Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
16 Cards in this Set
- Front
- Back
|
What is the definition of vasculitis?
What is the pathogenesis? |
A group of rare disease involving inflammatory destruction of blood vessels with resulting ischemia or infarction of tissues
Inflammatory infiltrate in vessel wall --> fibrinoid necrosis --> extravasation of RBCs |
|
|
When should vasculitis be suspected? |
Unexplained prolonged constitutional illness Multi-organ system disease Recurrent upper respiratory disease Pulmonary-renal syndrom PVD not from atherosclerosis Palpable purpura Acute peripheral neuropathy Inflammatory eye disease New HA in elderly person |
|
|
What are considered small vessels? Medium? Large? |
Small=arteriole, capillary, venule Medium=artery (SM present) Large=aorta or primary branch vessel |
|
|
What are the small vessel vasculitis?
What are the medium vessel vasculitis?
What are the large vessel vasculitis? |
Small: Granulomatosis with polyangiitis, microscopic poly angiitis, Eosinophilic granulomatosis with polyangiitis, cryoglobulinemic vasculitis, connective tissue disease-associated, Henoch-Schonlein purpura
Medium: Polyarteritis nodosa, Kawasaki's
Large: Takayasu's arteritis, giant cell arteritis |
|
|
What are the complications of small vessel vasculitis? |
Purpura Scleritis Glomerulonephritis Alveolar hemorrhage |
|
|
What are the complications of medium vessel vasculitis? |
Coronary aneurysm in Kawasaki's
Stenosis and aneurysms of renal and mesenteric arteries in polyarteritis nodosa |
|
|
What are the complications of large vessel vasculitis? |
Stenosis, occlusion and aneurysms esp of subclavian and carotids |
|
|
What are the Anti-Neutrophil Cytoplasmic Antibody (ANCA) associated vasculitis?
Which specific autoantibodies are diagnostic for vasculitis? |
GPA (aka Wegener's) MPA Eosinophilic GPA (aka Churg-Strauß Syndrome)
Proteinase 3 (PR3) or myeloperoxidase (MPO) are types of ANCAs that are only seen in vasculitis |
|
|
What are the frequencies that ANCA (and subtypes) are seen in ANCA-associated vasculitis? |
GPA has ANCA 70-90%, with PR3 85%
MPA has ANCA 90%, with MPO >90%
Churg Strauss has ANCA 40%, but MPO in 80% of those with ANCA |
|
|
What are the two pathological features of GPA and what clinical manifestations do each have? |
Granulomas: pulmonary nodules, sinonasal disease (often starts here), mass lesions
Vasculitis: alveolar hemorrhage, glomerulonephritis, scleritis, purpura, neuropathy, digit ischemia |
|
|
What are the three pathological features of Churg-Strauß?
How does it present? |
Tissue eosinophilia Extra-vascular granuloma Necrotizing vasculitis
Prodromal phase: asthma and rhinitis/sinus inf/nasal polyps Hypereosinophilia: blood and lung Vasculitis all over body |
|
|
What are the characteristics of Takayasu's arteritis?
Which diseases does it mimic? |
Large vessel vasculitis that causes problems via arterial stenosis (ex. absent peripheral pulses, bruits without atherosclerosis, thickening on imaging) Most all patients are female <40yo Chronic relapsing course Mimics: atherosclerosis, fibromuscular dysplasia, congenital vascular abnormalities |
|
|
What are the characteristics of giant cell (temporal) arteritis? |
Most common form of vasculitis. Affects the cranial arteries (temporal, middle meningeal etc.) Can see jaw claudication, HA, vision loss with elevated inflammatory markers in elderly |
|
|
What are the classification criteria for giant cell arteritis? |
≥50 years at onset Elevated ESR or CRP Temporal artery abnormality New HA Abnormal artery biopsy |
|
|
What are the visual/CNS manifestations of GCA?
What are the other ischemic manifestations?
What are the musculoskeletal manifestations? |
HA (70%) Blindness/Visual loss (15% temporary, 15% perm) CVAs (15%, though controversial)
Jaw, tongue, peripheral extremity claudication, Raynauds
Polymyalgia rheumatica, synovitis |
|
|
What is polymyalgia rheumatica? |
Profound arthralgias, stiffness and muscle "weakness" Age >50 ESR elevated Remarkable response to steroids
Not a true vasculitis, but marked association with GCA, thus must monitor |
