vascular Pathology Flashcards
Response of vascular smooth muscle to injury (minor and major)
- in minor injuries: adjacent endothelial cells migrate to restor luminal surface
- in major injuries: smooth muscle is stimulated to migrate to intima (bad) and create collagen (worse)
Review:
- inter-endothelial attachment protein
- expressed in HEVs for niece Tcell attachment
- binds integrins for stable adhesion
- CD31
- CD34
- CD54
Stenosis outcomes
- chronic ischemia
- rupture -> thrombus
- complete progressive occlusion
5 leading Causes of death in 2010
1 heart disease 2 malignant neoplasms 3 chronic lower resp diseases 4 Cerebrovascular disease 5 accidents
Clinically significant stages of atherosclerosis
- fiberoatheroma (lipid core with fibrotic layer): stage IV
- complicated lesion with surface defect and adherent hematoma or thrombosis: stage V
Plaque structure
- cells (smooth muscle cells, foam cells, lymphocytes)
- matrix
- lipid (in necrotic core)
- neovascularization
- important: media is thinned at base of plaque: rupture/ulceration significant risk
Preclinical stage
- Generally 40-50 yearsish. Silent until age related comorbidities show up
- exception: familial hypercholesterolemia and TIIDM
Diagnosis of atherosclerosis
- angiography is gold standard
- one clear artery doesn’t guarantee another (patchy)
Tissue ischemia in atherosclerosis
- may be direct occlusion
- may also be decreased Plat in collateral vessels
- lower ext gangrenous ulcers and bowel ischemia are common presentations of mesenteries and LEx atherosclerosis
Risk factors for MandM in atherosclerosis
- synergistic
- HTN, cholesterol LDL/HDL ratio, smoking male, genetics
Heterozygous familial hypercholesterolemia
- LDL receptor mutation: LDL>220 Mg/dL
- 1:500
Familial defective apoprotein B
- Apo B 100
- 1:700
Familial combined hyperlipidemia
- unknown etiology
- LDL and trigs elevated
- HD! Suppressed
Inflammatory conditions effecting development if atherosclerosis
- high CRP
- homocysteine (B12 deficiency)
- lipoprotein LPa
Berry aneurysm
- incidence, location, rupture risk, clinical sequela
- 2 %
- 90% are in MCA/ACA near branch points
- Marfan and Ehlers danlos at increased risk for rupture
- results in subarachnoid hemorrhage
Fibromuscular dysplasia
- Segmental lesions affecting renal, carotid, splanchnic, vertebral
- renovascular HTN: decreased afferent pressure -> JG cells -> renin release
Etiology of HTN
- 90% essential: unknown
- secondary: renal lesions most common (glomerulonephritis, renal artery stenosis et al)
> CV: coarctation of aorta, poly arteritis nodosa
> endocrine
> Neuro: increased ICP
Hyaline arteriolesclerosis
- thickening of media and intima by hyaline material in HTN
- sequelae: diffuse renal ischemia, worsening HTN
True vs. false aneurysm
- true aneurysm has all three walls dilated
- false aneurysm has had the media and the intima separated
Cystic media degeneration
- ischemic degeneration of medial layer of aorta due to stenosis of vaso vasorum
- risk factor for AAA
Thoracic aortic aneurysm etiology and sequelae
- rare
- most are HTN in origin
- infectious etiology: syphillis
- sequelae: encroachment on esophagus, lungs, mediastinum, bones; aortic valvular insufficiency with LVH, coronary artery obstruction, rupture (uncommon)
Aortic aneurysms
Class A: surgical emergency
- proximal to descending aorta
Class B: distal to arch
Raynaud’s phenom vs disease
- disease is primary, less severe, presents younger and essentially an exaggerated vasomotor response
- phenomena is typically secondary to disease process (arterial stenosis) presents in more variable age group,
Vasculitis
- rarely infectious, typically immune mediated
- large: GTA (giant cell, takayasu’s, the other one)
- medium: PK (polyarteritis nodosa, kawasakis)
- small: Good Small White CHurch (goodpastures, SLE vasc, Wegeners, Churg-Strauss)
Polyarteritis nodosa
- NO LUNG involvement (pan no pulm)
- obliterated arterial lumen, trans mural neutrophils
- transmural necrotizing inflammation of muscular arteries (sans lung)
- on continuous inflammation (esp bifurcations)
- microaneurysms, ischemia with ulceration, hemorrhage with necrosis and infarct
Kawasakis disease
- aka mucocutanious lymphnode syndrome
- PAN-like arteritis of medium vessels in young kids
> mucocutaneous erythema, palmar and pedal erythema, desquamation - autoantibodies against smooth muscle
- cardiac sequelae most significant pathology (rare)
Cardiac sequelae of Kawasakis etiology and treatment
- coronary vasculitis
- coronary aneurysms (large)
- fatal in ~ 1%
- epidemic in Japan and Hawaii, but #1 acquired heart diseas in kids in us
- Aspirin and IVIG
Wegeners granulomatosis (it’s a klr)
- classic triad: upper resp lesions, lung lesions (necrotizing capsulitis) kidney lesions
- all three together are uncommon presentation clinically
Infectious arteritis
- bacteria or fungi esp. Aspergillus and mu or (angioinvasive)
- infarction and aneurysm rupture
Varicose veins
- pathenogenesis: venous stasis
- can be painful, unsightly
- pulmonary emboli rare (only 1 of three in virchows)
- varices in esophagus and anorectal junction due to portal HTN
Phlebothrmbitis (thrombophlebitis)
- not an inflammatory state, thrombosis within vein
- immobilization with inactivity
- Vircows triad: venous stasis, endothelial injury, hypercoagulable state
- most originate in deep leg veins
Clinical manifestations of thrombophlebitis
- few signs of early disease
- first symptom often embolus
- leg edema, redness, pain
- Homan sign: pain in popliteus upon abrupt dorsiflexion
Pulmonary thromboembolus
- # 1 cause of sudden death in post op PTs
- lines of Zahn in antemortem clots
- can be visualized in ventilation/perfusion scan
SVC/ IVC syndrome
- 90% neoplasm, 10% thrombosis
- facial edema, dilated head/neck veins (SVC)
- leg edema, distention of superficial abdomen, if renal veins effected massive proteinuria
> hepatocellular and renal cell CA
Lymphangiitis
- typically GAStrep
- inflammation along lymphatic drainage pathway
- bacteremia -> sepsis; monitor closely
Lymphadema
- primary is rare, defects in lymph drainage, or agenesis
- secondary much more common: inflammation or cancer
Weird complication of large hemangioma
- high output cardiac failure
- don’t remove heagioma unless necessary
Vascular ectasias
- talengectasia
- osler-weber-rendu disease: talengectasias of mouth and tongue; risk of lethal intestinal bleed
Bacillary angiomatosis
- opportunistic infection presenting as vascular proliferations of blood vessels
- Infection typically GNBac (often Bartonella)
Vascular tumors: intermediate grade and malignant
- intermediate: > kaposis sarcoma (assoc with HIV and HHV8) coalescing patches and plaques > hemagioendothelioma - malignant: > angiosarcoma > hemangiopericericytoma
Arterioloslerosis
- similar to atherosclerosis but generally localized t heart and kidneys
Boot shaped heart with
- overriding aorta, VSD, pulmonary stenosis r vent hypertrophy
- tetralogy of fallot
- untreated 30 year survival is ~ 10%
- right to left shunting
Neonat with lower body cyanosis
- coarctation of the aorta
Pulmonary atresia/stenosis
- results in post-stenotic pulmonary dilation
Circumferential infarct
- subendocardial infarction
> can be due to global hypoperfusion in presence of reduced atherosclerotic vessels
Kerley B lines on roentgenogram
- represent pulmonary edema (vascular and interstitial)
- especially in interlobar speta
Panners don’t mine for gold so polyart nod doesn’t involve lung. Wegener is the coal miner
,
Osler-Weber-Rendu disease
Oropharyngeal Talengectasias
- potentially lethal GI bleeds
C-ANCA/P-ANCA
- C-ANCA: Wegeners
- P-ANCA: microscopic polyangiitis, Churg-Strauss
Anti-endothelial cell Abs
Associated with kawasakis
Pulseless (weakened pulse in UEX), similar to giant cell arteritis except pathology in aorta
Takayasu’s
- more common in japanese
Hypersensitivity type as units of small/medium blood vessels, hemoptysis, hematuria, muscle pain/weakness, palpable cutaneous purpura
Microscopic polyangiitis
- fragmenting neutrophils: leukocytoklastic vasculitis
Allergic symptoms with eosinophilic rhinitis
- Churg- Strauss
- ANCAs probably responsible for vascular manifestation
Beurgers disease (thromboangiitis obliterans)
- smokers
- cold sensitivity, raynauds, chronic ulcers in fingers toes, feet
- stop smoking
Most common infectious vasculitis
- mucor/aspergillus
Most common vascular tumor
- hemangioma (capillary variant)
Pedunclulated rapidly growing hemangioma
- pyogenic granulomas
- can occur in pregnant women
Exquisitly painful benign tumor typically under finger nail bed
Glomus tumor
- excision is curative
Large facial vasicular lesion in presence of mental deficiency
- Sturge-Weber syndrome
kaposis sarcoma variants (4)
- KS preceded by HHV8 (requires a cofactor to progress as a tumor)
- chronic: Eastern European men, NOT assoc with HIV
- Lymph: African variant NOT assoc with HIV
- transplant assoc is aggressive: not assoc with HIV (immunosuppressive)
- AIDS assoc IS assoc with AIDS
Malignant tumor arising due to exposure to arsenic, radiation or PVC
- angiosarcoma
Fragmented neutrophils
- microscopic angiitis
Munkeyburg phenomena
- benign calcification of media of arteries not constricting flow.
Show up on x ray
