What is vasculitis?
Inflammation of blood vessel walls with narrowing and occlusion leading to tissue or organ damage. Most forms of vasculitis have no known cause
What can trigger vasculitis?
Infection - Hep B, C, HIV, Staph aureus
Drugs - Antithyroids, cocaine, levamisole
What are the main large vessel vasculitidies?
- Giant cell arteritis
- Takayasu’s arteritis
What are the main medium vessel vasculitidies?
- Kawasaki’s Disease
- Polyarteritis nodosa
What are the main small vessel vasculitidies?
- Granulomatosis with polyangitis (Wegener’s)
- Eosinophilic granulomatosis with polyangitis (Churg Strauss)
- Microscopic polyangitis
- HSP
- Goodpasture’s syndrome
What is the typical renal lesion that occurs in small vessel vasculitis?
Focal segmental necrotizing glomerulonephritis
Focal because it affects only a few glomeruli, not all of them; segmental because it may affect only a part of the glomerulus; and necrotizing implying tissue destruction
What are the ANCA positive small vessel vasculites?
- Microscopic polyangitis
- Eosinophilic granulomatosis with polyangitis
- Granulomatosis with polyangitis
What are the ANCA negative small vessel vasculites?
- HSP
- Goodpastures
- Cryoglobulinaemia
What is c-ANCA associated with?
Granulomatosis with polyangitis - Wegeners GRanulomatosis
What is p-ANCA associated wtih?
- Microscopic polyangitis
- Eosinophilic granulomatosis with polyangitis - Churg-Strauss
What is the significance of ANCA testing?
ANCA defines a subgroup of patients with small-vessel vasculitis who have a predilection for renal disease (typically GPA and microscopic polyangiitis) and who require similar treatment.
When would you consider vasculitis as part of your differential diagnosis?
If someone was presenting with any multisystem disorder
What are systemic features of vasculitis?
- Fever
- Malaise
- WEight loss
- Arthralgia
- Myalgia
What are skin features of vasculitis?
- Purpura
- Ulceration
- Livedo reticularis
- Nailbed infarcts
- Digital gangrene
What eye features can present in vasculitis?
- Episcleritis
- Scleritis
- Visual loss
What ENT features can present in vasculitis?
- Epistaxis
- Nasal crusting
- Stridor
- Deafness
What pulmonary features can present in vasculitis?
Haemoptysis and dyspnoea - pulmonary haemorrhage
What cardiac features can present in vasculitis?
- Angina
- MI
- Heart failure
- Pericarditis
What GI features can present in vasculitis?
- Pain/perforation
- Malabsorption - due to chronic ischaemia
What renal features can occur in vasculitis?
- Hypertension
- Haematuria
- Proteinuria
- Casts
- Renal failure
What are neurological features of vasculitis?
- Stroke
- Seizures
- Chorea
- Psychosis
- Confusion
- Impaired cognition
- Altered mood
- Mononeuritis multiplex
- Sensorimotor polyneuropathy
What GU features can occur in vasculitis?
- Orchitis
- Testicular pain/tenderness
How would you investigate someone with suspected vasculitis?
- Bloods - ESR/CRP, ANCA, U+E’s, serum creatinine
- Urinalysis
- CXR
- Angiography
- Biopsy
What results might you see on bloods for vasculitis?
- Increased ESR/CRP
- ANCA positive
- Raised creatinine if renal failure
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Rheumatoid Arthritis51
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Osteomalacia, Rickets, Osteogenesis Imperfecta and Pagets Disease35
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Juvenile Idiopathic Arthritis24
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Seronegative Arthropathies28
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Polmyalgia Rheumatica and GCA15
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Crystal Disease27
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Immunological Testing0
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SLE50
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GCA11
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Scleroderma/Systemic Sclerosis37
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Sjogren's Syndrome20
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Polymyositis/Dermatomyositis27
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Granulomatosis with Polyangitis (Wegner's Granulomatosis)13
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Microscopic Polyangitis5
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Eosinophilic granulomatosis with polyangitis (EGPA)4
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Fibromyalgia3
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Polyarteritis Nodosa0
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Clinical Signs/Symptoms - Systemic Disease0
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Vasculitis26
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investigations and Management30
