Vascular Pathology

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jknell
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194054
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Vascular Pathology
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2013-01-20 20:06:32
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  1. Basic Principles of Vasculitis
    • Three layers of arterial wall:
    • -Intima
    • -Smooth Muscle
    • -Adventitia

    • Clinical Presentation
    • -Nonspecific sx: fever, fatigue, weight loss, myalgias
    • -Organ ischemia: due to thrombosis or fibrosis
  2. Large Vessel Vasculitis
    • Temporal (Giant Cell) Arteritis
    • Takayasu Arteritis
  3. Medium Vessel Vasculitis
    • Polyarteritis Nodosa
    • Kawasaki Disease
    • Buerger Disease
  4. Small Vessel Vasculitis
    • Wegener Granulomatosis
    • Microscopic Polyangitis
    • Churg Strauss Syndrome
    • Henoch-Schonlein Purpura
  5. Temporal (Giant Cell) Arteritis
    Granulomatous vasculitis that involves branches of the carotid artery, segmental lesions

    Epidemiology: female, > 50 years

    • Presentation:
    • -Unilateral HA
    • -Jaw claudication
    • -Vision problems (Blindness)
    • -associated with polymyalgia rheumatica

    • Lab Findings:
    • -elevated ESR (>100)
    • -bx: giant cells and intimal fibrosis
    • -negative bx does not rule out!!!

    • Treatment:
    • -Corticosteroids (empiric)
  6. Takayasu Arteritis
    Granulomatous vasculitis that involves aortic arch and branch points

    Epidemiology: Young Asian females

    • Presentation:
    • -pulseless disease
    • -fever
    • -night sweats
    • -arthritis
    • -myalgias
    • -skin nodules
    • -visual and neurologic symptoms

    • Lab Findings:
    • -elevated ESR

    • Treatment:
    • -corticosteroids
  7. Polyarteritis Nodosa
    Necrotizing vasculitis involving multiple organs, sparing the lungs

    Epidemiology: young adults, associated with Hepatitis B seropositivity

    • Presentation:
    • -hypertension (renal artery involvement)
    • -abdominal pain with melena
    • -neurologic dysfunction
    • -cutaneous eruptions

    • Lab Findings:
    • -transmural fibrinoid necrosis
    • -lesions of different ages
    • -string of pearls (aneurysms and constrictions)

    • Treatment:
    • -Corticosteroids
    • -Cyclophosphamide
  8. Kawasaki Disease
    Epidemiology: Asian children, < 4 years

    • Presentation:
    • -fever
    • -conjunctivitis
    • -enlarged cervical LNs
    • -Strawberry tongue
    • -hand-foot erythema

    • Complications:
    • -coronary aneurysms, thrombosis (MI)

    • Treatment:
    • -IVIg
    • -ASA
  9. Buerger Disease
    Necrotizing vasculitis involving digits

    Epidemiology: heavy smokers, males, <40

    • Presentation:
    • -gangrene
    • -autoamputation of digits
    • -superficial nodular phlebitis
    • -Raynaud's (neural involvement)

    Treatment: smoking cessation
  10. Wegener Granulomatosis
    Necrotizing granulomatous vasculitis involving nasopharynx, lungs and kidneys

    Epidemiology: middle aged men

    • Presentation:
    • -nasopharyngeal ulceration, otitis media, chronic sinusitis, mastoiditis
    • -hemoptysis, cough, dyspnea
    • -hematuria

    • Lab Findings:
    • -c-ANCA
    • -CXR: large nodular densities

    • Treatment:
    • -Cyclophosphamide
    • -Corticosteroids
  11. Microscopic Polyangiitis
    Necrotizing vasculitis involving especially lung and kidney

    • Presentation:
    • -palpable purpura
    • -similar to Wegener
    • -but NO nasopharyngeal involvement

    • Lab Findings:
    • -NO granulomas
    • -p-ANCA

    • Treatment:
    • -corticosteroids
    • -cyclophosphamide
  12. Churg-Strauss Syndrome
    Necrotizing granulomatous inflammation with eosinophils affecting especially lung and heart

    • Presentation:
    • -asthma
    • -sinusitis
    • -palpable purpura
    • -peripheral neuropathy

    • Lab Findings:
    • -granulomatous vasculitis with eosinophilia
    • -p-ANCA

    ***distinguish from Microscopic Polyangiitis (asthma, granulomas, eosinophilia)
  13. Henoch-Schonlein Purpura
    Epidemiology: most common form of childhood systemic vasculitis

    • Pathophysiology:
    • -IgA immune complex deposition usually following URI

    • Presentation (Triad):
    • -palpable purpura on buttocks and legs
    • -GI pain and melena
    • -Arthralgia

    -often associated with IgA nephropathy

    • Treatment:
    • -often self limited-Steroids
  14. Sturge-Weber Disease
    • Pathophysiology:
    • -congenital disorder that affects capillaries

    • Presentation:
    • -port-wine stain (nevus flemmeus) on face
    • -ipsilaterial leptomeningeal angiomatosis
    • -seizures
    • -early onset glaucoma
  15. Vascular Tumors
    • Strawberry hemangioma
    • Cherry hemangioma
    • Pyogenic granuloma
    • Cystic hygroma
    • Glomus tumor
    • Bacillary angiomatosis
    • Angiosarcoma
    • Lymphangiosarcoma
    • Kaposi's Sarcoma
  16. Strawberry Hemangioma
    Benign capillary hemangioma of infancy

    Appears in first few weeks of lifegrows rapidly and regresses spontaneously around 5-8yrs

    blanchable
  17. Cherry Hemangioma
    Benign capillary hematoma of the elderly

    Does NOT regress

    Increased frequency with age
  18. Pyogenic granuloma
    Polypoid capillary hemangioma

    May ulcerate and bleed

    Associated with trauma and pregnancy
  19. Cystic hygroma
    Cavernous lymphangioma of the neck

    Associated with Turner Syndrome
  20. Glomus Tumor
    Benign, painful, red-blue tumor under fingernails

    Arises from modified smooth muscle cells of glomus body
  21. Bacillary angiomatosis
    Benign capillary skin papules found in AIDS patients

    Caused by Bartonella henselae

    Don't confuse with Kaposi Sarcoma
  22. Angiosarcoma
    Malignant proliferation of endothelial cells

    Highly lethal malignancy of liver

    Associated with Polyvinyl chloride (PVC), arsenic and thorotrast exposure
  23. Lymphangiosarcoma
    Lymphatic malignancy associated with persistent lymphedema

    ie: post radical mastectomy
  24. Kaposi's Sarcoma
    Low grade malignant proliferation of endothelial cells

    Associated with HHV-8 infection

    • Seen in:
    • -old, eastern european males
    • -AIDS
    • -transplant recipients

    NOT blanchable

    Don't mistake for bacillary angiomatosis

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