B4.071 Vasculitides Flashcards by Lauren Sinik

what is vasculitis

inflammation and necrosis of blood vessels

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etiology of vasculitis

infectious agents, mechanical, radiation, toxins
HBV, HSV, CMV, parovirus
serum sickness
bacterial antigen
autoantibodies: ANCA

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pathogenesis of “noninfectious” vasculitis

immune complex deposition in vessel wall
antineutrophil cytoplasmic antibodies (ANCA)
direct attack of endothelial cells by circulating anti-endothelial Abs
cell mediated immunity (autoreactive T cells)

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what are ANCAs antibodies against?

neutrophil granules/monocyte lysosomes/endothelial cells
anti-myeloperoxidase (MPO-ANCA) (p-ANCA)
anti-proteinase-3 (PR3-ANCA) (c-ANCA)
other

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what diseases are MPO/p-ANCAs associated with

microscopic polyangiitis

Churg-Strauss Syndrome

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what diseases are PR3/c-ANCAs associated with

granulomatosis with polyangiitis (GPA) (wegeners)

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why are ANCAs measured?

track disease activity

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what are some ways that vasculitis is classified

sixe of blood vessel
location
type
nature of inflammatory infiltrate
specific etiological factor
immunopathogenic mechanism

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large vessel vasculitides

Giant-cell arteritis

Takayasu arteritis

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medium vessel vasculitides

polyarteritis nodosa

kawaski disease

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small vessel vasculitides

granulomatosis with polyangiitis (GPA) (wegeners)
churg-strauss syndrome
microscopic polyangiitis

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giant cell arteritis

focal, chronic, granulomatous inflammation of temporal artery

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epidemiology of giant cell arteritis

old age (70-80 yr)
women > men
most common form of vasculitis

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which arteries does giant cell arteritis primarily affect

temporal, cranial, aorta

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pathogenesis of giant cell arteritis

T cell mediated
proinflammatory cytokines (TNFa)
HLA-DR4

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pathology of giant cell arteritis

cordlike, nodular vessel w narrow lumen
granulomatous inflammation, plasma cells, multinucleated giant cells
necrosis, fragmentation of elastic lamina
fibrosis of media and thick intima

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clinical features of giant cell arteritis

benign, self limiting
muscle aches
visual symptoms, diplopia, blindness

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treatment of giant cell arteritis

steroids

anti-TNF

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how does fragmentation of elastic lamina appear on histology?

blue stained lamina abruptly stops and occurs in segments rather than being continuous

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takayasu arteritis

inflammatory disease of unknown etiology in large arteries (aorta and branches)

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epidemiology of takayasu

young women

autoimmune?

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pathology of takayasu

aorta and branches involved
thick intima, thick wall
panarteritis with giant cells
luminal narrowing, scarring

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why is takayasu called pulseless disease

blood flow can’t reach extremities well

pulses cant be found

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clinical features of takayasu

constitutional symptoms
high BP, asymmetry, weak pulse
ocular disturbances

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treatment of takayasu

steroid | surgical reconstruction

polyarteritis nodosa (PAN)

acute necrotizing vasculitis of medium sized muscular arteries

epidemiology of polyarteritis nodosa

men > women | whites > blacks

etiology of polyarteritis nodosa

immune complex mediated damage of vessels | 30% of PAN patients have chronic hep B

pathology of PAN

small to medium sized and large vessels fibrinoid necrosis, mixed inflammatory cells thrombosis, infarction of organ

clinical features of PAN

young adults acute, subacute, or chronis varies w affected site (kidney, heart, liver, GI) constitutional symptoms, infarcts, necrosis

treatment of PAN

steroids anti inflamm drugs immunosuppressive agents control hep B

unique characteristic of PAN

does NOT affect lungs | all other do

Kawasaki disease

acute necrotizing vasculitis of infancy and early childhood with fever, rash, conjunctival and oral lesions, and lymphadenitis

etiology of kawasaki

delayed hypersensitivity T cell reaction | infection with parovirus B19

Kawasaki epidemiology

85% in kids <5 | 95% in kids <10

clinical features of kawasaki

strawberry red tongue | red rashes on hands and feet

treatment of kawasaki

self limited IVIg aspirin

more serious complications of kawasaki

cardiac symptoms: | -coronary arteries affects, aneurysms, rupture, death

granulomatosis with polyangiitis (GPA) (wegeners)

systemic necrotizing granulomatous vasculitis of unknown etiology

organ systems affected by GPA

lungs kidneys upper resp tract

pathology of GPA

``` necrotizing vasculitis parenchymal/geographic necrosis granulomatous inflammation (T cell) small arteries, veins, capillaries crescentic glomerulonephritis (but could potentially not manifest in kidneys) ```

clinical features of GPA

``` males > females hematuria, proteinuria (renal disease) sinusitis (URT) pneumonitis, pulm infiltrate with cavitation (LRT) PR3-ANCA (over 90% of cases) ```

treatment of GPA

steroids cyclophosphamide anti-TNF

Churg-Strauss Syndrome

systemic vasculitis w prominent eosinophilia that occurs in young persons w asthma or allergy

pathology of churg strauss

necrotizing vasculitis of small and medium vessels granulomas, eosinophilia, fibrinoid necrosis similar to microscropic angiitis or PAN MPO-ANCA (< half of cases)

organ systems affected by churg strauss

``` lungs kidney heart liver spleen CNS ```

differential for churg strauss

``` PAN parasitic, fungal infections GPA eosinophilic pneumonia syndrome drug vasculitis ```

treatment for churg strauss

steroids | anti-TNF

microscopic polyangiitis

a group of inflammatory vascular lesions affecting small vessels that are thought to represent a response to exogenous substances localized and generalized forms

localized form of microscopic polyangiitis

skin leukocytoclastic vasculitis cutaneous vasculitis/venulitis palpable purpura

what is the localized form associated with

``` drugs bacteria viruses TB bacterial endocarditis ```

pathology of localized form of microscopic polyangiitis

fibrinoid necrosis acute inflammation (neutrophils) extravasation of RBCs

generalized form of microscopic polyangiitis

systemic hypersensitivity polyarteritis | microscopic polyarteritis

what is the generalized form associated with

``` collagen vascular disease SLE RA HSP malignancy dysproteinemia ```

pathology of generalized form of microscopic polyangiitis

arterioles and small arteries MPA-ANCA, PR3-ANCA kidney, rapidly progressing glomerulonephritis and renal failure skin involvement

microscopic features of leukocytoclastic vasculitis (localized microscopic polyangiitis)

``` fibrinoid necrosis of vessel wall intramural neutrophilic infiltrates leukocytoclasis (cell death) RBC extravasation involvement of small venules ```

thromboangiitis obliterans (bueger)

occlusive inflammatory disease of medium and small arteries in the distal arms and legs

epidemiology of buerger

smokers, young middle aged men

pathogenesis of buerger

smoking (tobacco products) cell mediated immunity, collagen types 2, 3 genetic factors (Israeli, Indians, Japanese) HLA A9, B5

pathology of buerger

acute inflamm of medium and small arteries thrombosis, infarcts segmental (acute or chronic) gangrene, amputations

clinical features of buerger

25-40 y/o men, smokers intermittent claudication, Raynaud phenomenon (loss of feeling and blueness of extremities) painful ulcers, gangrene of toes/fingers