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26 Cards in this Set
- Front
- Back
Where are dissecting aneurysms most frequently seen?
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In the ascending aorta. There is tearing of the elastic and muscular tissues resulting in rupture into the pericardial sac.
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Site of vascular leakage and leukocyte emigration during inflammation?
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Postcapillary venules.
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What is the most common type of vasculitis?
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Giant-Cell (Temporal) Arteritis
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Giant cell(temporal) arteritis
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Adults > 50 years old
A granulomatous large vessel vasculitis involving superficial temporal and ophthalmic arteries temporal headache, jaw claudication, blindness on ipsilateral side, polymyalgia rheumatica(muscle & joint pain with normal serum creatine kinase), increased ESR Tx: corticosteroids |
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Takayasu arteritis is also known as?
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aka "pulseless disease"
in young Asian women and children a granulomatous large vessel vasculitis involving aortic arch vessels absent upper extremity pulse, discrepancy in BP between arms > 10 mm Hg, visual defects, stroke. Tx: corticosteroids |
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Polyarteritis Nodosa
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middle-aged men; 30% have HBsAg-Ab immune complexes
necrotizing medium-sized vessel vasculitis; renal, coronary, mesenteric arteries involved. Pulmonary a. are spared. Vessels at all stages of acute & chronic inflammation Focal vasculitis produces aneurysms(detected with angiography) Organ infarction in kidneys, heart, bowels(bloody diarrhea), skin(ischemic ulcer), testicle(pain) Diagnosis: biopsy of lesions Tx: corticosteroids & cyclophosphamide |
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Kawasaki disease
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children< 5 years; boys> girls.
cause is unknown; kids of Asian descent have highest incidence necrotizing medium-sized vessel vasculitis involving coronary arteries(i.e. thrombosis & aneurysms) fever, erythema, edema of hands and feet convalescing with desquamative rash; cervical lymph node swelling, oral erythema and cracking of lips Abnormal EKG(i.e. acute MI) Tx: IV immunoglobulin; aspirin; corticosteroids contraindicated(danger of vessel rupture) |
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What is the most common cause of acquired heart disease in children?
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Kawasaki disease
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Kawasaki disease
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children< 5 years; boys> girls.
cause is unknown; kids of Asian descent have highest incidence necrotizing medium-sized vessel vasculitis involving coronary arteries(i.e. thrombosis & aneurysms) fever, erythema, edema of hands and feet convalescing with desquamative rash; cervical lymph node swelling, oral erythema and cracking of lips Abnormal EKG(i.e. acute MI) Tx: IV immunoglobulin; aspirin; corticosteroids contraindicated(danger of vessel rupture) |
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What is the most common cause of acquired heart disease in children?
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Kawasaki disease
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Microscopic polyangitis
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affects everyone (children & adults); precipitated by drugs(penicillin), infection(streptococci), immune disorder(SLE)
small vessel vasculitis of skin, LUNG, brain, GI tract, postcapillary venules, GLOMERULAR capillaries vessels are at SAME stage of inflammation; Palpable Purpura, Glomerulonephritis, p-ANCA antibodies in >80% of cases |
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Wegener's granulomatosis
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affects childhood to middle age
Necrotizing medium and small-sized vessel vasculitis involving LUNG necrotizing GRANULOMAS in skin, upper respiratory tract (saddle nose deformity, chronic sinusitis, collapse of trachea), lower respiratory tract (cavitating nodular lesions) Necrotizing vasculitis in lungs (infarction, hemoptysis), kidneys (crescentic glomerulonephritis), c-ANCA, correlates erratically w/therapy Tx: corticosteroids, cyclophosphamide 3 C's: c-ANCA, corticosteroids, cyclophosphamide |
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Churg-Strauss syndrome
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children and adults
small vessel vasculitis involving skin, lung, heart vessels clinical findings: allergic rhinitis, asthma, p-ANCA antibodies, eosinophilia |
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Thromboangitis obliterans (Buerger's disease)
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Men 25-50 years old who smoke cigarettes; highest prevalence in Middle East, Far East, India
medium-sized vessel vasculitis with digital vessel thrombosis and damage to neurovascular compartment Resting pain on forefoot is characteristic w/possible ischemic ulcers or gangrene of foot/toes; upper limb ischemia in 1/2 of PT with ulceration and gangrene; Raynaud's phenomenon Tx: smoking cessation essential; IV iloprost(PG analogue) |
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Henoch-Schonlein purpura
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children and young adults; males> females, most common vasculitis in children; IgA-anti-IgA immune complexes
small vessel vasculitis involving skin, GI, renal, joint vessels often follows a viral URI, group A strep pharyngeal infection pathogens may act as antigen trigger that causes Ab formation leading to immunocomplex formation PALPABLE purpura of butt and lower extremities Polyarthritis, nephropathy, GI bleeding often seen may have spontaneous recovery in 4 months w/o therapy Tx: corticosteroids if severe GI or renal disease |
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What is the most common vasculitis in children?
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Henoch-Schonlein purpura
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Cryoglobulinemia
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adults only
associated with HepC virus, type I membranoproliferative glomerulonephritis, multiple myeloma(monoclonal type) small vessel vasculitis involving skin, GI tract, renal vessels PALPABLE purpura, acral cyanosis of nose and ears, Raynaud's phenomenon, glomerulonephritis, arthritis, abdominal pain |
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What are cryoglobulins?
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Immunoglobulins that gel at cold temperatures
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Infectious vasculitis
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children and adults, involves all microbial pathogens
small vessel vasculitis involving skin vessels. Rocky Mtn spotted fever- tick transmission of Rickettsia rickettsii which invades endothelial cells and causes vasculitis; petechia begin on palms and spread to trunk Disseminated meningococcemia due to Neisseria meningitidis; capillary thrombosis produces petechia and confluent ecchmoses |
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Raynaud's disease
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Young women; exaggerated vasomotor response to cold or stress
medium-sized vessel vasculitis involving digital vessels in fingers and toes; sometimes also tip of nose and ears Paroxysmal digital color changes (white-blue-red sequence) ulceration and gangrene in chronic cases Tx: Avoid cold temps; Calcium channel blockers |
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Raynaud's phenomenon
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adult men and women; secondary to other disease (i.e. systemic sclerosis, CREST syndrome, SLE)
medium-sized vessel vasculitis involving fingers and toes, sometimes tip of nose and ear systemic sclerosis and CREST syndrome: digital vasculitis w/vessel fibrosis, dystrophic calcification, ulceration, gangrene Tx: Avoid cold temps; Calcium channel blockers |
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Sturge-Weber syndrome
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aka encephalotrigeminal angiomatosis
uncommon congenital disorder w/aberrant mesoderm and ectoderm development Clinical pic: venous angiomatous masses in cortical leptomeninges, ipsilateral facial port wine nevi, mental retardation, seizures, hemiplegia, skull radio-opacities *large facial vascular malformation in a retarded child--> hints towards a more extensive vascular malformation(Sturge-Weber) |
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Spider Telangiectasia
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caused by an AV fistula; disappears when compressed
seen on face, neck, upper chest associated w/hyperestrinism (pregnancy, cirrhosis) |
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Hereditary Telangiectasia
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aka Osler-Weber-Rendu disease
autosomal dominant dilated capillaries and veins present from birth found in mouth, GI tract, respiratory, urinary tracts associated w/iron deficiency anemia if lesions rupture, can cause serious epistaxis, GI bleed, hematuria |
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Bacillary angiomatosis
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opportunistic infection in immunocompromised PT
caused by Gram negative bacilli of Bartonella henselae (cat scratch disease) and Bartonella quintana ("trench fever" transmitted by lice) skin lesions w/red papules, nodules or masses; capillary proliferation w/epithelioid ECs showing nuclear atypia and mitoses diagnosis: PCR Tx: macrolide antibiotics (erythromycin) |
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What is the most prevalent malignancy in AIDS patients in the U.S.?
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Kaposi Sarcoma
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