Vasculitis

Question 1

Define vasculitis

Answer 1

Inflammation within or through a vessel wall → impairment of blood flow and sometimes damage to vessel integrity → haemorrhage/infarction to organs

Question 2

What is polymyalgia rheumatica?

Answer 2

Pain and stiffness of the neck, shoulder and pelvic girdle. Caused by inflammatory condition of the blood vessels, such as GCA.

Morning stiffness, imrpoves with activity.

Question 3

What is the treatment for polymyalgia rheumatica?

Answer 3

Small dose of corticosteroids → dramatic response

Question 4

Which vessels does GCA attack?

Answer 4

Large vessels - vessels with internal elastic component

i.e. Arch of Aorta and branches

Question 5

What are the common features of GCA?

Answer 5

Headaches +/- scalp tenderness

Jaw claudication

Visual disturbance

Fatigue

Question 6

What happened if GCA is untreated? What is the treatment?

Answer 6

Immediate treatment necessary, treat as GCA if suspected.

If untreated → blindness, stroke/MI, tissue necrosis

High dose prednisolone 40mg (60-80mg if ocular symptoms) + Aspirin

Question 7

What specific investigation is implicated for GCA?

Answer 7

Temporal Artery Biopsy

Question 8

Name 4 types of small vessel vasculitis.

Is small vessel vasculitis common or uncommmon?

Answer 8

Wegener’s (GPA)

Microscopic Polyangiitis

Polyarteritis Nodosum

Churg-Strauss Disease (EGPA)

Question 9

In addition to the normal bloods (FBC, ESR/CRP, cultures…) what other investigations should be carried out for small vessel vasculitis?

Answer 9

ANCA (or other autoAB)

Complement/Cyroglobulins/Hepatitis Serology

Organ involvement tests

Question 10

What would the ANCA tests show for Wegener’s and Microscopic Polyangiitis?

Answer 10

Question 11

Which structures are commonly affected in Wegener’s?

Answer 11

Nose/ENT (most common presentation 92%)

Trachea and Lungs (50%)

Eyes, Renal, Systemic Symptoms

Question 12

What is this sign? and What vasculitis is it associated with?

Answer 12

Saddle Nose Deformity

Wegener’s

Question 13

What is polyarteritis nodosa? What is a common sign for it?

Answer 13

Multisystem Vasculitis - formations of aneurysms in mediu sized vessels

Mononeuritis Multiplex - multiple single nerves affected

i.e. Foot drop and Wrist drop

Question 14

What condition is Polyarteritis Nodosa associated with?

Answer 14

Hepatitis B

Question 15

What small vessel vasculitis types are similar in symptoms to polyarteritis nodosa?

Answer 15

Microscopic Polyangiitis

Churg Strauss Disease

Question 16

Which small vessel vasculitis is associated with Adult-Onset Asthma?

Answer 16

Churg Strauss (EGPA)

Question 17

What is the treatment plan for small vessel vasculitis?

Answer 17

Induce remission - steroid/cyclophosphamide/biologics

Maintain remission - azathioprine/methotrexate/MMF/biologics

Monitor - damage/withdraw treatment

Question 18

What are three things associated with Anti-phospholipid syndrome?

Answer 18

Recurrent Vascular Thrombosis

Pregnancy loss

Thrombocytopaenia

Question 19

What are the clinical features of anti-phospholipid syndrome?

Answer 19

Complications of arterial and venous thromboembolism (DVT/stroke/MI/organ infarction)

Livedo Reticularis

Thrombocytopaenia

Cardiac Valvular Abnormalities

Migraine

Recurrent unexplained pregnancy loss (IUGR)

Question 20

What is the treatment for anti-phospholipid syndrome?

Answer 20

Warfarin/Anticoagulation

High INR (3-4)