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16 Cards in this Set

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two vasculitides affecting larger vessels?
takayasu
GCA
wegener's granulomatosis def
primary granulomatous vasculitis of unknown aetiology which affects small vessels predominantly
wegener's clinical features
pattern: URTI, lungs, renal
URTI: necrotising vasculitis upper respiratory tract (epistaxis)
lungs: haemoptysis
renal
wegener's diagnosis
(diagnostic test, helpful test, appearances on CXR)
diagnostic: biopsy of affected organ (best renal or skin)
helpful: cANCA positive, CXR (migratory pattern nodules, cavities, infiltrates)
wegener's treatment
mainstay: IV methylprednisolon and cyclophosphamide
Polymyalgia rheumatica def
PMR is an inflammatory condition of the muscle causing sudden onset of pain or stiffness
Polymyalgia rheumatica clinical features
eldery (always >50) usually F 70yr
sudden onset severe pain and stiffness of shoulders, neck and hips and lumbar spine. ("limb girdle pattern")
systemic features: fever, tiredness, depression, nocturnal sweats
PMR diagnosis
(diagnostic, helpful)
diagnostic: no diagnostic test
clinical features + high ESR/CRP
temporal biopsy may show GCA but not necessary normally for diagnosis.
PMR treatment and prognosis
steroids over 1-2 years
NSAIDs not helpful
Giant cell arteritis/temporal arteritis def
Inflammatory granulomatous arteritis of large arteries which occurs in association with PMR
GCA - clinical features
(epidemology, classical features and signs, associated disease)
>50
severe headache
scalp tenderness ("pain when combing hair")
claudicatin of jaw
tender swelling of temporal or occipital arteries
VISUAL LOSS
systemic features: fever, tiredness
50% have PMR
GCA - diagnosis
(diagnostic, helpful)
diagnostic: 3 out of 5 (American college rheumatology)
1. age >50
2. new headache
3. temp. artery abnormality (tenderness, pulselessness)
4. very high ESR (>50)
5. positive temporal artery biopsy
GCA histological features
intimal hypertrophy
inflammation of intima, subintima, breaking up internal elastic lamina, giant cells, lymphocytes, plasma cells in internal elastic lamina
GCA management
if any visual loss or severe headache, presumptive clinical dx and immediate high dose steroids
biopsy within 1 week to confirm
high dose steroids: 60-100mg prednisolone/day (divided doses)
tapering dose according to ESR
Ca+VitD!!!
NSAIDs NOT USEFUL. AVOID
Takayasu arteriits def
Takayasu arteritis is a granulomatous inflammation of the aorta and its branches
Takayasu clinical features
female, japanese
"pulsless disease"
symptoms of vessel obstruction (heart, TIAs, peripheral arteries) with tenderness over affected arteries
systemic features: fever, malaise