Vasculitis Flashcards
What is vasculitis?
Vasculitis = inflammation of the vessel wall characterised by 2 main features:
i. size of the blood vessels involved:
=> large vessel vasculitis i.e. aorta and major tributaries
=> medium vessel vasculitis i.e. medium and small sized arteries and artieroles
=> small vessel vasculitis i.e. small arteries, arterioles, venules and capillaries
ii. presence/absence of anti-neutrophil cytoplasmic antibodies (ANCA)
Large vessel vasculitis
What is polymyalgia rheumatica?
What are the presenting features?
- Sudden onset of severe pain and stiffness of the shoulders and neck, hips and lumbar spine (limb girdle pattern)
=> worse in the morning
=> lasts 30mins to several hours
- Inflammation of peripheral joints in 25%
- Systemic features i.e. tiredness, fever, weight, depression and nocturnal sweats in 1/3 patients
- > 50yrs old
* clinical hx is diagnostic
Large vessel vasculitis
What are the investigations for polymyalgia rheumatica (PMR)?
- Raised ESR and/or CRP (hallmark of this PMR - rare to see without an acute phase response)
- Serum alkaline phosphatase and gamma-glutamyl-transpeptidase
=> may be raised as markers of inflammation - Anaemia
=> mild normochromic / normocytic - Temporal artery biopsy
=> giant cell arteritis in 10-30% (only performed if GCA suspected)
Large vessel vasculitis
What is giant cell arteritis?
What are the clinical signs?
Inflammatory granulomatous arteritis of large cerebral arteries - occurs in assoc. with polymyalgia rheumatica affecting those >50yrs
Presenting symptoms:
- Severe headaches, tenderness of the scalp or temple (combing hair is painful)
- Claudication of jaw when eating + tenderness & swelling one or more temporal or occipital arteries. Facial pain on facial, maxillary and lingual inflammation
Most feared manisfestation:
3. Sudden, painless temporary or permanent loss of vision in one eye due to involvement of ophthalmic artery
=> amaurosis fugax may precede permanent blindness
- Systemic manifestation = severe malaise, tiredness and fever
Large vessel vasculitis
What is the investigation for giant cell arteritis?
- Normochromic, normocytic anaemia
- ESR raised ; very high CRP
- Abnormal LFT ; low albumin => as in PMR
- Temporal artery biopsy from the affected side (taken before steroids)
- Ultrasound scanning of temporal arteries
- Histological features of GCA:
=> cellular infiltrates of CD4+ T lymphocytes, macrophages and giant cells in the vessel wall
=> granulomatous inflammation of the intima and media
=> Breaking up of the internal elastic lamina
=> Giant cells, lymphocytes, plasma cells in the internal elastic lamina
Large vessel vasculitis
What is the management of polymyalgia rheumatica / giant cell arteritis?
- Corticosteroids = massive reduction in inflammation within 24-48h
=> corticosteroids reduce risk of GCA in patients with PMR
*NSAIDs not affective - should not be used
=> corticosteroids in GCA mandatory - sig. reduce risk of irreversible visual loss
=> if GCA is suspected treatment should not be delayed esp. if there has been visual episodes / stroke - don’t wait for ultrasound/temporal artery biopsy
PMR : 15mg prednisolone as single dose in the morning
GCA : 60-100 mg prednisolone
- Calcium + vitamins D supplements and sometimes bisphosphonates to prevent osteoporosis
Large vessel vasculitis
What is takayasu’s arteritis?
Granulomatous inflammation of the aorta - very rare except Japan
Medium-sized vessel vasculitis
What is polyarteritis nodosa?
Polyarteritis nodosa - very rare ; occurs in middle aged men
Fibrinoid necrosis of vessel walls with micro-aneurysm formation, thrombosis and infarction
Medium-sized vessel vasculitis
What are the clinical features of polyarteritis nodosa?
Systemic features: fever, malaise, weight loss and myalgia
Acute features due to organ infarction:
- Neurological: mononeuritis
- Abdominal: arterial involvement of the abdominal viscera => mimics acute cholecystitis, pancreatitis, appendicitis
=> gastrointestinal haemorrhage occurs due to mucosal ulceration
- Renal: haematuria and proteinuria. Hypertension and acute/chronic kidney disease
- Cardiac: coronary arteritis causes MI, heart failure and pericarditis
- Skin: subcutaneous haemorrhage and gangrene.
=> persistent livedo reticularis in chronic disease
=> cutaneous & subcutaneous palpable nodules
Medium-sized vessel vasculitis
What is the investigation and management of polyarteritis nodosa?
Blood count: anaemia, leucocytosis, raised ESR
Biopsy
Angiography: microaneurysm in hepatic, intestinal or renal vessels
If needed: ECG, abnormal ultrasound,
Treatment: corticosteroids with immunosuppressants i.e. azathioprine
Medium-sized vessel vasculitis
What is Kawasaki disease?
Acute systemic vasculitis involving medium-sized vessels
Affects mainly children <5yrs
Common in Japan
Infective trigger?
Medium-sized vessel vasculitis
What are the clinical features of Kawasaki’s disease?
Fever >4days
Bilateral conjunctival congestion
Dry red lips & oral cavities
Cervical lymphadenopathy
Rash & redness of palms and soles
CVS changes in acute stage = pancarditis and coronary arteritis => aneurysms or dilation visible on echo, MRI or angiography
Medium-sized vessel vasculitis
What is the management for Kawasaki’s disease?
Single dose of high-dose IV immunoglobulin => prevents coronary artery disease
After acute phase => aspirin 200-300mg daily
Small vessel vasculitis
What are the 2 categories of small vessel disease?
- ANCA-associated vasculitis including:
=> granulomatosis with polyangiitis
=> eosinophilic granulomatosis with polyangiitis
=> microscopic polyangiitis
- Immune complex (ANCA negative) small vessel vasculitis with vessel wall immunoglobulin including:
=> anti-glomerular basement membrane (anti-GBM) disease
=> IgA vasculitis i.e. Henoch-Schönlein)
=> cutaneous leucocytoclastic vasculitis
Small vessel vasculitis
What is cutaneous leucocytoclastic vasculitis?
Small vessel vasculitis only affecting skin characterised by:
Signs:
=> palpable purpuric lesion involving dermal post-capillary venules e.g. on legs
=> nodules, ulcers, livedo reticularis
=> may be accompanied by arthralgia and glomerulonephritis
Causes:
=> drugs i.e. penicillin, sulphonamides, thiazides
=> idiopathic
=> neoplasia
=> systemic vasculitis i.e. polyarteritis nodosa, Henoch-Schonlein purpura (vasculitic rash on legs/buttocks ± arthralgia, abdo pain & glomerulonephritis)
=> Wegener’s granulomatosis
