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28 Cards in this Set
- Front
- Back
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Late: start at vasculitis: immunological role
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What are Antineutrophil Cystoplasmic Antibodies?
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Auto-Ab's react to enzymes in nphils and endothelial cells
Useful for diagnosing vasculities |
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Giant Cell Arteritis:
Vessels Affected Populations Affected Effects Histology |
Large vessel vasculitis (can affect aorta, mostly affects medium-sized and small arteries--carotid, esp cranial arteries--ophthalmic)
Disease of older people, can cause blindness Nodular swellings (grossly) can narrow lumen and form thrombi Mech: Immune Tx: Steroids Histology: granulomatous inflammation plus giant cells OR nonspecific artheritis w/lymphs, macs, nphils, ephils sans giant cells |
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Takayasu Artheritis:
Vessels Affected Populations Affected Histology |
Large vessel dz affects aorta, medium branches of arch
Pulseless dz; affects upper extremities Mostly affects young women Histology: adventitial, medial mononuc infiltrate cuffing of vasa vasorum giant cells and medial necrosis Hard to distinguish from giant cell arteritis; need demographic part to make dx |
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Polyarteritis Nodosa:
Vessels Affected Populations Affected Histology |
Involves small to medium sized arteries
Segmental pattern, not circumferential NO involvement of arterioles ANCA is NEGATIVE Can appear in skin, peripheral nerves; 30% positive for Hep B Ag |
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Kawasaki Disease
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AKA mucutaneous LN syndrome
Affects CHILDREN Appears in clusters, may be autoimmune Acute necrotizing vasculitis of entire wall, often coronaries; later dev't of aneurysms |
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Microscopic Polyangitis
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Small vessel vasculitis
Restricted to arterioles, capillaries, venules Lung involved, kidney involved with necrotizing glomerulonephritis Histo: polys, nphils, fibrinoid necrosis, NO granulomas p-ANCA positive Syx; Palpable purpura |
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Churg-Strauss Syndrome
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Wall infiltration by polys, fibrinoid necrosis
Has granulomas and TONS of EPHILS in vessel walls and surrounding tissues Ephils in blood too! likely to involve lungs p-ANCA positive |
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Wegener Granulomatosis
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Small to medium-sized arteries and veins
Respiratory and renal involvement Strong association with c-ANCA Histology: Granulomas, lymphs, macs, giant cells Vascular and tissue giant cells (need to dx/dy from Tb and fungal infections) |
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Diseases associated with c- and p-ANCA
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Microscopic polyangiitis – p-ANCA
Churg-Strauss syndrome - p-ANCA Wegener granulomatosis - c-ANCA |
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Thromboangitis Obliterans
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Dz of young smokers
Thromboses in medium and small arteries, esp radial and tibial arteries |
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Aneurysm:
True vs False |
True: localized dilation in which all layers of vessel wall or heart involved
Mostly due to atherosclerosis (thins media) False: Extravascular hematoma communicating with chamber/lumen to rupture of wall of vessel or heart chamber |
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True Aneurysms:
Fusiform vs Saccular |
Fusiform: shaped like spindle, tapered at both ends; involves entire circumference
Saccular: involves portion of circumference; spherical, often filled with thrombus |
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Berry Aneurysm
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Thin-walled outpouching at branch points in circle of willis; rupture occurs at apex of sac
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Aortic Dissection:
Definition Cause |
Blood enters defective media through tear; media splits into inner and outer layers
Dissecting blood can re-enter lumen or rupture into surrounding tissue/body cavity Cause: HTN in older pts Younger pts, due to rare diseases (genetic defect of collagen, fibrillin or elastin) Cystic medial degeneration: elastic tissue fragmentation with replacement by ECM ; not truly cystic |
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Myxoma
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Most common heart tumor in adults
Grows from endocardium |
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Lipoma
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Occur anywhere in heart
ENcapsulated Lipoamtaous hypertrophy of septum Myocytes entrapped in mass and can result in arrythmia |
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Hamartoma
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excessive overgrowth of cells in proper location in body
(mass of tissue gone awry) |
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Cardiac rhabdomyoma
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Hamartoma of fesutses and infants
Benign histologically but can be fatal depending on size/location Gray-white masses in left heart Often part of tuberous sclerosis (hamartoma syndrome)--AD but de novo in 2/3 of cases |
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Fibroma
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Tumor of fibrous tissue w/benign histology
Usually in ventricular myocardium, esp septum Assocd ith Gorlin syndrom (assocs with bifid ribs, multiple nevoid BCC, jaw cysts) |
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Malignant Cardiac Neoplasms
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Primary are rare
Mets very more common; can be of any germ layer from any non-CNS organ |
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Vascular Tumors
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Benign: easily recognizable vessels formed
Malignant: more cellular, atypia and/or mitoses |
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Hemangiomas
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Benign, common in kids
Can be in skin, but also occur in internal organs |
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Capillary vs Cavernous Hemangioma
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Cavernous: large, dilated, vascular channel; Von Hippel-Landau Syndrome
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Lymphangioma
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Lymphatic analogues of hemangiomas
Most often in head/neck Capillary and Cavernous Cavernous = cystic hygromas |
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Glomus Tumor
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Painful small lznz on digits
Dervied from glomus body |
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Angiosarcomas
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Can be due to arsenic, thorotrast, polyvinyl chloride (hepatic angiosarcomas)
Differs from well-diff'd to anaplastic |
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Kaposi Sarcoma
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Chronic in older men (European): multiple red-purple lesions/extremities
Lymphadenopathic KS (African): in younger people; sparse skin lesions Transplant-assocd KS: prominent in skin of IC'd transplant pts AIDS-assocd KS: 1/3 of AIDS pts Human Herpes Virus 8 in 95% of KS lzns! |
