VASCUITIDES 

Size                      Examples  

Large                    Giant Cell Arteritis (GCA), Takayasu’s arteritis  

Medium                Kawasaki disease, Polyarteritis nodosa (PAN)

Small - Medium   GPA (Wegener’s), MPA, EGPA (Churg–Strauss), 

Small                     Connective tissue diseases 

Variable                 Behçet’s  

Large Vessel Vasculitis

Giant Cell Arteritis (GCA)

•    Demographics: elderly, women > men

•    Key features: new headache, jaw claudication, scalp tenderness, visual loss, temporal artery tenderness

•    Tests:

o    ESR >50, CRP raised

o    Temporal artery biopsy: granulomatous inflammation

•    Urgent treatment:

o    Start high-dose steroids immediately to prevent blindness

o    Add PPI and bone protection

•    Associated with: Polymyalgia rheumatica

Takayasu’s Arteritis

•    Demographics: young women, esp. Asian

•    Key features: systemic symptoms + absent pulses, BP discrepancies, bruits

•    Complications: limb claudication, aortic aneurysm

•    Investigations:

o    Angiography or MRI angiogram: arterial narrowing

•    Treatment: steroids ± immunosuppressants

Medium Vessel Vasculitis

Polyarteritis Nodosa (PAN)

•    Key features:

o    Mononeuritis multiplex, livedo reticularis

o    Mesenteric ischaemia, renal infarcts, HTN

•    No pulmonary involvement

•    Association: Hepatitis B

•    Investigations:

o    Angiography: microaneurysms, segmental stenoses

o    Hepatitis serology

•    Treatment: steroids + cyclophosphamide ± antivirals (if HBV)

Kawasaki Disease (Paediatric)

•    Features (CRASH + burn):

o    Conjunctivitis, Rash, Adenopathy (cervical), Strawberry tongue, Hand/foot swelling + peeling

o    Fever >5 days

•    Complication: coronary artery aneurysms

•    Test: Echocardiography

•    Treatment: IV immunoglobulin + high-dose aspirin

Small-Medium Vessel Vasculitis (ANCA-associated)

Granulomatosis with Polyangiitis (GPA / Wegener’s)

•    Features:

o    ENT: sinusitis, nasal crusting, epistaxis

o    Resp: cavitating lung nodules, haemoptysis

o    Renal: rapidly progressive glomerulonephritis

•    Autoantibody: cANCA (anti-PR3)

•    Biopsy: necrotising granulomatous vasculitis

•    Treatment: steroids + cyclophosphamide or rituximab

Microscopic Polyangiitis (MPA)

•    Features:

o    Renal-pulmonary syndrome (e.g. glomerulonephritis + alveolar haemorrhage)

o    No granulomas, no ENT disease

•    Autoantibody: pANCA (anti-MPO)

•    Treatment: steroids + immunosuppressants (similar to GPA)

Eosinophilic GPA (EGPA / Churg–Strauss)

•    Features:

o    Asthma

o    Eosinophilia

o    Peripheral neuropathy (mononeuritis multiplex)

o    Pulmonary infiltrates

•    Autoantibody: pANCA (MPO)

•    Biopsy: eosinophilic granulomas

•    Treatment: steroids ± cyclophosphamide

Behçet’s Syndrome

•    Features:

o    Recurrent oral + genital ulcers

o    Anterior uveitis

o    Erythema nodosum, pathergy test +

o    Can cause DVT, cerebral venous sinus thrombosis

•    Management: colchicine, steroids, immunosuppressants for organ involvement

Cryoglobulinaemia (Type II/III – Mixed)

•    Associated with: Hepatitis C

•    Features:

o    Palpable purpura, arthralgia, peripheral neuropathy, glomerulonephritis

•    Test:

o    Serum cryoglobulins

o    HCV serology

•    Treatment: treat underlying HCV ± immunosuppression if severe