vasculitis part 1 Flashcards
what are the diseases associated with large vessels vasculitis ?
takayasu arteritis
giant cell arteritis
what are the medium vessel vasculitis ?
polyarteritis nodosa
kawasaki disease
what artery is specifically affected in GCA ?
superficial temporal arteries
ophthalmic arteries
what social habit increases the incidence of GCA ?
smoking
what are the clinical features associated with GCA ?
constitutional
headache
temporal tenderness
jaw claudication
visual /ocular symptoms - amaurosis fugax
CNS affection
arterial aneurysms
what is the presentation of polymyalgia rheumatica ?
morning stiffness of more than 45 minutes
sero-negative
pelvic girdle pain and shoulder pain
what disease is associated with GCA ?
polymyalgia rheumatica
what investigations should be done for patients with GCA ?
visual or ocular symptoms - should be assessed
ESR and CRP
temporal artery biopsy ( negative biopsy does not exclude a diagnosis)
what are the histology findings associated with GCA ?
narrowed lumen
giant cells
inflammatory infiltrates
skip lesions
interruption of elastic lamina
what is the management for polymyalgia rheumatica ?
prednisolone 15 mg
bone protection - biphosphonate, calcium and vitamin D
GI protection - PPI
if no improvements reconsideer diagnosis
what is the management of GCA ?
1- prednisolone 40-80 mg ( immediately for eye protection )
40 mg - if no visual symptoms
60-80 mg - if we suspect visual symptoms
2- bone protection ( biphosphonate, calcium and vitamin D)
3- GI protection (PPI)
4- low dose aspirin is usually given to prevent further complications
what is the management of relapses ?
increase dose to previous level or slightly higher
consider steroid-sparing agents like methotrexate
what are the criteria for the diagnosis of takayasu arteritis ?
1- age at disease onset less than 40 years old ( more common in asian women )
2- claudication of the extremities
3- difference of 10 mmhg in systolic blood pressure between thee
arms
4- bruit over one or both subclavian arteries or the abdominal aorta
5- arteriographic narrowing or occlusion
at least 3 of the 6
what are the clinical features of takayasu arteritis ?
early - non-specific, arthritis
late occlusive phase - limb claudication, visual symptoms, HBP, CVA, headache
late complications - CVD and aneurysm
what are the arteriographic changes in takayasu arteritis ?
corckscrew
narrowing irregularity
what is the management for takayasu vasculitis ?
prednisolone
monitor and treat cardiovascular risk factors
surgery
what are the dominant features of polyarteritis nodosa ?
medium sized vessels
aneurysm and organ infarction
what disease is specifically associated with polyasrteritis nodosa ?
hepatitis B
what vessels are spared in nPAN ?
pulmonary
what are the clinical features associated with polyarteritis nodosa ?
digital gangrene
subcutaneous nodules
radial nerve palsy
micro-aneurysms
livedo reticularis
what are the diagnostic criteria for kawasaki ?
CREAM - happens in infants and children
along with a high grade fever that has lasted for more than 5 days , and 4/5 of the following criteria :
Conjunctivitis
Rash
Edema / erythema of thee hands and the feet
Adenopathy ( cervical commonly unilateral)
Mucosal involvement ( strawberry tongue)
what is the major cause of mmorbidity and morttality in kawasaki disease ?
cornoary vasculitis
what is the treatment for kawasaki vasculitis ?
IV Ig and high dose Aspirin
echo forr surveillance of cornoary arteery aneurysm
at what age do we see GCA ?
ages above 50
more common in white people
what are the complications associated with GCA ?
Permanent monocular blindness
Stroke
Aortic aneurysms
first line investigations for GCA ?
(ESR and CRP), FBC and LFTs
what is the gold standard investigation for GCA ?
temporal artery biopsy
what does dopple US show in GCA ?
halo sign
