VASCULITIS

Question 1

What is vasculitis?

Answer 1

• a histological term describing inflammation of the vessel wall
• can be seen in many diseases including; RA, SLE, polymyositis, and some allergic drug reactions

Question 2

Describe the pathophysiology of vasculitis

Answer 2

Inflammation and necrosis of blood vessel walls with subsequent impaired blood flow resulting in:

• Vessel wall destruction - aneurysm, rupture and stenosis—> perforation and haemorrhage into tissues
• Endothelial injury:–>thrombosis + ischaemia/infarction of dependent tissues

Question 3

How is vasculitis classified?

Answer 3

-By size of blood vessel involved and the presence or absence of antineutrophil cytoplasmic antibodies (ANCA)

Question 4

Give examples of large-vessel vasculitis

Answer 4

Refers to the aorta and its major tributaries

Examples:

• Giant cell arteritis/polymyalgia rheumatic
• Takayasu’s arteritis

Question 5

Give examples of medium-vessel vasculitis

Answer 5

Refers to medium and small-sized arteries and arterioles

Examples:

• Classical polyarteritis nodosa (PAN)
• Kawasaki’s disease

Question 6

Give examples of small-vessel vasculitis

Answer 6

Refers to small arteries, arterioles, VENULES and capillaries

Examples: 
>ANCA-associated: 
-Microscopic polyangitis 
-Granulomatosis with polyangitis
>ANCA-negative: 
-Essential cryoglobulinaemia 
-Cutaneous leucocytoclastic vasculitis

Question 7

What size vasculitis tends to be ANCA positive?

Answer 7

Small-vessel vasculitis e.g microscopic polyangitis and granulomatosis with polyangitis

Question 8

What are all vasculitis associated with?

Answer 8

-All associated with anaemia and a raised ESR

pAre ALL RARE except giant cell (temporal) arteritis

Question 9

What is PMR?

Answer 9

Systemic disease of the elderly, the pathogenesis is unknown (Large cell vasculitis)

Question 10

What are the risk factors of PMR

Answer 10

• SLE
• Polymyositis/dermatomyositis
• age>50
• female

Question 11

What is the epidemiology of PMR?

Answer 11

• Affects those over 50 yrs

- More common in FEMALES than males

Question 12

What are the signs/symptoms of PMR?

Answer 12

S-udden onset of severe pain and stiffness of the shoulders and neck, and of the hips and lumbar spine; a limb girdle pattern

• worse in the morning, lasting from 30 mins - several hours
• Mild polyarthritis of peripheral joints
• 1/3rd experience; fatigue, fever, weight loss, depression

Question 13

What are the investigations for PMR?

Answer 13

• Clinical history is usually diagnostic and the patient is ALWAYS OVER 50
• Bloods; ESR & CRP raised, ANCA negative, ALP raised, mild normochromic, normocytic anaemia may be present
• Temporal artery biopsy: Shows giant cell arteritis in 10-30% cases
• Note: creatinine kinase is normal - helps to distinguish from myositis/ myopathies

Question 14

What is the treatment and management of PMR?

Answer 14

• Corticosteroids; produce a dramatic reduction of symptoms of PMR within 24-48 hours of starting treatment e.g. ORAL PREDNISOLONE
• If improvement does not occur then diagnosis should be questioned
• Decrease dose slowly
• Used long-term so give GI and bone protection (to prevent osteoporosis due to steroid use) e.g. LANSOPRAZOLE and ALENDRONATE and Ca2+ and vitamin D

Question 15

What is GCA?

Answer 15

Inflammatory granulomatous arteritis of large Cerebral Arteries as well as other large vessels e.g aorta, which occurs in association with PMR (Large cell vasculitis)

Question 16

Describe the pathophysiology of GCA?

Answer 16

• Arteries become inflamed, thickened and can obstruct blood flow
• Cerebral arteries affected in particular e.g. temporal artery
• Opthalmic artery can also be affected potentially resulting in permanent or temporary vision loss

Question 17

What are the risk factors of GCA?

Answer 17

• Over 50
• Female
• RA, SLE, scleroderma

Question 18

Describe the epidemiology of GCA

Answer 18

• Primarily in those OVER 50
• Incidence increases with age
• More common in FEMALES than males

Question 19

What are the signs of GCA?

Answer 19

• Tenderness and swelling of one or more temporal or occipital arteries
• SUDDEN PAINLESS VISION LOSS - EMERGENCY - Arteritic anterior ischaemic optic neuropathy - optic disc is very pale/swollen

Question 20

What are the symptoms of GCA?

Answer 20

• Severe headaches (temporal pulsating)
• Tenderness of scalp (combing hair can be painful) or temple
• Claudication of the jaw (pain in jaw) when eating
• Malaise, lethargy, fever

Question 21

WHat are the investigations for GCA?

Answer 21

• Temporal artery biopsy; DEFINITIVE DIAGNOSTIC TEST - Should be taken BEFORE or within 7 days of starting high dose corticosteroids
• Bloods; CRP&ESR high, ANCA negative, ALP may be raised, Normochromic, normocytic anaemia

Question 22

What is the diagnostic criteria of GCA?

Answer 22

• Over 50
• New headache
• Temporal artery tenderness or decreased pulsation
• ESR raised
• Abnormal artery biopsy - inflammatory infiltrates present

Question 23

What is the treatment of GCA?

Answer 23

• HIGH DOSE CORTICOSTEROIDS RAPIDLY e.g. PREDNISOLONE to stop vision loss - gradual reduction of steroids over 12-18 months
• Used long-term so give GI and bone protection (to prevent osteoporosis due to steroid use) e.g. LANSOPRAZOLE and ALENDRONATE and Ca2+ and vitamin D
• Monitor treatment progress by looking at ESR/CRP (should fall)

Question 24

What is polyarteritis nodosa(PAN)?

Answer 24

• Necrotising vasculitis that causes aneurysms and thrombosis in medium sized arteries, leading to infarction in affected organs
• Has severe systemic symptoms

Question 25

What are the risk factors for PAN?

Answer 25

- Male - Hep B - RA, SLE, scleroderma

Question 26

What is the epidemiology of PAN?

Answer 26

- Rare in the UK - Usual occurs in middle-aged men - More common in MALES than females - Associated with Hep B

Question 27

What are the initial symptoms of PAN?

Answer 27

Fever, malaise, weight loss and myalgia these initial symptoms are followed by dramatic acute features that are due to organ infarction

Question 28

What are neurological symtoms of PAN?

Answer 28

-Mononeuritis multiplex due to arteritis of the vasa nervorum - Numbness, tingling, abnormal/lack of sensation and inability to move part of the body

Question 29

What are the abdominal symptoms of PAN?

Answer 29

- Pain due to arterial involvement of the abdominal viscera, mimicking acute cholecystitis, pancreatitis or appendicitis - GI haemorrhage due to mucosal ulceration

Question 30

What are the renal symptoms of PAN?

Answer 30

- Presentation is with haematuria and proteinuria | - Hypertension and acute/chronic kidney disease occur

Question 31

What are the cardiac and skin symptoms of PAN?

Answer 31

Cardiac: Coronary arteritis causes myocardial infarction and heart failure Skin: Subcutaneous haemorrhage and gangrene occur

Question 32

What are the investigations for PAN?

Answer 32

- Bloods; Anaemia, WCC raised, ECR raised, ANCA negative - Biopsy, of kidney in particular to look for hypertension and other damage - can be diagnostic - Angiography - Demonstrates micro-aneurysms in heaptic, intestinal or renal vessels

Question 33

What is the treatment of PAN?

Answer 33

- CONTROL BLOOD PRESSURE - ACE-inhibitors e.g. RAMIPRIL - Corticosteroids e.g. PREDNISOLONE in combination with immunosuppressive drugs e.g. AZATHIOPRINE or CYCLOPHOSPHAMIDE - Hep B should be treated with an antiviral after initial treatment with steroids