answer 11

Management

1) Analgesia, rest and fluids – supportive.
2) Usually recover within 4-6 weeks.
3) 1/3 patients have a recurrence within 6 months.
4) 1% go on to develop end stage renal failure.

Question 12

what is eosinophilic granulomatosis with polyangiitis (Churg-Strauss) ?

Answer 12

• Small and medium vessel vasculitis
• Associated with lung and skin problems but can also affect kidneys.
• Presents with severe asthma in late teen/adulthood.

Question 13

what would you see on FBC in pt with Churg-Strauss?

Answer 13

• Characteristic finding: elevated eosinophils on FBC.

Question 14

what is microscopic polyangiitis?

Answer 14

• Small vessel vasculitis
• Main feature is renal failure
• Can affect lungs  SOB and haemoptysis

Question 15

what is granulomatosis with polyangiitis (Wegener’s polyangiitis) ?

Answer 15

• Small vessel vasculitis affecting the respiratory tract and kidneys.

Question 16

what sort of systems might be affected in granulomatosis with polyangiitis?

Answer 16

• Ears  hearing loss
• Sinuses  sinusitis
• Saddle shaped nose due to a perforated nasal septum  dip halfway down the nose.
• Lungs  cough, wheeze, haemoptysis, CXR might show consolidation (not to be confused with pneumonia).
• Kidneys  glomerulonephritis.

Question 17

what kind of vasculitis is polyarteritis nodosa?

Answer 17

• Medium vessel vasculitis
• Associated with hep B but can occur without clear cause or with hep C/ HIV.
• Affects vessels of the skin, GI tract, kidneys, heart  renal impairment, MI and stroke.
• Can also cause rash known as livedo reticularis

Question 18

what is kawasaki’s disease, who does it affect?

Answer 18

• Medium vessel vasculitis
• Affects young children <5 years
• No clear cause

Question 19

clinical features of Kawasaki’s ?

Answer 19

Clinical features

1) Persistent fever > 5 days
2) Erythematous rash
3) Bilateral conjunctivitis
4) Erythema and desquamation of palms and soles
5) Strawberry tongue

Question 20

what is the most significant complication of Kawasaki’s ?

Answer 20

LEADS TO CORONARY ARTERY ANEURYSMS

Question 21

how to manage kawasaki?

Answer 21

1) Aspirin

2) IV immunoglobulin

Question 22

what type of vasculitis is Takayasu?

Answer 22

• Large vessel vasculitis
• Affects aorta and its branches and pulmonary arteries.
• Can form aneurysms, become narrowed and blocked  aka called pulseless disease.

Question 23

how do takayasu patients present?

Answer 23

• Usually present before 40 years with non-specific sx: fever, malaise, muscle aches, syncope or arm claudication.

Question 24

what Ix would you use for takayasu’s arteritis?

Answer 24

CT, MRI, U/S for carotid disease.