Pathology: Ch. 10 Blood Vessels

Front 1

Where are dissecting aneurysms most frequently seen?

Back 1

In the ascending aorta. There is tearing of the elastic and muscular tissues resulting in rupture into the pericardial sac.

Front 2

Site of vascular leakage and leukocyte emigration during inflammation?

Back 2

Postcapillary venules.

Front 3

What is the most common type of vasculitis?

Back 3

Giant-Cell (Temporal) Arteritis

Front 4

Giant cell(temporal) arteritis

Back 4

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

Front 5

Takayasu arteritis is also known as?

Back 5

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

Front 6

Polyarteritis Nodosa

Back 6

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

Front 7

Kawasaki disease

Back 7

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)

Front 8

What is the most common cause of acquired heart disease in children?

Back 8

Kawasaki disease

Front 9

Kawasaki disease

Back 9

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)

Front 10

What is the most common cause of acquired heart disease in children?

Back 10

Kawasaki disease

Front 11

Microscopic polyangitis

Back 11

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

Front 12

Wegener's granulomatosis

Back 12

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

Front 13

Churg-Strauss syndrome

Back 13

children and adults

small vessel vasculitis involving skin, lung, heart vessels

clinical findings: allergic rhinitis, asthma, p-ANCA antibodies, eosinophilia

Front 14

Thromboangitis obliterans (Buerger's disease)

Back 14

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)

Front 15

Henoch-Schonlein purpura

Back 15

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

Front 16

What is the most common vasculitis in children?

Back 16

Henoch-Schonlein purpura

Front 17

Cryoglobulinemia

Back 17

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

Front 18

What are cryoglobulins?

Back 18

Immunoglobulins that gel at cold temperatures

Front 19

Infectious vasculitis

Back 19

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

Front 20

Raynaud's disease

Back 20

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

Front 21

Raynaud's phenomenon

Back 21

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

Front 22

Sturge-Weber syndrome

Back 22

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)

Front 23

Spider Telangiectasia

Back 23

caused by an AV fistula; disappears when compressed

seen on face, neck, upper chest

associated w/hyperestrinism (pregnancy, cirrhosis)

Front 24

Hereditary Telangiectasia

Back 24

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

Front 25

Bacillary angiomatosis

Back 25

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)

Front 26

What is the most prevalent malignancy in AIDS patients in the U.S.?

Back 26

Kaposi Sarcoma