Cv Pathophys Non-Atherosclerotic Vascular Disease Chart

Front 1

Calcific Medial Sclerosis
(Monckeberg Disease)

Arteriosclerosis

Back 1

Deposits of calcium within the media of small to medium-sized muscular arteries – usually an incidental finding

Front 2

Abdominal Aortic Aneurysm (AAA)

Aneurysm

Back 2

Atherosclerosis in older male smoker w/genetic predisposition -> progressive thinning & destruction of media

Thin-walled, fusiform

Complications:
- Rupture
- Vascular occlusion
- Embolism (renal, low extre)
- Compression of neighboring structures

Front 3

Syphilitic (Luetic) Aneurysms

Aneurysm

Back 3

Obliterative endarteritis of vasa vasorum of the aorta (tertiary syphilis) -> ischemic damage to aortic media
Plasma cells Fusiform dilation of aortic & thoracic aorta w/ a white folded appearance to intima (“tree-barking”

1) aortic adventitia
small vessels of the vasa vasorum surrounded and narrowed by lymphocytes and plasma cells
2) aortic media
scattered patches of smooth muscle cell loss associated with inflammation and, subsequently, fibrosis
3) aortic intima
ribbon-like (scarring & contracts) effect with alternating bulges into the lumen

Complications:
a. aortic insufficiency due to dilatation of the valve ring  volume
overloaded left ventricle and heart failure
b. superimposed atherosclerosis -> coronary artery ostial narrowing
-> ischemic heart disease
c. compression or erosion of adjacent structures (e.g., bronchi,
esophagus, recurrent laryngeal nerves, vertebrae)

Front 4

Aortic Dissection

Back 4

Hypertension, Inherited CT disorder (“cystic” medial degeneration), peripartum, iatrogenic

a. * proximal (type A) aortic dissection
involving the ascending aorta +/- descending aorta
I – ascending II – ascending & descending
b. distal (type B) aortic dissection
only involving the descending aorta

Signs & symptoms: severe chest pain radiating to back & extending downward

Intima tear – blood inner 2/3 outer 1/3
"cystic" medial degeneration – fragmentation and loss of elastic tissue within the aortic media with replacement by pale, amorphous, extracellular matrix

Complications:
Rupture – pericardial sac, double barreled aorta

Aortic insufficienct

External vascular compression of aortic branches

Other: renal infarcts & MI

Front 5

Vasculitides

Back 5

vascular inflammation and injury -> luminal narrowing, thrombosis &/or aneurysm formation (with risk of rupture) -> ischemia of the tissue in the distribution of the involved vessel (+/-
hemorrhage) -> constitutional symptoms (fever, malaise, weight loss); occasionally myalgias, arthralgias and neuralgias; and signs referable to the specific foci of ischemic damage

Front 6

Temporal Arteritis

Vasculitides with granulomatous inflammation

Back 6

- Temporal & ophthalmic arteries
- >50 yr olds
- Medium – large arteries

Vascular pathology:
typically granulomatous inflammation lined up along the internal elastic lamina causing its focal destruction, followed by fibrosis of the vessel wall; associated with wall thickening and luminal narrowing of the involved vessel (+/- thrombosis)

Clinical Features:
Painful & nodular temporal artery, headache &/or visual impairment (necessitating immediate treatment)

Front 7

Takayasu Arteritis
(“pulseless disease”)

Vasculitides with granulomatous inflammation

Back 7

- Aortic Arch & major branches (includes temporal)
- Young Women <50

Vascular pathology:
mononuclear inflammation with giant cells extending through the entire thickness of the vessel wall, along with fibrosis; associated with wall thickening and luminal narrowing
(+/- thrombosis)

Clinical features:
Neurologic &/visual deficits, weak upper extremity pulses, symptoms of myocardial ischemia, &/or hypertension (renal arteries)

Front 8

Wegener Granulomatosis

Vasculitides with granulomatous inflammation

Back 8

- c-ANCA : proteinase-3 (PR3)
- Middle aged Men
- Responds well to immunotherapy
- Necrotizing granulomas inside & outside vessels

Vascular pathology:
1) necrotizing granulomatous inflammation of the respiratory tract (upper &/or lower)
2) necrotizing granulomatous vasculitis of small vessels, especially in the respiratory tract (upper &/or lower)
3) necrotizing, proliferative glomerulonephritis  hematuria, proteinuria &/or renal failure

Front 9

Polyarteritis Nodosa

Vasculitides with acute & chronic inflammation

Back 9

- Smaller to medium-sized arteries of kidney, GI, Heart (NOT the lungs)
- Immune complexes – Type 3 hypersensitivity
- Responds well to immunotherapy
- Young adults (20-30s)

Vascular pathology:
patchy, transmural, acute and chronic inflammation with associated fibrinoid necrosis and thrombosis, alternating with regions of vascular wall fibrosis -> aneurysm formation, rupture &/or luminal narrowing, with associated
tissue ischemia

Clinical features:
Remittent episodes of fever, malaise and myalgias, associated with
symptoms / signs suggestive of multifocal vascular injury
(e.g., hypertension, abdominal pain and GI bleeding)

Front 10

Kawasaki Disease
(mucocutaneous lymph node syndrome)

Vasculitides with acute & chronic inflammation

Back 10

- Coronary arteries
- Young children (<4-5 yr olds)
- Anti-endothelial cell antibodies
- Usually self limited but can have problems w/ coronary arteries

Vascular pathology: Similar to polyarteritis

Clinical features:
Persistent fever, erythema of various mucosal surfaces (red conjunctiva, strawberry lips), palms, soles; desquamating skin rash; peripheral edema; cervical lymphadenopathy; &/or cardiovascular complications (e.g., coronary artery aneurysms, myocardial ischemia, sudden death)

Front 11

Microscopic polyangiitis (leukocytoclastic vasculitis)

Vasculitides with acute & chronic inflammation

Back 11

- p-ANCA – myeloperoxidase (MPO)
- hypersensitivity vasculitis
widely disseminated arterioles, capillaries and venules (small vessels)

Vascular pathology:
similar to that of polyarteritis nodosa, except for
the predominant involvement of smaller
vessels, the presence of fragmented
neutrophils (leukocytoclasia) and the similar
"age" of the lesions

Clinical Features:
development of hemorrhagic complications (e.g.,
palpable purpura, hemoptysis, hematuria), frequently status/post exposure to a new antigen (e.g., drug, malignancy)

Front 12

Henoch-Schonlein Purpura

Vasculitides with acute & chronic inflammation

Back 12

- Small blood vessels, esp the skin, GI & kidney
Necrotizing vasculits w/ deposition of IgA- containing immune complexes
- Glomeruli
Mesangial deposits of IgA immune complexes often associated with hypercellularity, predominately in the mesangium (similar to that of IgA nephropathy)
- Children (M>F) Good prognosis
Adults if severe renal disease

Clinical features:
palpable purpura (e.g., buttocks, dorsal legs and arms), arthralgias, abdominal pain, intestinal
bleeding, &/or renal abnormalities (e.g., hematuria, proteinuria, hypertension, edema)

Front 13

Thromboangiitis obliterans (Buerger Disease)

Vasculitides with acute & chronic inflammation

Back 13

- Small to medium-sized arteries of distal extremities
- young heavy smoking men

Vascular pathology:
segmental, transmural, acute and chronic inflammation; luminal thrombosis and microabscesses (followed by fibrous scarring), with extension into adjacent veins and nerves

Clinical features:
Distal extremity ulcers and gangere, associated with severe pain

Front 14

Churg-Strauss Syndrome
(allergic granulomatous angiitis)

Vasulitis with eosinophilic-predominant inflammation

Back 14

- occasionally p-ANCA myeloperoxidase (MPO)
- rare, highly variable disorder of middle-aged adults

Clinical Features:
phase 1 – allergic rhinitis, sinusitis and asthma
phase 2 – peripheral hypereosinophilia (in blood
and tissues, commonly the lungs and
gastrointestinal tract)
phase 3 – extravascular necrotizing granulomas and a systemic small-vessel necrotizing vasculitis (with eosinophils and granulomas)
involving the lungs, skin, peripheral and central nervous systems, heart, gastrointestinal tract,
kidneys, &/or musculoskeletal system