Vasculitis

Question 1

Define

Answer 1

The inflammation and necrosis of blood vessels

Vasculitis may also occur secondary to infections, abscesses, malignancies and connective tissue diseases (e.g. RhA, SLE)

Primary vasculitides are classified according to main vessel size affected

• Large: Giant cell arteritis, Takayasu’s aortitis
• Medium: Polyarteritis nodosa, Kawasaki’s disease
• Small: Churg–Strauss syndrome, microscopic polyangiitis, Henoch–Schonlein purpura, Wegener’s granulomatosis, mixed essential cryoglobulinaemia, relapsing polychondritis

Question 2

Causes

Answer 2

aetiology of primary vasculitis is unknown ?AI origin

Immune complex deposition in the vessel wall triggers classical complement activation and inflammation

Question 3

Epidemiology

Answer 3

annually 1/10 000
Takayasu’s aortitis is more common in young Japanese females

Question 4

Symptoms and signs

Answer 4

Medium and small vessel vasculitides are characterized by multi- organ involvement with less specific clinical features

Possible features of all diseases:

• General: Fever, night sweats, malaise, weight loss
• Skin: Rash (vasculitic, purpuric, maculopapular, livedo reticularis)
• Joint: Arthralgia or arthritis
• GI: Abdominal pain, haemorrhage from mucosal
• ulceration, diarrhoea
• Kidney: Glomerulonephritis, renal failure
• Lung: Dyspnoea, cough, chest pain, haemoptysis, lung haemorrhage
• CVS: Pericarditis, coronary arteritis, myocarditis, heart failure, arrhythmias
• CNS: Mononeuritis multiplex, infarctions, meningeal involvement
• Eyes: Retinal haemorrhage, cotton wool spots

Question 5

Features characteristic of specific subtypes

Answer 5

Takayasu’s aortitis

Constitutional upset, ,head/neck pain, tenderness over affected arteries (aorta and major branches)
Dizziness, fainting, ↓peripheral pulse, HTN

Polyarthritis nodosa

Microaneurysms, thrombosis, infarctions, HTN, testicular pain

Kawasaki’s disease

Age <5 years
Fever >5 days
Fissured lips, red swollen palms/soles followed by desquamation Skin rash, inflamed oral cavity, conjunctival congestion Lymphadenopathy, coronary artery aneurysms

Churg–Strauss syndrome

Asthma, eosinophilia

Henoch–Schonlein purpura

Purpura (leg/buttocks), arthritis, gut symptoms, glomerulonephritis

Microscopic polyangiitis

Non-specific, multiple organs affected
Glomerulonephritis (no IG deposits, unlike HSP which has IgA)

Wegener’s granulomatosis

Granulomatous vasculitis of upper and lower respiratory tract Nasal discharge, ulceration and deformity, haemoptysis, sinusitis, corneal thinning, glomerulonephritis

Relapsing polychondritis

Affecting cartilage (e.g. ear pinna, nose, larynx) causing swelling, hoarse voice, tenderness, cartilage destruction and deformity (e.g. saddle nose)

Mixed essential cryoglobulinaemia

Arthritis, splenomegaly, skin vasculitis, renal disease, cryoglobulins (IgG and IgM mix)

Question 6

Investigations

Answer 6

Bloods

• FBC - normocytic anaemia, high platelets, high neutrophils
• 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)