Vasculitis

Question 1

Describe Granulomatosis with Polyangitis

Answer 1

Describes a small-vessel vasculitis primarily affecting the respiratory tract and kidneys

Question 2

What classic facial feature is seen in granulomatosis with polyangitis?

Answer 2

Saddle shaped nose (due to nasal bridge collapse)

Question 3

Give 4 clinical features of granulomatosis with polyangitis

Answer 3

URT - Epistaxis, Sinusitis, Nasal Crusting

LRT - Dyspnoea, Haemoptysis, Cough

Kidney - Rapidly Progressing Glomerulonephritis

Facial Features - Saddle Shaped Nose

Question 4

What investigation result would be indicative of granulomatosis with polyangitis?

Answer 4

cANCA positive

Question 5

Describe eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Answer 5

Describes a small-vessel vasculitis primarily affecting the lungs and skin (can affect the kidneys)

Question 6

How does eosinophilic granulomatosis with polyangiitis typically present? (3)

Answer 6

Late onset asthma (asthma presenting in middle age)

Sinusitis and rhinitis

Eosinophilia

Question 7

What 2 investigation results would be indicative of eosinophilic granulomatosis with polyangitis?

Answer 7

p-ANCA

Raised eosinophils

Question 8

How is granulomatosis with polyangiitis managed? (3)

Answer 8

Steroids

Cyclophosphamide

Plasma exchange

Question 9

Define Henoch-Schonlein Purpura (IgA vasculitis)

Answer 9

Commonly presents in children as a purpuric rash affecting the lower limbs and buttocks.

Typically occurs in children following an infection

Question 10

Give 4 clinical features of Henoch-Schonlein Purpura

Answer 10

Palpable Purpuric Rash over buttocks and extensor surfaces of arms and legs

Joint pain

Abdominal Pain

Renal involvement (IgA nephritis - Berger’s disease - Haematuria/Renal failure)

Question 11

In Henoch-Schonlein Purpura, inflammation occurs due to accumulation of what within blood vessels?

Answer 11

Immunoglobulin A

Question 12

What tests is it important to repeat in patients with Henoch-Schonlein Purpura? (2)

Answer 12

Urine Dipstick (monitor renal involvement)

Blood pressure (monitor hypertension)

Question 13

When investigating possible vasculitis, what blood tests ate important to conduct?

Answer 13

Inflammatory markers - ESR/CRP

ANCA - pANCA (eosinophilic) or cANCA (granulomatosis)

Question 14

Give 5 common features seen in vasculitis

Answer 14

Joint and muscle pain

Peripheral neuropathy

Renal impairment

Purpura (purple non-blanching spots on skin)

GI symptoms (diarrhoea, abdo pain, bleeding)

Question 15

Give 5 systemic symptoms seen in vasculitis

Answer 15

Fatigue

Fever

Night sweats

Weight loss/Anorexia

Anaemia

Question 16

How are most vasculitis’ managed?

Answer 16

Steroids to affected area;

Oral - Prednisolone

IV - Hydrocortisone

Nasal - For nasal symptoms (granulomatosis with polyangitis)

Inhaled - For lung involvement (eosinophilic granulomatosis with polyangitis)