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)