B4.071 - Vasculitides Flashcards
clinical features of GPA
males>F
hematuria, proteinuria, sinusitis, pneumonitis pulmonary infiltrate with cavitation
PR3-ANCA (cytoplasm)
pathology of microscopic polyangiitis
arterioles, small arteries
MPO-anca
PR3 - anca
kidney, RPGN, renal failure
skin involvement
pathology of giant cell arteritis
cordlike, nodular vessel with narrow lumen. Granulomatous inflammation, plasma cells, MO, eos, Multinucleated giant cells, fibrosis of media and thick intima
microscopic polyangiitis is associated with what
drugs (penicillin, thiazides), bacteria, viruses, TB, endocarditis
pathology of microscopic polyangiitis
fibrinoid necrosis, acute inflammation, extravasation of RBCs
epidemiology of polyarteritis nodosa &
etiology
men>women
white
immune complex mediated
30% of PAN pts have HBV
what is kawasaki disease
acute, necrotizing vasculitis of incancy and early childhood, with fever, rash, conjuctival and oral lesions (strawberry toungue) and lymphadenitis
what is PR3-ANCA
anti protease 3 (c-ANCA) Seen in granulomatosis iwth polyangiitis, formerly known as Wegeners granulomatosis
generalized form of microscopic polyangiitis
systemic HS
asociated with vascular disease, SLE, RA, SS
HSP, malignancy, dysproteinemis
what are types of medium vessel vasculitis
polyarteritis nodosa kawasaki disease
etiology of vasculitis
Infectious agents HBV, HSV, CMV, Parvovirus serum sickness bacterial antigens autoantibodies: ANCA
clinical features of PAN
young adults
acute, subacute or chronic
varies with affected site (kidney, Ht, liver, GI)
constitutional symptoms, infarcts, necrosis

giant cell arteritis
narrow lumen, inflammation and giant cells
clinical features of buergers syndrome
25-40 yo
men, smokers
intermittend claudication, raynaud syndrome
painful ulcers, gangrene of toes
pathogenesis of beurgers disease
smoking
cell mediated immunity, collagen types 2 and 3
genetic factors, HLA A9, B5

microscopic polyangiitis
what is thromboangiitis obliterans
buerger disease
occlusive inflammatory disease of medium and small arteries in distal arms and legs
what is wegners granulomatosis aka GPA
systemic necrotizing granulomatous vasculitis
who gets buerger disease
smokers, young middle aged men
what is MPO-ANCA
antimyeloperoxidase (p-ANCA) seen in microscopic polyangiitis and Churg Strauss syndrome
treatment of GPA
steroids
what is ANCA
autoantibodies against neutrophil granules, monocyte lysosomes, endothelial cells
what is giant cell arteritis
focal, chronic, granulomatous inflammation of temporal artery
treatment of churg strauss
steroids

giant cell arteritis
granulomas
what is pauci immune
non infectious vasculitis where you dont see any deposition of anything in the vessels
treatment of PAN
steroids and antiinflammatory, immunosuppressive
Treat HBV
what does wegners affect
lungs, kidney, upper respiratory tract

microscopic polyangiitis
note: extravasated RBCs and tons of neutrophils
what is vasclutitis
inflammation and necrosis of blood vessels (arteries, veins, capillaries)
how do you monitor an autoimmune vasculitis
ANCA MPO and PR3 titers, trends with disease activity
localized form of microscopic polyangiitsi
leukocytoclastic vasculitis
cutaenous necrotizing vasculitis
palable purpura
clinical features of takayasu
constitutiona
high BP, asymmetry, weak pulse
occular disturbances, cardaic symptoms, CHF, syncope, dizziness

ojo a lymphocytes breaking through the wall
PAN
what is churg strauss syndrome
systemic vasculitis with prominent eosinophilia that occurs in young persons with asthma or allergy
typical takayasu patient
young women
under 50
pulseless disease
classic patient presentation of giant cell arteritis
sudden blindness, temporal pain, headache

giant cell arteritis
what are causes of small vessel vasculitis continued
CVD, malignancy, mixed cryoglobulinemia radiation GPA, wegners lymphomatoid granulomatosis behcet disease duerger disease vascular allograft rejection
epidemiology of giant cell arteritis
older people 70-80 women>men
pathogenesis of giant cell arteritis
a form of T cell mediated proinflammatory cytokintes (TNF, HLA-DR4)
what is the most common form of vasculitis
giant cell arteritis
pathology of buergers disease
acute inflammation
thrombosis, infarcts
segmental (acute or chronic)
gangrene, amputation
treatment of giant cell artritis
steroids, anti TNF

left - vasculitis
right - necrotizing granuloma
GPA
what vasculitis is associated with HBV
PAN

giant cell arteritis
mixed inflammation and necrosis

gross appearance of purpura in microscopic polyangiitis
what part of the body is immune from PAN

lungs

PAN
fibrinoid necrosis
what are types of small vessel vasculitis
granulomatosis with polyangiitis (GPA) (WG) churg strauss syndrome microscopic polyandiitis
pathogenesis of noninfectious vasculitis
immune complex deposition in vessel wall antineutrophil cytoplasmic antibodies (ANCA) Direct attack of endothelial cells by circulating endothelial Abs Cell mediated immunity (T cell)
pathology of takayasu arteritis
aorta and branches
thick intima, thick wall
panarteritis, with giant cells
luminal narrowing, scarring
Pulseless disease
pulseless disease
takayasu
what is leukocytoclasis
cell death
pathology of churg strauss
necrotizing vasculitis of small/medium sized vessels
granulomas, eosinophilia
ubiquitous similar to microscopic angiitis or PAN
MPO-ANCA (para nuclear)
lungs, kidneys,m Ht, spleen, liver, CNS
treatment of takayasu
steroids

left - normal
right - giant cell arteritis
fragmentation of elastic lamina

giant cell arteritis
what arteries does giant cell arteritis affect
temporal artery, cranial, aorta

buergers syndrome

PAN
kidney
pathology of GPA
necrotizing vasculitis. parenchymal/geographic necrosis
granulomatous inflammation
crescentic glomerulonephritis
small arteries, veins and capillaries
what is microscopic polyangiitis
group of inflammatory vascular lesions affecting small vessels that are thought to represent a response to exogenous substances

takayasu
what are types of large vessel vasculitis
giant cell arteritis takayasu arteritis
symptoms of kawasaki
affects coronary arteries, aneurysms, rupture, death

takayasu
left - intimal proliferation
thick intima right
what is takayaso arteritis
inflammatory diesase of unknown etiology of large arteries (aorta and branches)
what is polyarteritis nodosa
acute necrotizing vasculitis of medium sized muscular arteries
etiology of kawasaki and treatment
delayed HS t cell reaction
infection with parvovirus B19
IVIG and aspirin

PAN
fibrinoid necrosis, mixed inflammatory cells
clinical features of giant cell arteritis
benign, self limiting 6-12 mo
muscle aches, polymyalgia rheumatica
visual symptoms, diplopia, blindness