VASCULAR PATHOLOGY

ATHEROSCLEOROSIS

Name 3 types:

§    Atherosclerosis

§    Mönckeberg medial calcific stenosis

§    Arteriolosclerosis

 

What are the components of a well developed atheromatous plaque?

§    Fibrous cap: endothelial cells, macrophages, foam cells, lymphocytes, collagen, proteoglycans, elastin, neovascularization

§    Necrotic centre: foam cells, cell debris, cholesterol clefts, calcium

 

What are some features of a complicated atheromatous plaque?

§    Calcification

§    Rupture, fissure, ulceration, hemorrhage

§    Thrombosis,

§    Aneurysm

HYPERTENSION

Pathology

Hyaline arteriolosclerosis (seen in HTN and DM)

 

Hyperplastic arteriolosclerosis:

§    features seen in malignant HTN: onion skinning, concentric laminated thickening of blood vessels

§    +/- necrotizing arteriolitis

 

Give 2 prototypical examples of vasculitis for large medium and small sized blood vessels.

LARGE-SIZED (2)

GIANT CELL ARTERITIS

§    Large/medium blood vessel (temporal, ophthalmic artery and extracranial branches of carotid a.)

§    Nodular thickenings of blood vessel →patchy (2cm biopsy)

§    M/Granulomatous inflm OR panarteritis. Chronic: collagenous thickening of the intima

Clinical

§    Facial pain, headache, diplopia

§    Rapidly progressive dementia[1]

TAKAYASU ARTERITIS

§    Large and medium vessel “Granulomatous inflammation of the aorta and its branches”

§    Women < 40

§    May look just like GC arteritis (granulomatous inflammation and adventitial/medial mononuclear infiltrate) SO, age and sex of patient imp.

§    At risk: aortic dilatation and aortic insufficiency

§    Clinical

Ø   Called “pulseless” disease because of decreased pulses in the upper extremities. Also at risk for HTN, ocular defects (blindness, retinal hemorrhages), pulm. HTN from involvement of pulmonary arteries

MEDIUM-SIZED (2)

POLYARTERITIS NODOSA

§    Medium-small vessels

§    Young adults. Remittent.

§    Dx. Biopsy of kidney or skin nodules.

§    M/ “poly” inflm: nφ, eφ, + fibrinoid necrosis → transmural

§    Segmental involvement of the blood vessel.

§    Later…fibrosis with mono. infiltrate

§    finally…fibrosis

§    All stages of disease in same or different vessel!!!

§    At risk…

§    Aneurysmal dilation of blood vessel.

§    HTN

§    Melena

§    renal problems

KAWASAKI DISEASE

§    Large, medium, small arteries

§    Clin: young children: fever + oral erythema + erythema of hands and feet “mucocutaenous”

§    + cervical l.n. enlargement

§    Histo: like PAN but with less fibrinoid necrosis.

§    Coronary artery aneurysm (during subsiding phase)→ may lead to thrombus formation →and MI

SMALL-SIZED (3)

ANCA-related

Pauci-immune (type III of the RPGNs)

MICROSCOPIC POLYANGIITIS

§    p-ANCA

§    small vessels (arterioles and venules, capillaries)

§    lesions of same age

§    hypersensitivity reaction

§    leukocytoclastic vasculitis +/- fibrinoid necrosis

§    Necrotizing glomerulitis (90%) (Type III pauci-immune RPGN)

§    cutaneous leukocytoclastic vasculitis→ purpura

CHURG-STRAUS

§    + eosinophilia, asthma + extravascular granulomas

WEGENER GRANULOMATOSIS

§    Define

§    Granulomatous vasculitis

§    small-medium blood vessel

§    40s

§    Triad:

§    acute necrotizing granulomatous inflammation (poor defined granulomas) → UPPER AND LOWER RESPIRATORY TRACT : bilateral infiltrates, sinusitis, mucosal ulceration: cANCAs “cankers”

§    DDx in lung is lymphomatoid granulomatosis (nodules of lφ and pφ’cytoid cells with atypia→ this dz 50% get NHL)

§    focal necrotizing or granulomatous vasculitis → BLOOD VESSELS OF THE LUNG + OTHER (geographic necrosis)

§    DDx TB, fungal (b/c cavitary ctr.)

§    focal or necrotizing GN:: RPGN→KIDNEY GLOMERULI (pauci-immune)

§    c-ANCA

THROMBOANGIITIS (BUERGER DISEASE)

§    Small and medium vessels (tibial and radial arteries)

§    Heavy smoking

§    Very segmental distribution

§    Acute and chronic inflammation in walls

§    Thrombusà microabscess of PMN surrounded by granulomas

Clinical

§    Claudication

OTHER

§    Mixed cryoglobulinemia

§    HSP (sm blood vessel, no ANCA)

§    AI : Rheumatoid arthritis, SLE

§    Infectious

POPLITEAL ARTERY ENTRAPMENT SYNDROME

§    From abnormal insertion of gastrocnemius muscle causing arterial trauma if muscles gets hypertrophiedà common in athletes

Treatment

§    Surgical release of muscle or bypass

DDx

§    Buerger

§    Emboli

§    PVD

§    Cystic adventitial disease

ANEURYSMS

Name 5 types of aneurysms

§    Atherosclerotic[2]

§    Syphilitic

§    Congenital vascular aneurysm

§    Post-MI aneurysm

§    Others: mycotic, vasculitis, traumatic

SYPHILITIC ANEURYSM (luetic aneurysm)

Clinical

§    Tertiary syphilis

Manifestations

§    Syphilitic aortitis

§    Luetic aneurysm[3], +/-AV dilation[4]

§    Cor bovinum[5]

Micro

§    Aortitis starts as adventitial inflammation involving vasa vasorumà obliterative endarteritis[6][7]à aortic medial ischemia[8]à tree barking[9]à aneurysmal dilation of ascending aorta and dilation of AV ring

Complications

§    Mediastinal compression

§    Dysphagia

§    Respiratory difficulty

§    Pain

§    LVH from AV insufficiency

§    MI if coronaries compromised

§    Rupture of aneurysm (rare)

ABDOMINAL AORTIC ANEURYSM

3 gross types

§    Saccular

§    Fusiform

§    Cylindrical

Micro: what’s important to rule out? Germs responsible?

§    Medial destructionà weakens wall

§    Associated with mural thrombi

§    Atheromatous ulcersà at risk for thrombi

Ø   Inflammatory AA (lφ, pφ, GC)

Ø   Infected AAA (salmonella in AAA)

§    Must rule out infectious origin (TB, gram negative bacilli[10],

Complications of AAA

§    Rupture, death

§    Occlusion of BLOOD VESSELs

Ø   Impingement on ureter, vertebrae

Ø   Embolism

AORTIC DISSECTION

Name 4 main causes

§    HTN (95%)

§    Cystic medionecrosis (degeneration): Marfan’s

Ø   Loss of elastin fibers, amorphous ECM material deposition

§    Secondary to cannulation injury

§    Pregnancy

Clinical

§    Excruciating pain

Ø   Begins at anterior chest

Ø   Radiates to back between scapulae

Ø   Confused with MI

§    Not associated with atherosclerosis

§    Classification

Ø   Type A

§    Proximal to subclavian a.

§    More common

§    AKA DeBakey I and II

Ø   Type B

§    Begins distal to subclavian a.

§    AKA De Bakey III

§    COD: rupture into pericardial, pleural, or peritoneal cavities

Ø   Cardiac tamponade

Ø   Aortic insufficiency

Ø   Myocardial infarction

 

 

 

 

THROMBOPHELBITIS

§    Risks

§    CHF, obese, pregnancy, post-sx, stasis,

§    legs: swelling, pain, PE

§    Migratory thrombophlebitis → Trousseau’s sign (thromboses which appear and dissolve). Secondary to procoagulant release in neoplasms

SVC SYNDROME

§    obstruction of SVC from bronchogenic ca or mediastinal neoplasms

§    dusky cyanosis, dilation of head and neck veins

IVC SYNDROME

§    compression of IVC

§    edema

§    proteinuria

LYMPHANGITIS

§    most common cause gp A strep

LYMPHEDEMA

§    Primary: Milroy dz (congenital ~)

§    Secondary: neoplastic, post-irr, fibrosis, post thrombosis and fibrosis, filariasis

TUMORS

HEMANGIOMA

Capillary Hemangioma

§    skin, internal viscera

§    strawberry ~ (newborns)

§    few mm

§    thin epithelial covering

§    closely packed blood vessel with scant stroma

§    pyogenic ~ : on a stalk, lips, gingival, +/- pregnancy

Cavernous

§    face, extremities

§    1-2cm

§    dilated blood vessel with thromboses or dystrophic calcification, scant CT

LYMPHANGIOMA

§    Capillary and Cavernous (Cystic Hygroma)

§    cap ~: no RBC

§    Cystic hygroma: newborns, on the side of the neck or axilla (up to 15cm)

GLOMUS TUMOR

§    painful, subungual

§    actin +

§    M/ branching vascular channels + surrounding glomus cells (nests/aggregates of round or cuboidal with scant cytoplasm)

VASCULAR ECTASIAS

Nevus Flammeus

§    dilation of preformed vessels

§    port wine stain: Sturge Weber (CN V) (assoc./ seizures and MR)

Spider telangiectasia

§    blanch when pressed

§    pregnancy or cirrhosis-related (sec. to estrogens)

Hereditary Hemorrhagic Telangiectasia

§    telangiectasia all over the place (skin, organs)

BACILLARY ANGIOMATOSIS

§    Secondary to Bartonella genus.

§    gross: looks like pyogenic granuloma (raised nodule)

§    micro: proliferation of epithelioid blood vessel +neutrophils+ dust + bacteria with W.S. silver stain

KAPOSI SARCOMA

Name the four types of KS

§    Chronic (old Ashkenazi Jews)

§    Endemic/African

§    Transplant

§    HIV-related

Describe the 3 stages of tumor progression

§    HHV-8

§    Distal extremities

§    Patch: dilated and slightly irreg. blood vessel with stromal lφ,mφ,pφ

§    Plaque: dilated and jagged blood vessel lined by plump spindle cells + stromal plump spindle cells +RBCs +lφ +pink globules

§    Nodule: slit like spaces + cells + stromal plump spindle cells +RBCs +lφ +pink globules + m.f.

Ø  -mets and lymph node

HEMANGIOENDOTHELIOMA

§    in b/w hemangioma and angiosarcoma

§    epithelioid hemangioendothelioma (blood vessel. lined by plump epithelioid endothelial cells)

ANGIOSARCOMA

§    Hepatic ~: secondary to PVC, Thorotrast

§    Others: secondary to lymphedema post mastectomy, radiation

§    Gross: multiple red nodules that coalesce to form large fleshy mass with hemorrhage and necrosis

§    M/ variable.

§    Poor px. mets and lymph nodes

BALLOON ANGIOPLASTY

§    Risks: dissection (plaque rupture, medial dissection, stretching of the media), restenosis

SAPHENOUS VEIN GRAFT

§    1. Thrombotic occlusion 2. Intimal fibrous HP 3. Atherosclerosis 4. Neointima (near anastomosis) + pseudointima

§    saphenous v. graft restenosis faster than internal mammary

CALCIPHYLAXIS

§    Seen in chronic renal failure patients

§    Unexpectedly slough skin from abdomen or legs

§    Occurs with peripheral vascular disease and may explain some poor healing

DD

§    Fat necrosis

§    Panniculitis

§    HIT/warfarin skin necrosis

Treatment

§    Support

§    Hyperbaric O2

TUMORS OF LARGE BLOOD VESSEL

§    Intimal sarcoma[11]

GIANT CELLS IN LUNG BLOOD VESSEL WALLS

§    Pulmonary HTN[12]

§    PVOD

§    Foreign body

§    IVDU

§    Wegener

§    Infection (TB)

§    Hypersensitivity pneumonitis

§    Sarcoidosis