Vasculitides

Question 1

What happens in polymyalgia rheumatica?

Answer 1

Usually following an infection (parvovirus/adenovirus)

Inflammatory reaction around the soft tissue surrounding joints (bursae and tendons)

Question 2

What does histology show on polymyalgia rheumatica?

Answer 2

Vasculitis with giant cells
‘Skips’ certain areas of the arteries

Muscle bed arteries most affected

Question 3

What are the features of polymyalgia rheumatica?

Answer 3

Usually women >60 years
Rapid onset
Aching, morning stiffness in proximal muscles

Anorexia, fever (due to IL1&6 deposition in brain), depression, polyarthritis, carpal tunnel syndrome, tenosynovitis

Question 4

What are the investigations for polymyalgia rheumatica?

Answer 4

Raised CRP&ESR
ALP raised in 30%
Reduced CD8+ cells

CK levels are normal (can distinguish between myositis/myopathies)

Question 5

What is the management of polymyalgia rheumatica?

Answer 5

Prednisolone 15mg PO daily
Should see a dramatic response in one week

Most need steroids for >2 years so give bone protection

Question 6

What criteria is used for the vasculitides?

Answer 6

Chapel Hill criteria

Classified according to the size of blood vessels affected

Question 7

What conditions fall into the ‘large’ category of vasculitides?

Answer 7

Giant cell arteritis

Takayusu’s arteritis

Question 8

What conditions fall into the ‘medium’ category of vasculitides?

Answer 8

Polyarteritis nodosa

Kawasaki disease

Question 9

What conditions fall into the ‘small’ category of vasculitides?

Answer 9

ANCA associated
Microscopic polyangiitis
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)
Immune complex vasculitis
Goodpasture's disease
IgA vascultis (Henoch-Schonlein)

Question 10

What are the two variable vessel vasculitides?

Answer 10

Behcet’s

Cogan’s syndrome

Question 11

What are the main symptoms/signs of the vasculitides?

Answer 11

Extreme fatigue, raised ESR/CRP
ANCA may be positive
May be proteinuria, haematuria, renal casts

Question 12

What is the management of large vessel vasculitides?

Answer 12

Steroids in most cases

Question 13

What is the management of medium/small vessel vasculitides?

Answer 13

Immunosuppression (steroids and cyclophosphamide or methotrexate)

Question 14

What happens in temporal arteritis?

Answer 14

Also called giant cell arteritis

Common in the elderly. Consider Takayasu’s if <55

Symptoms: Headache, temporal artery and scalp tenderness, tongue/jaw claudication, amarousis fugax/sudden unilateral blindness

Malaise, weight loss, morning stiffness

Question 15

What is a complication of temporal arteritis?

Answer 15

Irreversible bilateral visual loss

Question 16

What do tests show in temporal arteritis?

Answer 16

Raised ESR & CRP
Raised platelets
Raised ALP
Decreased Hb

Do a temporal artery biopsy within 14 days of starting steroids or FDG-PET

Question 17

What is the management of temporal arteritis?

Answer 17

Start prednisolone 60mg/d PO immediately or methylprednisolone if evolving visual loss

Give PPI, bisphosphonate, calcium with cholecalciferol and consider aspirin

Question 18

What happens in polyarteritis nodosa?

Answer 18

Necrotising vasculitis that causes aneurysms and thrombosis in medium sized arteries

Question 19

What are the symptoms/signs of polyarteritis nodosa?

Answer 19

Skin (rash, punched out ulcers)
Renal (main cause of death, renal artery narrowing, RAS)

Coronary aneurysms occur in Kawasaki disease

Question 20

What do tests show in polyarteritis nodosa?

Answer 20

Increased WCC
Mild eosinophilia
Anaemia
Raised ESR & CRP
ANCA negative 

‘String of beads’ on angiogram

Question 21

What do tests show in microscopic polyangiitis?

Answer 21

Rapidly progressing glomerulonephritis
Pulmonary haemorrhage in 30%
pANCA +

Treatment: steroids + methotrexate

Question 22

What antibodies are associated with Churg-Strauss syndrome and granulomatosis with polyangiitis?

Answer 22

Churg-Strauss: pANCA

Granulomatosis: cANCA