Vasculitides

Question 1

Definition

Answer 1

Vasculitis is the inflammation and necrosis of blood vessels.

Question 2

Classifications

Answer 2

Primary vasculitides are classified based on the MAIN VESSEL SIZE affected:

• LARGE
▪ Giant cell arteritis
▪ Takayasu’s aortitis

• MEDIUM
▪ Polyarteritis nodosa
▪ Kawasaki’s disease

• SMALL
▪ Churg-Strauss syndrome
▪ Microscopic polyangiitis
▪ Wegner's granulomatosis
▪ Henoch-Schonlein purpura
▪ Mixed essential cryoglobulinaemia (MEC)
▪ Relapsing polychondritis

Question 3

Aetiology

Answer 3

• UNKNOWN
• Suggested autoimmune origin
• Immune complex deposition in the walls of blood vessels leads to inflammation

Question 4

Risk factors

Answer 4

o Hepatitis B - polyarteritis nodosa
o Hepatitis C - mixed essential cryoglobulinaemia
o pANCA - microscopic polyangiitis + Churg-Strauss
o c-ANCA - Wegner’s granulomatosis

Question 5

Epidemiology

Answer 5

• RARE

* Takayasu’s arteritis is most common in JAPANESE FEMALES

Question 6

Presenting symptoms and signs on physical examination (general)

Answer 6

Large vessel vasculitides have classic clinical patterns based on the vessels affected (e.g. GCA and loss of vision/headache)

Medium and small vessel vasculitides are characterised by multiorgan involvement and have less specific clinical features

Question 7

Presenting symptoms and signs on physical examination (possible features of all)

Answer 7

Possible Features of ALL Vasculitides

o General: fever, malaise, night sweats, weight loss
o Skin: rash
o Joint: arthralgia, arthritis
o GI: abdominal pain, haemorrhage, diarrhoea
o Kidneys: glomerulonephritis, renal failure
o Lungs: dyspnoea, cough ,chest pain, haemoptysis, haemorrhage
o CVS: pericarditis, coronary arteritis, myocarditis
o CNS: mononeuritis multiplex, infarctions
o Eyes: retinal haemorrhage, cotton wool spots

Question 8

Presenting symptoms and signs on physical examination (specific)

Answer 8

Features Characteristics of Specific Vasculitides

o GCA: loss of vision, jaw claudication, headache, scalp tenderness
o Polyarteritis Nodosa: microaneurysms, thrombosis, infarctions, hypertension, testicular pain
o Henoch-Schonlein Purpura: purpura, arthritis, gut symptoms,
glomerulonephritis, IgA deposition
o Wegner’s Granulomatosis: granulomatous vasculitis of upper and lower respiratory tract, nasal discharge, ulceration and deformity, haemoptysis, sinusitis, glomerulonephritis, saddle nose

Question 9

Investigations

Answer 9

• Bloods
o FBC - normocytic anaemia, high platelets, high neutrophils
o High ESR/CRP

• Autoantibodies - e.g. cANCA in Wegner’s
• Urine - haematuria, proteinuria, red cell casts (if glomerulonephritis)
• CXR - diffuse, nodular or flitting shadows, atelectasis
• Biopsy - renal, lung, temporal artery (in GCA)
• Angiography - to identify aneurysms (in PAN)