VASCULITIS Flashcards
What is vasculitis?
- a histological term describing inflammation of the vessel wall
- can be seen in many diseases including; RA, SLE, polymyositis, and some allergic drug reactions
Describe the pathophysiology of vasculitis
Inflammation and necrosis of blood vessel walls with subsequent impaired blood flow resulting in:
- Vessel wall destruction - aneurysm, rupture and stenosis—> perforation and haemorrhage into tissues
- Endothelial injury:–>thrombosis + ischaemia/infarction of dependent tissues
How is vasculitis classified?
-By size of blood vessel involved and the presence or absence of antineutrophil cytoplasmic antibodies (ANCA)
Give examples of large-vessel vasculitis
Refers to the aorta and its major tributaries
Examples:
- Giant cell arteritis/polymyalgia rheumatic
- Takayasu’s arteritis
Give examples of medium-vessel vasculitis
Refers to medium and small-sized arteries and arterioles
Examples:
- Classical polyarteritis nodosa (PAN)
- Kawasaki’s disease
Give examples of small-vessel vasculitis
Refers to small arteries, arterioles, VENULES and capillaries
Examples: >ANCA-associated: -Microscopic polyangitis -Granulomatosis with polyangitis >ANCA-negative: -Essential cryoglobulinaemia -Cutaneous leucocytoclastic vasculitis
What size vasculitis tends to be ANCA positive?
Small-vessel vasculitis e.g microscopic polyangitis and granulomatosis with polyangitis
What are all vasculitis associated with?
-All associated with anaemia and a raised ESR
pAre ALL RARE except giant cell (temporal) arteritis
What is PMR?
Systemic disease of the elderly, the pathogenesis is unknown (Large cell vasculitis)
What are the risk factors of PMR
- SLE
- Polymyositis/dermatomyositis
- age>50
- female
What is the epidemiology of PMR?
- Affects those over 50 yrs
- More common in FEMALES than males
What are the signs/symptoms of PMR?
S-udden onset of severe pain and stiffness of the shoulders and neck, and of the hips and lumbar spine; a limb girdle pattern
- worse in the morning, lasting from 30 mins - several hours
- Mild polyarthritis of peripheral joints
- 1/3rd experience; fatigue, fever, weight loss, depression
What are the investigations for PMR?
- Clinical history is usually diagnostic and the patient is ALWAYS OVER 50
- Bloods; ESR & CRP raised, ANCA negative, ALP raised, mild normochromic, normocytic anaemia may be present
- Temporal artery biopsy: Shows giant cell arteritis in 10-30% cases
- Note: creatinine kinase is normal - helps to distinguish from myositis/ myopathies
What is the treatment and management of PMR?
- Corticosteroids; produce a dramatic reduction of symptoms of PMR within 24-48 hours of starting treatment e.g. ORAL PREDNISOLONE
- If improvement does not occur then diagnosis should be questioned
- Decrease dose slowly
- Used long-term so give GI and bone protection (to prevent osteoporosis due to steroid use) e.g. LANSOPRAZOLE and ALENDRONATE and Ca2+ and vitamin D
What is GCA?
Inflammatory granulomatous arteritis of large Cerebral Arteries as well as other large vessels e.g aorta, which occurs in association with PMR (Large cell vasculitis)
Describe the pathophysiology of GCA?
- Arteries become inflamed, thickened and can obstruct blood flow
- Cerebral arteries affected in particular e.g. temporal artery
- Opthalmic artery can also be affected potentially resulting in permanent or temporary vision loss
What are the risk factors of GCA?
- Over 50
- Female
- RA, SLE, scleroderma
Describe the epidemiology of GCA
- Primarily in those OVER 50
- Incidence increases with age
- More common in FEMALES than males
What are the signs of GCA?
- Tenderness and swelling of one or more temporal or occipital arteries
- SUDDEN PAINLESS VISION LOSS - EMERGENCY - Arteritic anterior ischaemic optic neuropathy - optic disc is very pale/swollen
What are the symptoms of GCA?
- Severe headaches (temporal pulsating)
- Tenderness of scalp (combing hair can be painful) or temple
- Claudication of the jaw (pain in jaw) when eating
- Malaise, lethargy, fever
WHat are the investigations for GCA?
- Temporal artery biopsy; DEFINITIVE DIAGNOSTIC TEST - Should be taken BEFORE or within 7 days of starting high dose corticosteroids
- Bloods; CRP&ESR high, ANCA negative, ALP may be raised, Normochromic, normocytic anaemia
What is the diagnostic criteria of GCA?
- Over 50
- New headache
- Temporal artery tenderness or decreased pulsation
- ESR raised
- Abnormal artery biopsy - inflammatory infiltrates present
What is the treatment of GCA?
- HIGH DOSE CORTICOSTEROIDS RAPIDLY e.g. PREDNISOLONE to stop vision loss - gradual reduction of steroids over 12-18 months
- Used long-term so give GI and bone protection (to prevent osteoporosis due to steroid use) e.g. LANSOPRAZOLE and ALENDRONATE and Ca2+ and vitamin D
- Monitor treatment progress by looking at ESR/CRP (should fall)
What is polyarteritis nodosa(PAN)?
- Necrotising vasculitis that causes aneurysms and thrombosis in medium sized arteries, leading to infarction in affected organs
- Has severe systemic symptoms
What are the risk factors for PAN?
- Male
- Hep B
- RA, SLE, scleroderma
What is the epidemiology of PAN?
- Rare in the UK
- Usual occurs in middle-aged men
- More common in MALES than females
- Associated with Hep B
What are the initial symptoms of PAN?
Fever, malaise, weight loss and myalgia
these initial symptoms are followed by dramatic acute features that are due to organ infarction
What are neurological symtoms of PAN?
-Mononeuritis multiplex due to arteritis of the vasa nervorum - Numbness, tingling, abnormal/lack of sensation and inability to move part of the body
What are the abdominal symptoms of PAN?
- Pain due to arterial involvement of the abdominal viscera, mimicking acute cholecystitis, pancreatitis or appendicitis
- GI haemorrhage due to mucosal ulceration
What are the renal symptoms of PAN?
- Presentation is with haematuria and proteinuria
- Hypertension and acute/chronic kidney disease occur
What are the cardiac and skin symptoms of PAN?
Cardiac:
Coronary arteritis causes myocardial infarction and heart failure
Skin:
Subcutaneous haemorrhage and gangrene occur
What are the investigations for PAN?
- Bloods; Anaemia, WCC raised, ECR raised, ANCA negative
- Biopsy, of kidney in particular to look for hypertension and other damage - can be diagnostic
- Angiography - Demonstrates micro-aneurysms in heaptic, intestinal or renal vessels
What is the treatment of PAN?
- CONTROL BLOOD PRESSURE - ACE-inhibitors e.g. RAMIPRIL
- Corticosteroids e.g. PREDNISOLONE in combination with immunosuppressive drugs e.g. AZATHIOPRINE or CYCLOPHOSPHAMIDE
- Hep B should be treated with an antiviral after initial treatment with steroids
