Vasculitis

Question 1

What is vasculitis?

Answer 1

Inflammation of the blood vessel walls which can be seen in many diseases including RA, SLE, polymyositis and some allergic drug reactions

Question 2

What are the common features of all systemic vasculitides?

Answer 2

Anaemia

Raised ESR

Question 3

How are the systemic vasculitides classifed?

Answer 3

According to the size of vessel affected

Question 4

What are the large systemic vasculitides? Which vessels do these affect?

Answer 4

Giant cell arteritis
Takayasu’s arteritis

Affect the aorta and its main branches

Question 5

What are the medium vasculitides? Which vessels do these affect?

Answer 5

Polyarteritis nodosa
Kawasaki’s disease

Affect the main visceral vessels e.g. renal, coronary

Question 6

What are the small vasculitides? Which vessels do these affect?

Answer 6

• ANCA positive vasculitis: tends to affect the respiratory tract and kidneys. Includes Microscopic polyangiitis, Wegener’s granulomatosis and Churg-Strauss syndrome
• ANCA negative vasculitis: includes Henoch-Schonlein purpura, Goodpasture’s syndrome and cryoglobulinaemia

Question 7

How does vasculitis present?

Answer 7

Different vasculitides preferentially affect different organs, causing different patterns of symptoms. Often presentation is only of extreme fatigue with raised ESR/CRP.

N.B. consider vasculitis in any unidentified multisystem disorder

Question 8

What are the systemic features of vasculitis?

Answer 8

Fever
Malaise
Weight loss
Arthralgia
Myalgia

Question 9

What are the skin features of vasculitis?

Answer 9

Purpura
Ulvers
Livedo reticularis
Nailbed infarcts
Digital gangrene

Question 10

What is livedo reticularis?

Answer 10

Pink-blue mottling caused by capillary dilatation and stasis in skin venules.

Cause may be physiological e.g. cold or vasculitis

Question 11

What general tests might indicate a vasculitis?

Answer 11

Raised ESR/CRP
ANCA may be positive
Raised creatinine if there is renal failure

Question 12

What is polymyalgia rheumatica (PMR)?

Answer 12

It is not a true vasculitis but it shares the same demographic characteristics as GCA and, although two separate conditions, the two often occur together

Question 13

What are the clinical features of PMR?

Answer 13

Age >50 years
Subacute onset (<2 weeks) of bilateral aching
Tenderness and morning stiffness in shoulders, neck, hip and lumbar spine
Muscle weakness is NOT a sign
May be mild polyarthritis, tenosynovitis and carpal tunnel syndrome
May be associated fatigue, fever, weight loss, anorexia and depression

Question 14

What results might be seen on investigation of a patient with PMR?

Answer 14

Raised CRP
ESR is typically >40 but may not be raised
ALP is increased in 30%

Question 15

How is PMR distinguised from myositis/myopathies?

Answer 15

Creatinine kinase levels are normal

Question 16

How is PMR managed?

Answer 16

Prednisolone PO
Expect dramatic response within 1 week and consider alternative diagnosis if not
Reduce dose slowly e.g. by 1mg/month according to symptoms and ESR
Investigate apparent ‘flares’ during the withdrawal
Most need steroids for ≥2 years so give gastric and bone protection

Question 17

What is giant cell arteritis (GCA)?

Answer 17

Giant cell arteritis (GCA) is a systemic immune-mediated vasculitis affecting medium-sized and large-sized arteries, particularly the aorta and its extracranial branches. Arteritic involvement is most commonly noticed in the superficial temporal arteries

The clinical connections between polymyalgia rheumatica (PMR) and GCA have suggested that they are different manifestations of the same disease process

Question 18

What are the symptoms of GCA?

Answer 18

Headache
Tenderness over the scalp or temple (often noticed when combing hair)
Claudication of the jaw when eating

Question 19

What are the complications of GCA?

Answer 19

• GCA affecting the vertebrobasilar and sometimes carotid circulation may result in stroke
• GCA involving the opthalmic artery may cause sudden loss of vision or amaurosis fugax.

Question 20

How should you act if you suspect GCA?

Answer 20

Do ESR

Start prednisolone 60mg PO immediately to reduce risk of irreversible bilateral blindness

Question 21

What results might be seen on investigation of a patient with GCA?

Answer 21

Very high ESR and CRP
Often a normochromic, normocytic anaemia
Temporal artery biopsy is performed if GCA is suspected, within a week of started steroids

Question 22

How is GCA managed?

Answer 22

Prednisolone 60mg daily at first and then gradually reduced by around 5mg a week. Once 10mg dose is reached a reduction of 1mg every 2-4 weeks is sufficient. Dose is titrated against symptoms and ESR. Prophylaxis against steroid-induced osteoporosis should be given.

Question 23

What is the prognosis in GCA?

Answer 23

Typically a 2 year course of steroids then complete remission. Main cause of death and morbidity is long-term steroid treatment so must consider risk vs benefit and give long term gastric and bone protection

Question 24

What is the gastric and bone protection given in conjunction with corticosteroids in GCA?

Answer 24

Gastric: PPI
Bone: Bisphosphonate

Question 25

What is Takayasu;s arteritis?

Answer 25

A vasculitis involving the aortic arch and other major arteries causing hypertension, absent peripheral pulses, strokes and cardiac failure. It is very rare except in Japan but should be considered in patients presenting with features of CGA who are under 55 years old. Treatment is with corticosteroids

Question 26

What is polyarteritis nodosa? (PAN)

Answer 26

A necrotizing vascultis that causes aneurysms and thrombosis in medium sized arteries leading to infarction in affected organs

Question 27

What demographic is most commonly affected by PAN?

Answer 27

Middle aged men

Question 28

What infection is PAN associated with?

Answer 28

Hepatitis B

Question 29

What is the paediatric variant of PAN?

Answer 29

Kawasaki’s syndrome

Question 30

What are the clinical features of PAN?

Answer 30

Fever
Malaise
Weight loss
Mononeuritis multiplex
Abdomial pain (resulting from visceral infarcts)
Renal impairment
Hypertension
Lungs are rarely involved

Question 31

How is PAN diagnosed?

Answer 31

Angiography- microaneurysms in the hepatic, renal or intestinal vessels
OR
biopsy of an affected organ, often the kidney

WCC is often increased
ESR and CRP are increased
ANCA negative
Anaemia

Question 32

How is PAN treated?

Answer 32

Control BP meticulously

Control PAN with corticosteroid usually in combination with an immunosuppressive drug- cyclophosphamide

Question 33

What is microscopic polyangiitis?

Answer 33

A necrotising vasculitis affecting small- and medium-size vessels

Question 34

What are the symptoms of microscopic polyangiitis?

Answer 34

Mainly affects lungs and kidneys
Causes:
-Haemoptysis
-Haematuria
-Proteinuria
-Progressive renal failure

Other features include:

• Arthralgia
• Purpuric rash

Question 35

How is microscopic polyangiitis diagnosed?

Answer 35

Renal biopsy

Measurement of serum perinuclear-ANCA

Question 36

How is microscopic polyangiitis treated?

Answer 36

Corticosteroid + cyclophosphamide

Question 37

What is ANCA?

Answer 37

Anti-neutrophil cytoplasmic antibodies- a group of autoantibodies, mainly IgG, against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell) and monocytes. They are particularly associated with systemic vasculitis, so called ANCA-associated vasculitides.