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Year 2 Unit 2 > Vasculitides > Flashcards

Vasculitides Flashcards

1
Q

Leukocytoclastic

A
  • vascular damage caused by nuclear debris and neutrophils
  • associated with purpura
  • intimal thickening (common to all vascular wall injury)
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2
Q

Noninfectious vasculitis

A
  • drugs, autoimmune diseases, idiopathic
  • immune complex deposition (Type III HS)
  • antineutrophil cytoplasmic antibodies
  • antiendothelial cell antibodies
  • auto reactive T cells (type IV HS)
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3
Q

Infectious vasculitis

A
  • direct invasion of vascular walls with pathogen triggers immune response (syphilis, RMSF- Rocky Mountain spotted fever)
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4
Q

Post infectious vasculitis

A
  • triggering one of four non infectious types by infectious aggravation
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5
Q

ANCAs

A
  • antineutrophil cytoplasmic antibodies
  • diagnostic markers, titer can fluctuate with disease activity
  • can directly activate neutrophils and trigger intense inflammation in vascular beds
  • pauci-immune - biopsies and IF fail to show much Ab left
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6
Q

Anti-proteinase-3

A
  • previously C-ANCA

- associated with polyangiitis, others

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7
Q

Anti-myeloperoxidase

MPO-ANCA

A
  • previously p-ANCA
  • induced by propylthiouracil
  • associated with microscopic polyangiitis and Churg-Strauss
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8
Q

Large Vessel vasculitides

A
  • Granulomatous disease (Giant cell arthritis, Takayasu arteritis)
  • Immune complex mediated* (polyarteritis nodosa)

*also medium vessels

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9
Q

Medium Vessel vasculitides

A
  • Immune complex mediated (polyarteritis Nodosa)
    • also large vessels
  • Anti-endothelial cell antibodies (Kawasaki disease)
    • also small vessels
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10
Q

Small vessel vasculitides - Paucity of immune complexes

A
  • Vasculitis w/o asthma or granulomas (microscopic polyangiitis)
  • Granulomas, no asthma (Wegener granulomatosis
  • Eosinophilia, asthma, and granulomas (Churg-Strauss syndrome)
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11
Q

Small vessel vasculitides - immune complex mediated

A
  • SLE
  • IgA (Henoch-Schoenlein purpura)
  • Cryoglobulin
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12
Q

Takayasu “Pulseless” arteritis

A
  • “Aortic Arch Syndrome”
  • Granulomatous inflammation of aortic arch and offshoots
  • T-cell mediated (unusual)
  • 1:9 M:F predominance, esp. In Asia
  • Sym: downstream effects of stroke or embolism
  • -> absent, asymmetric, or decreased upper extremity pulses, discrepancy BPs, stroke or visual defects
  • Skip lesions
  • Hist: Granulomatous inflammation in media, elastic Latin fragmentation
  • Dx w eye exam, any-endothelial Ab, anti-smooth mm Ab in 2/3 pts
  • aspirin, corticosteroids, heals w stenosis
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13
Q

Giant Cell/ Temporal arteritis

A
  • Chronic inflammation of large to small arteries of head
  • same pathogenesis as Takayasu
  • Presents in middle aged to older pts
  • headache, facial pain, jaw claudication, sudden blindness
  • Medical emergency! Start corticosteroids right away, then get temporal artery biopsy and eye exam
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14
Q

Polyarteritis Nodosa

A
  • Segmental transmutation necrotizing inflammation of small to medium arteries of whole body
  • 80% pts have renal involvement, coronary and mesenteric arteries are common
  • Spares pulmonary vessels
  • Renal infarcts with prerenal AKI( 20:1 BUN: creatinine)
  • bowel infarct (bloody diarrhea), coronary infarct, skin or testicle infarct
    30% have chronic Hep B + HBSAG
    –> anti-hep b immune complexes (type III HS)
  • Dx w angiography, bx if possible
  • tx w corticosteroids
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15
Q

Kawasaki Disease (Mucocutaneous LN Syndrome)

A
  • Arteritis of large to small vessels in young children
  • Dx only by clinical criteria: 5 days of fever over 101 plus 4/5 CRASH; or 2/5 CRASH + coronary aa changes on echo
  • very very high CRP elevation, elevated ESR
  • similar path as PAN, feared com;citation of giant aneurysms on coronaries in 20%
  • acute tx (w/in first 10 days) - high dose aspirin and IVIG reduces heart aneurysms to 1%
  • chronic tx - aspirin low dose long term
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16
Q

CRASH syndrome

A 
Conjunctivitis (bilateral)
Rash (polymorphous) 
Adenopathy
Strawberry tongue, cracked peeling lips
Hands and feet swollen or red
17
Q

Microscopic Polyangiitis

A
  • Disease of capillaries and small vessels more than medium vessels
  • Hypersensitivity vasculitis/ Leukocytoclastic vasculitis (Neutrophils)
  • 90% w necrotizing glomerulonephritis , pulmonary capillitis, palpable purpura
  • infarcts uncommon
  • most associated w MPO-ANCA, can be part of other vasculitic disorders or in response to drugs, infections, tumors
  • pauci-immune if not found early enough
  • tx w steroids, cyclophosphamide
18
Q

Churg-Strauss Syndrome

Eosinophilic Granulomatosis and Polyangiitis

A
  • initially presents as asthma, allergic rhinitis, eczema, recurrent pneumonia in early 20s and 30s
  • lung infiltrates, peripheral eosinophilia, extravascular necrotizing granulomas when vasculitis progresses into 40s and 50s
  • MPO-ANCA in 50% (IgE elevation)
  • path similar to PAN and MP, but with eosinophils >1500/microL and granulomas
19
Q

Behcet disease

Silk Road disease

A
  • small to medium vessels
  • recurrent oral aphthous ulcers, genital ulcers, uveitis
  • Erythema nodosum, arthritis
  • Pulmonary aneurism formation and rupture (high mortality), CNS involvement
  • HLA-B-51, Th17 recruitment of neutropils
20
Q

Wegener Granulomatosis

Granulomatosis w Polyangiitis

A
  • Triad: necrotizing granulomas of respiratory tract, necrotizing/ Granulomatous vasculitis of small to medium vessels (upper airways) –> infarctions, focal necrotizing glomerulonephritis
  • saddle nose, tracheal collapse, cavitation
  • recurrent sinus infections or pneumonia w cavitation
  • PR3-ANCA (C-ANCA) positive in 95%
  • tax w steroids and cyclophosphamide
21
Q

Buerger disease

Thromboangiitis Obliterans

A
  • exclusive to smokers, mostly
22
Q

Henoch-Schoenlein Purpura

IgA vasculitis

A
  • small vessel vasculitis of skin, GI, kidneys, joints

- clinical Dx in kids

23
Q

Raynauds Phenomenon v. Disease

A
  • Exaggerated vasoconstriction response to stimulus
  • “Red, white, and blue”
  • Primary (disease) or secondary (phenom.) associated with SLE, scleroderma, CREST syndrome, Buerger, atherosclerosis
  • Tx: avoid cold, use calcium channel blockers

Year 2 Unit 2 (6 decks)

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