Peripheral Arterial Disease Aortic Aneurysms / Vasculitis Peter B. Baker, MD.
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Transcript of Peripheral Arterial Disease Aortic Aneurysms / Vasculitis Peter B. Baker, MD.
Peripheral Arterial Disease Aortic Aneurysms / Vasculitis
Peter B. Baker, MD
Objective for Part 1
1. Describe aneurysms of the peripheral vessels by type and morphology
2. Differentiate atherosclerotic aneurysm, syphilitic aneurysm and dissecting hematoma by pathology, clinical presentation and prognosis
3. Describe the life threatening cardiovascular manifestations of Marfan syndrome
Types of AneurysmsHandout I.A
True aneurysm
Aneurysm wall has all 3 layers False aneurysm
Aneurysm wall is adventitia only
Types of Aneurysms
Vascular Aneurysms
Etiologies Atherosclerosis Syphilis Vasculitis (ie PAN) Infection (mycotic) Trauma Congenital defect
Atherosclerotic Aortic AneurysmHandout I.C
Men > Women - familial Abdominal aorta most common site Pathogenesis involves medial atrophy Complications emboli (athero- or thrombo-) branch occlusion compression of adjacent structure risk of rupture increases with aneurysm size
4 – 4.9 cm, 1% per year5 – 5.9 cm, 11% per year> 6 cm, 25% per year
Atherosclerotic Aneurysm
AtheroscleroticAneurysm
AbdominalAorta
Atherosclerotic Aneurysm - HistologyAtherosclerotic Plaque
Atherosclerotic Aortic Aneurysm
Syphilitic Aortic AneurysmHandout I.D
Thoracic aorta Pathology inflammation of vasa vasorum obliterative endarteritis ischemic necrosis of media fibrosis (“tree bark”) of intima Complications compression of adjacent structures aortic valve regurgitation
Syphilitic Aortic Aneurysm
Syphilitic Aortitis
Vasa Vasorum
Syphilitic Aortitis
Obliterative EndarteritisObliterative Endarteritis in Syphilitic Aortitis
Dissecting Aortic HematomaHandout I.E
Pathology intimal tear split between middle and outer third of the media media may be normal or show degenerationComplications rupture – hemorrhage branch obstructionPredisposing conditions hypertension connective tissue disorders with medial degeneration
Aortic Dissection Classification Based on Extent of Involvement
Dissecting Aortic Aneurysm Gross Specimen
H
Aorta
PA
Dissecting Hematoma Extending Into Common Iliac Arteries
H
H
Dissecting Hematoma Extending Into Common Iliac Arteries
Dissecting Hematoma of the Aorta – Histologic Section
Lumen
MediaDissection
Dissecting Hematoma - Aorta
Aorta – Normal Media (Elastic Stain)Media of Normal Aorta
Aorta with Severe Medial DegenerationAorta with Medial Degeneration
Aorta with Severe Medial Degeneration – Colloidal Iron Stain
Aorta with Medial Degeneration – Colloidal Iron Stain
Objective for Part 2
Describe the etiology, pathology and selected clinical presentations of vasculitides involving large, medium size and small arteries
Causes of VasculitisHandout II.A
Infection Immunologic mechanisms Radiation Trauma Caustic substances Unknown
Vasculitis – PathogenesisHandout II.A - B
Immune complexes circulating or form in-situ Antineutrophilic cytoplasmic antibodies (ANCA) perinuclear (pANCA, Myeloperoxidase-ANCA) – microscopic polyangiitis cytoplasmic (cANCA, Proteinase3-ANCA) – WegenerAntiendothelial antibodiesCell-mediated immunity
Vasculitis-Classification
Large Vessel Giant cell (temporal) TakayasuMedium – sized vessel Polyarteritis nodosa KawasakiSmall – sized vessel Microscopic polyangiitis Wegener granulomatosis
Temporal ArteritisHandout II.C.1
Etiology – unknown ?T-cell mediated injury Clinical – rare before age 50, F > M, headache,
visual disturbances, fever, weight loss, swelling over temporal artery
Pathology - Granulomatous or nonspecific chronic inflammation
- Intimal proliferation / fibrosis with luminal narrowing
- Segmental distribution
Temporal Arteritis - Histology
L
Temporal Arteritis
Temporal Arteritis - HistologyTemporal Arteritis
Takayasu ArteritisHandout II.C.2
Etiology – unknown Clinical – age < 40 years, visual disturbances,
weak arm pulses, neurologic manifestations, hypertension
Pathology - Involves aortic arch and branches
- May involve descending aorta and pulmonary artery
- Intimal fibrosis
- Granulomatous or nonspecific chronic inflammation
Takayasu Arteritis with Severe Narrowing of the Carotid Artery
Takayasu Arteritis - Histology
A
Takayasu Arteritis
Polyarteritis NodosaHandout II.D.1
Etiology – unknown Clinical
- most often in young adults, occasionally in pediatric
age or older adults
- can be acute, relapsing or chronic
- manifestations include hypertension, abdominal pain,
renal failure, GI hemorrhage, muscle pain, neuritis,
fever, weight loss, infarcts
- 30% have circulating HBsAg-HBsAb immune
complexes
Polyarteritis NodosaHandout II.D.1
Pathology
Medium to small muscular arteries involved
Vasculitis is haphazard and segmental
Frequency: kidney > liver > heart > GI
Early findings - fibrinoid necrosis, neutrophils
Vasculitis heals by fibrosis
May develop thrombosis, aneurysm formation
Polyarteritis Nodosa in a Small Muscular Artery
Polyarteritis Nodosa
Polyarteritis Nodosa in a Small Muscular Artery
Polyarteritis Nodosa
Polyarteritis Nodosa with Aneurysm and Thrombosis
Thrombus
Polyarteritis Nodosa with Aneurysm and Thrombus
Hemorrhagic Small Bowel Infarction in Polyarteritis Nodosa
Polyarteritis Nodosa with Early Healing
Intima
Lumen
Polyarteritis with Early Healing
Allergic Granulomatosis (Churg-Strauss)Handout II.D.1.d
Pathology similar to PAN and microscopic polyangiitis Bronchial asthma / allergic rhinitis / eosinophilia / pANCA
in <50% Granulomas / infiltration of vessels by eosinophils Involves lung, heart, spleen, peripheral nerves, skin
Kawasaki DiseaseMucocutaneous Lymph Node SyndromeHandout II.D.2 Etiology – ?T-cell response to an infection Clinical – Infants and children, fever, oral mucosa
erythema, skin rash, lymphadenopathy, erythema of palms and soles
Pathology
Coronary artery vasculitis (accounts for fatalities in 1 – 2%)
Microscopic PolyangiitisHandout II.E.1
Etiology – reaction to an antigen / pANCA Clinical – skin rash, other organs Specific disorders – H-S purpura, cryoglobulinemia,
malignancy Pathology Involves microvasculature Fibrinoid necrosis Karyorrhexis of PMN’s (leukocytoclastic)
Skin Biopsy with Microscopic Polyangiitis
Skin Biopsy with Microscopic PolyangiitisMicroscopic Polyangiitis
Wegener GranulomatosisHandout II.E.2
Etiology – cANCA Clinical – sinusitis, pneumonitis, renal failure,
glomerulonephritis Pathology
- Distribution - kidneys / upper and lower
respiratory tract
- Necrotizing granulomas
- Vasculitis with fibrinoid necrosis
Wegener’s granulomatosis – Small Artery with Fibrinoid Necrosis
Wegener Granulomatosis
Wegener Granulomatosis
Thromboangiitis Obliterans Buerger DiseaseHandout II.E.3
Etiology – unknown / ? genetic influence Clinical – cigarette smoking, < 35 years, claudication of
extremities, ischemic ulcers, gangrene Pathology
Vasculitis with thrombosis
PMN collections in thrombi
Wegener Granulomatosis
Aneurysms Vasculitis Quiz
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