Flashcards in Vasculitis Deck (39):
1
What is vasculitis?
Heterogeneous group of disorders that all have inflammation of the blood vessel walls in common
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What is the problem with vascular damage?
It causes end organ damage
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How do the vasculitides present?
With fever/myalgias/arthralgias/malaise, then organ specific signs and symptoms
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Infectious vasculitis
Direct invasion of vessel wall by pathogens that result in inflammation.
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Fungal causes of vasculitis
Aspergillus, mucor
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Bacterial causes of vasculitis
Treponema pallidum, legionella, pseudomonas.
Rickettsia rickettsiae
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Non-infectious vasculitis
Not known to be caused by direct invasion of the vessel wall, perhaps immune mediated. q
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Why is it important to distinguish infectious/noninfectious vasculitis?
Because for noninfectious, immunosuppression could exacerbate infectious. Also know that noninfectous vasculitis can be precipitated by an infectious process.
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Important vasculitis classifications
Large medium small variable vessel vasculitis
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Medium vessels are...
Main visceral arteries, veins and their initial branches.
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Small vessels are..
intraparenchymal arteries, arterioles, capillaries, venules, and veins
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If a disease is classified as large vessel, or whatever, can it affect other vessels?
Yes.
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Some immunological mechanisms that can initiate non-infectious vasculitides
SLE: Dna-antiDNA complexes
Polyarteritis nodosa: HepBag and AB complexes
Streptokinase/penicillin are antigenic
Antiendothelial cell antibodies may predispose to kawasaki disease
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ANCA
Anti-neutrophil cytoplasmic antibody. Circulating that react with cytoplasmic antigens in neutrophils and endothelial cells. cytoplasmic anca is now Proteinase 3 anca (PR3anca). Perinuclear anca is now MPO-anca (myeloperoxidase).
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Blindness in giant cell arteritis due to
Infection of small vessels ,even though GCA is a large artery disease.
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Two major variants of LVV
Giant Cell Arteritis (>50)
Takayasu Arteritis (<50)
Histopathologically indistinguishable -- granulomatous
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Granuloma characteristics
Has giant cells, epithelioid macrophages and a collar of lymphocytes
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How to diagnose GCA
Temporal artery biopsy.
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Does a negative biopsy rule out GCA?
No. Start steroids
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Biopsy appearance for GCA
Intimal thickening with granulomatous inflammation. IEL fragmentation with elastic stain.
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Where does takayasu arteritis present
Left subclavian, left common carotid, brachiocephalic artery
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How to diagnose?
Can't do biopsy because of the vessels involved. Signs of limb ischemia, decreased pulse at brachial arteries, blood pressure difference between arms, subclavian bruits
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Patients with Takayasu?
80-90% females. Under 40.
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How to treat takayasu arteritis?
Glucocorticoids early, vascular surgery later.
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Medium vessel vasculitis onset compared to LVV
Onset in more acute and necrotizing than in LVV.
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Two major categories of MVV
Polyarteritis nodosa
Kawasaki's disease
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Polyarteritis nodosa
Necrotizing vasculitis of medium to small arteries (ONLY!). Often leads to inflammatory aneurysms or rupture
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Most commonly involved arteries in polyarteritis nodosa?
Renal arteries. causing hypertension and ischemic nephropathy But not glomerular capillaries. So no glomerulonephritis
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What set of arteries does PAN usually spare?
Pulmonary circulation
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Symptoms of PAN
Malaise fever weightloss, hypertension, abdominal pain/melena, diffuse muscular aches and pains.
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How to treat PAN?
Corticosteroids and cyclophosphamide
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Is PAN assoiciated with ANCA?
NO! 30 % have hep B
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Biopsy findings in PAN
Begins as a segmental transmural inflammation with neutrophil predominance. Later, fibrinoid necrosis (bright pink) comprised of deposits of immune complexes.Lots of fibrin too. Finally chronic inflammation and scarring. All stages of activity present within the same vessel!
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Kawasaki disease
Medium size arteries. Is associated with mucocutaneous lymph node syndrome and is found in infants and young childnre. Acute febrile and self limited illness. But has predelection for coronoarry arteries.
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How does Kawaksaki disease happen?
Autoantibodies to endothelial and smooth muscle cells
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Signs and symptoms
Fever and mucocutaneous lymph node senydome. Conjuctnival injection oral mucositis with strawberry ongue and cracked red lips. Accompanied by a skin rask and edema of hands and feet. Cerbival lymph node enalrgement.
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How many people w/ KD develop cardiac sequellar?
20% if untreated. Can cause coronary artery ectasia leading to aneurysms and can cause rupture or thrombosis causing MI.
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How to treat KD
ASPIRIN!!! This is super high yield.
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