Seminar G Vasculitides Flashcards
Definition
multisystem inflammatory diseases that are characterised by inflammation of blood vessel walls (=vasculitis).
Classification
The vasculitides represent an extremely heterogeneous group, but classification is generally by size of vessel wall affected. There have been many attempts at classification over the years, and none is entirely satisfactory.
Think of vasculitis when patient presents with
- unexplained pyrexia with normal cultures
- high inflammatory response
- unexplained weight loss
- unusual rashes (especially ulceration and “palpable purpura”)
- new onset renal disease (especially microscopic haematuria)
- widespread musculoskeletal symptoms
- mouth ulcers, abdominal pain, bloody diarrhoea
- dyspnoea, haemoptysis
- sensory or motor loss
Large arteries
disease
Giant cell (temporal) arteritis Takayasu’s arteritis
Medium Vessels disease
Polyarteritis nodosa
Kawasaki disease
Small Vessel disease
Wegener’s granulomatosis Chur-Struss syndrome Microscopic polyangiitis Henoch-Schonlein purpura Cutaneous leukocytoclastic angiitis
Arteries and veins disease
Behcet’s Syndrome
Secondary Vasculitis
Giant cell (temporal) arteritis
A granulomatous arteritis of the aorta and its major branches, with a predeliction for the extracranial branches of the carotid. A disease of the elderly, it is often associated with polymyalgia rheumatica
Takayasu’s arteritis
A granulomatous arteritis of the aorta and its major branches. Usually occurs under age 50.
Polyarteritis nodosa
Necrotizing inflammation of medium- or small-sized arteries, leading to aneurysm formation and organ infarction. Glomerulonephritis is usually absent.
Kawasaki disease
Usually affects infants and young children. Acute vasculitis of large-, medium- and small-sized arteries. Often affects coronary arteries. Also known as mucocutaneous lymph node syndrome - conjunctival injection, desquamation of hands and feet, cervical lymphadenopathy, oropharyngeal changes.
Wegener’s granulomatosis
Necrotizing granuloma of upper and lower respiratory tract, necrotizing vasculitis of capillaries, venules, arterioles and arteries. Necrotizing glomerulonephritis is common. Strongly associated with antineutrophil cytoplasmic antibodies (ANCA).
Chur-Struss syndrome
Granulomatous inflammation of respiratory tract, with necrotizing vasculitis of small- to medium-sized vessels. Associated with asthma. Eosinophilia a prominent feature. Strongly associated with ANCA.
Microscopic polyangiitis
Necrotizing vasculitis of capillaries, venules, and arterioles. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis (with haemorrhage) often occurs. Strongly associated with ANCA.
Henoch-Schonlein purpura
Vasculitis of small vessels (capillaries, venules, arterioles) with IgA-dominant immune deposits. Typically involves skin (“Palpable purpura”), gut (abdominal pain, bloody diarrhoea), and glomeruli (microscopic haematuria), and is associated with arthritis or arthralgias.
Cutaneous leukocytoclastic angiitis
Isolated cutaneous small-vessel vasculitis, associated with immune-complex deposition. “Palpable purpura” usually seen. Systemic features absent. Often caused by drug hypersensitivity or malignancy.
Behcet’s Syndrome
A systemic vasculitis affecting virtually any vessel type. Oral and genital ulceration, and eye lesions (uveitis and retinal vasculitis) are characteristic. Arthritis, rashes, and internal organ involvement also occur.
Secondary Vasculitis
Vasculitis may occur secondary to other rheumatic disease. The most common examples are rheumatoid arthritis and systemic lupus erythematosus.
Differential Diagnosis →
- Infective endocarditis
- Atrial myxoma
- Antiphospholipid antibody syndrome
- Acquired Immune Deficiency Syndrome
- Sarcoidosis
- Secondary syphilis
Giant-cell (temporal) arteritis (GCA)
is a granulomatous arteritis of the aorta and large blood vessels.
Giant-cell (temporal) arteritis (GCA) Clinical Features
Severe headaches
Scalp tenderness
Visual disturbances
Jaw claudication
Giant-cell (temporal) arteritis (GCA) Investigations show
An acute inflammatory response with a very high ESR
Giant-cell (temporal) arteritis (GCA) Temporal artery biopsy shows
Disruption of the elastic lamina, monoculear cell infiltrates with formation of giant cells and granulomata
Giant-cell (temporal) arteritis (GCA) Most important complication
Irreversible blindness due to central retinal artery occlusion
Giant-cell (temporal) arteritis (GCA) Treatment
Prompt treatment of glucocorticoids (prednisolone 1mg/kg)
Polymyalgia Rheumatica (PMR)
- Pain and intractable early morning stiffness around the shoulder and hip girdles, without obvious synovitis in the joints
- Malaise, weight loss and a low-grade fever may also occur.
Polymyalgia Rheumatica (PMR) Mean age of onset
70 years (extremely rare under age of 50 yrs)
Polymyalgia Rheumatica (PMR) Investigations show
Raised ESR or plasma viscosity
Polymyalgia Rheumatica (PMR) Treatment
Prompt treatment of glucocorticoids (prednisolone 1mg/kg)
Polymyalgia Rheumatica (PMR) Concomitant treatment with
bisphosphonate
Behcet’s Syndrome: Definition
A systemic vasculitis
Behcet’s Syndrome: Female to Male:
1:1
Behcet’s Syndrome:Association with
HLA-B51
Behcet’s Syndrome: Clinical Features
Orogenital ulceration Rashes Arthritis Eye involvement Panuveitis with hypopyon Retinal vasculitis
Behcet’s Syndrome: Pathergy reaction
Characteristics pustules form at the site of simple trauma such as a needle puncture would (28-48hr)
Behcet’s Syndrome: Treatment
Colchicine
Glucocorticoids
Azathioprine
