Vasculitis

Question 1

What are the large vessel vasculitides?

Answer 1

Giant cell arteritis

Question 2

What are the medium vessel vasculitides?

Answer 2

Polyarteritis nodosa, Kawasaki disease

Question 3

What are the small vessel vasculitides?

Answer 3

ANCA-associated - microscopic polyangitis, granulomatosis with polyangitis, churg strauss

Question 4

How do vasculitides typically present?

Answer 4

They present with overwhelming tiredness and raised ESR/CRP
Can affect any organ
Systemic - fever, malaise,weight loss, arthralgia
Skin - purpura, ulcers
Eyes - scleritis, episcleritis
ENT - Epistaxis, nasal crusting, stridor
Cardiac - angina or MI
GI - Pain or perforation (infarction)
Renal - hypertension, haematuria
Neurological - stroke fits

Question 5

What is the presentation of giant cell arteritis?

Answer 5

Typically in elderly
Headache - temporal artery and scalp tenderness - shaving or combing hair
tongue/jaw claudication
Amaurosis fugax or sudden monocular vision loss
Malaise, dyspnoea, weight loss, morning stiffness

Question 6

What is the risk in untreated temporal arteritis?

Answer 6

Bilateral irreversible visual loss

Question 7

What tests should be done in temporal arteritis?

Answer 7

Bloods - ESR and CRP, Platelets increased

Temporal artery biopsy within 14 days of starting steroids (can still miss the skip lesions)

Question 8

What is the treatment of temporal arteritis?

Answer 8

Start 60mg oral prednisolone immediately if involving vision

Once symptoms have resolved reduce dose but continue for 2 years at low dose for complete remission

Question 9

What is polyarteritis nodosa?

Answer 9

It is a necrotising vasculitis that causes aneurysms and thrombitis in medium sized vessels

Question 10

What are the symptoms of polyarteritis nodosa?

Answer 10

There are systemic symptoms, skin rash, renal main cause of death, cardiac, GI and neuro involvement through infarction

Question 11

What is the treatment of polyarteritis nodosa?

Answer 11

The treatment is with BP control, Steroids for mild cases, steroid sparing agents e.g. cyclophosphamide for more severe

Question 12

What is the treatment of raynaud disease?

Answer 12

It is a medium vessel vasculitis that is treated by avoiding cold and calcium channel blockers such as nifedipine

Question 13

What is the presentation of granulomatosis with polyagitis?

Answer 13

It causes necrosing gramulomas in the respiratory tract and kidneys causing haemoptysis and cresenteric glomerulonephritis with proteinuria and haematuria

Question 14

What investigations should be done for granulomatosis with polyangitis?

Answer 14

There will be increased CRP/ESR on blood testing
c-ANCA autoantibody is present in >90%
Urinalysis may show proteinuria or haematuria - if so biopsy may be indicated
CXR may show fluffy infiltrates from haemorrhage

Question 15

What is the treatment of granulomatosis with polyangitis?

Answer 15

It is treated with cyclophosphamide and corticosteroids

Remember 3 C’s c-ANCA, corticosteroids and cyclophosphamide

Question 16

What condition are p-ANCA antibodies associated with?

Answer 16

Churg-strauss syndrome

Question 17

What is the pathophysiology behind henoch-schonlein purpura?

Answer 17

This is an IgA-anti-IgA immunoclomplexes (Type III hypersensitivity reaction) leading to small vessel vasculitis

Question 18

What are the signs and symptoms of henoch-Schonlein purpura?

Answer 18

Follows a group A strep infection
Palpable non blanching purpura of the buttocks and lower limbs
Polyarthritis from bleeding into joints
Glomerulonephritis from immune complex deposition
generalised abdo pain from bleeding in the GI tract

Question 19

What is the treatment of henoch-schonlein purura?

Answer 19

Most spontaneously resolve within 4 months
Mostly conservative treatment
If severe GI or renal involvement then can use steroids