Block 6 - Path - Vasculitides

Front 1

granulomatous (in inner media) inflammation ususally in older patients and is associated with polymyalgia rheumatica - nodules or tenderness

Back 1

Giant Cell (temporal) arteritis

Front 2

Granulomatous inflammation in patients usually younger than 50, pulseless disease, aortic arch, intimal thickening, decreased BP in UE vs. LE

Back 2

Takayasu's Arteritis

Front 3

Necrotizing (fibrinoid)inflammation typically involving renal arteries but usually sparing pulmonary vessels. Hits small and medium sized muscular arteries, can lead to aneurysm, impaired perfusion, primarily in young adults

Back 3

PAN

Front 4

Arteritis with mucocutaneous lymph node syndrome usually in children. May involve coronary arteries with aneurysmal formation and or thrombosis. Delayed HS rxn of T cells (Ag likely viral). Thickening of entire artery wall.

Back 4

Kawasaki Disease

Front 5

Necrotizing small vessel vasculitis with few or no immune deposits, necrotizing arteritis of small and medium sized arteries can occur. Necrotizing glomerulonephritis and pulmonary capillaritis are common, and are associated with MPO-ANCAs - P-ANCAs segmental fibrinoid necrosis "pauci-immune" injury

Back 5

Microscopic Polyangiitis

Front 6

Granulomatous inflammation involving the respiratory tract and necrotizing vaculitis affecting small vessels, including glomerular vessels, Associated with PR3-ANCAs - cANCAs

Affects men > women

"Crescentic glomerulonephritis" often due to T cell HS rxn from inhaled toxins

Back 6

Wegener Granulomatosis

Front 7

Eosinophil-rich granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small vessels. Associated with asthma and blood eosinophilia. Associated with MPO-ANCAs

Ususally no renal disease

Back 7

Churg - Strauss

Front 8

Migratory Thrombophlebitis, often seen with adenocarcinoma

Back 8

Trousseau Sign

Front 9

neoplasmic compression - dilation of veins of the head, neck, arms, with cyanosis, pulmonary vessels also affected

Back 9

SVC syndrome

Front 10

Occurs with hepatocellular, renal cell carcinoma also thrombosis, causes distension of superficial collateral veins of the lower abdomen, massive proteinuria with renal involvement

Back 10

IVC syndrome

Front 11

Often due to group A beta hemolytic strep. red painful subQ streaks that follow vessels, nodal enlargement, possible bactermia or sepis

Back 11

lymphangiitis

Front 12

cand be due to primary milroy disease (lymphatic agenesis or hypoplasia) or secondary due to blockage (tumor, post op, fliariasis, inflammation) can cause "peau d'orange" apperance of skin, ulceration, or chylous build up (in abdomen, pericardium, and thorax)

Back 12

Lymphedema

Front 13

hemangioma, lymphangioma, glomus tumor, vascular ectasias

Back 13

benign

Front 14

kaposis, hemangioendothelioma

Back 14

intermediate

Front 15

angiosarcoma, hemangiopericytoma

Back 15

malignant

Front 16

most common - skin, subQ, mucous membranes, liver, spleen, kidneys - some spontaneous regression

bright red-blue, unencapsulated, little CT, hemosiderin pigment due to rupture

Back 16

Capillary Hemangioma

Front 17

red-blue, spongy - intravascular thrombosis, dystrophic calcification, vulnerable to trauma, problematic if in the brain

seen with von Hippel Lindau disease

Back 17

Cavernous Hemangioma

Front 18

benign but very painful, from SMCs, ussually found in distal digits, excision is curative

Back 18

glomus tumor

Front 19

usually regresses, aberrant ectodermal/mesodermal development causes port wine stain

if trigeminal involvement/distribution could be "Sturge- Weber" with extensive vascular malformation, mental retardation

Back 19

Nevus Flammeus Vascular Ectasias

Front 20

Often occurs in pregnancy or cirrhosis (i.e. hyperestrogenic state) on face, neck, upper chest

non-neoplastic

Back 20

spider telangiectasia

Front 21

AD disorder, dilated capillaries and veins - skin and oral mucosa, respiratory, GI, urinary tracts

can hemorrhage

Back 21

Osler-Weber-Rendu Disease

hereditary hemorrhagic telangiectasia

Front 22

Patches - difficult to distinguish from granulation tissue

Back 22

Early Kaposis

Front 23

Plaques - proximal spread - hemosiderin laden macrophages, lymphs, and plasma cells, pink hyaline globules

Back 23

middle stage Kaposis

Front 24

Nodular - neoplastic, proliferative

In AIDS - often accompanied by nodal and visual involvment

Back 24

Late Kaposis

Front 25

Can begin insidiously

Malignant endothelial neoplasms - spectrum from hemangioma to anaplasticity

Ususally occurs in older adults - at any site (most often skin, soft tissue, breast and liver)

Back 25

Angiosarcoma

Front 26

Due to carcinogenic exposure (arsenic, thorotrast, PVC, and radiation)

can also occur post radical mastectomy

Back 26

Hepatic Angiosarcoma

Front 27

focal, irregular thickening of the walls of medium and large muscular arteries, including renal, carotid, splanchnic, and vertebral arteries

cause unknown

Back 27

Fibromuscular Dysplasia

Front 28

PDGF, endothelin -1, thrombin, fibroblast growth factor, IFN gamma, IL 1

Back 28

promoters

Front 29

heparin sulfates, NO, TGF-B

Back 29

inhibitors

Front 30

Homogenous, pink hyaline thickening with associated luminal narrowing. Changes result from protein leakage across injured endothelial cells with deposition in wall, and increased smooth muscle cell matrix synthesis in response to chronic hemodynamic stress

Back 30

Hyaline Arteriolosclerosis

Front 31

Malignant HTN - "onion skin" lesions - concentric, laminated thickening of walls and luminal thickening. Laminations - smooth muscle cells with thickened, reduplicated basement membranes, which may be accompanied by fibrinoid deposits and vessel wall necrosis (necrotizing arteriolitis) particularly in the kidney. Lumina may be completely obliterated.

Back 31

Hyperplastic Arterioloscleosis

Front 32

Isolated foam cell lesion

Back 32

Type I

Front 33

Fatty streak lesion

Back 33

Type II

Front 34

Intermediate lesions

Back 34

Type III

Front 35

Atheroma

Back 35

Type IV

Front 36

Fibroatheroma

Back 36

Type V

Front 37

Complicated Lesion

Back 37

Type VI

Front 38

adventitial inflammation involving aortic vasa vasorum with resultant obliterative endaeteritis causing restriction of blood flow to the outer 2/3 of the aortic medial wall, patchy elastic fiber loss

Back 38

syphilitic (luetic) aneuysms