Vascular Pathology Flashcards
Large-vessel vasculitis
involves the Aorta and its major branches
Medium-vessel vasculitis
Involves muscular arteries that supply organs
Small-vessel vasculitis
Involves arterioles, capillaries, and venules
- Involvement of **branches of carotid artery, large to small arteries **of the head, intimal thickening, Granulomatous
- Older adults ( > 50 y.o.), mostly females
- Headache (temporal artery involvement)
- Visual disturbances (opthalmic artery involvement)
- Jaw claudication
- Flu-like symptoms with polymyalgia rheumatic
- ESR elevated and T-cell mediated immune response
- Biopsy: giant cells and intimal fibrosis, segmental lesions
- Tx: corticosteroids, high risk for blindness, surgery
Temporal arteritis
(Giant Cell)
- Granulomatous vasculitis (Aortic arch branch points) and Coronary and Renal arteries, aneurysm formation and dissection: Large to Medium
- Transmural fibrous thickening of Aorta and branches
- Adults ( < 50 y.o.), young asian females, and children
- Visual and Neurologic symptoms
- ‘pulseless disease’ - weak or absent **upper extremity, **fever night sweats, arthritis, myalgias, skin lesions
- ESR is elevated
- Tx: corticosteroids
Takayasu Arteritis
- Fibrinoid necrotizing vasculitis, multiple organs w/ varying stages of acute, chronic, and fibrosing lesions in Medium to Small arteries among All Ages
- Lungs are NOT involved, NOT a/w ANCA
- Young adults (w/ HTN) (renal artery involvement)
- Mesenteric artery involvement (abdominal pain w/ melena (black ‘tarry’ feces)
- Neurologic disturbances and headache
- Skin lesions, weight loss, malaise, microaneurysms
- A/w Hepatitis B Surface Antigen (HBsAg), HBV
- Transmural inflammation - ‘String of pearls’
- Tx: corticosteroids and cyclophoshamide, FATAL
Polyarteritis Nodosa
- Asian, children < 4 years old
- Large to Small arteries - ‘strawberry tongue’
- Non-specific signs: Acute febrile illness ‘Fever’, Conjuctivitis, Erythematous ‘skin’ rash on palms and soles, cervical lymph nodes , Lymphadenopathy
- Coronary artery involvment
- Thrombosis w/ myocardial infarction
- Aneurysm with rupture
- Tx: Aspirin (TXA2) and IV immunoglobulin- disease is self-limited
Kawasaki Disease
- Necrotizing vasculitis involving the digits
- Ulceration, gangrene, and autoamputation of fingers and toes (Small / Medium sized arteries of extremities)
- Superficial nodular phlebitis
- Thrombus and microabscesses
- Raynaud phenomenon is often present
- Males, < 40 y.o.
- Associated with heavy smoking
- Tx: smoking cessation
Buerger Disease
(Thromboangioitis obliterans)
-
Non- and Necrotizing granulomatous vasculatiis:
- Nasopharynx, Lungs, and Kidneys (‘palisading’)
- Middle aged male w/ sinusitis or nasopharyngeal ulceration (mainly adults), otitis media, mastoiditis
- Hemoptysis with bilateral nodular lung infiltrates
- Hematuria due to ‘rapidly progressive glomerulonephritis’
- Serum c-ANCA / PR3-ANCA correlate w/ disease activity
- Biopsy: large necrotizing granulomas w/ adjacent necrotizing vasculitis of small arteries, veins, capillaries
- Tx: cyclophosphamide and steroids, common relapse
Wegener Granulomatosis
-
Necrotizing vasculitis involving multiple organs
- arterioles, capillaries, and venules w/ **leukocytoclastic vasculitis **(Fragmentend / apoptotic neutrophils)
- Lung and Kidney, hemoturia, hemoptysis
- **MPO-ANCA / **p-ANCA **correlate w/ disease activity
- Nasopharyngeal involvement and granulomas absent
- Tx: corticosteroids and cyclophosphamide, immunosuppresion, Relapse is common
Microscopic Polyangiitis
- Necrotizing granulomatous inflammation w/ Eosinophils involving multiple organs (esp. Lungs, Heart, Kidney and GI (pauciimmune glomerulonephritis))
- **Asthma, sinusitis, palpable purpura, **and peripheral eosinophilia / neuropathy (wrist / foot drop)
- Serum MPO-ANCA / p-ANCA correlate with disease activity, increased IgE levels
Churg-Strauss Syndrome
- Vasculitis due to IgA immune complex deposition
- Most common vasculitis in children (2 - 6 y.o.)
- Palpable ‘purplish/red’ purpura on Buttocks (‘trunk’) and Legs (‘extremities’)
- GI pain, melena, multiple lesions, and bleeding
- Arthralgias
- Hematuria (IgA vasculitis / nephropathy)
- Following upper respiratory tract infection
- Self-limited
- Tx: steroids
Henoch-Schönlein Purpura (HSP)
- Stenosis decreases blood flow to glomerulus
- ATII raises blood pressure
- Contracting arteriolar smooth muscle –> increaseing total peripheral resistance
- Promoting adrenal release of Aldosterone, increases resorption of Na+ in distal convoluted tubule (expanding plasma volume)
- HTN with increased plasma Renin and unilateral atrophy (low blood flow) to affected kidney
- Different for Elderly Men and Young Females
Renal Artery Stenosis
Renal Artery Stenosis:
Elderly Men –> __________
Young Women –> __________
Renal Artery Stenosis:
Elderly Men –> Atherosclerosis
Young Women –> Fibromuscular dysplasia
- Blood pressure > 200 / 120 mmHg
- Acute end-organ damage
- Acute Renal failure
- Headache
- Papilledema
Malignant HTN –> medical emergency
-
Med/Lrg arteries w/ Intimal plaque
- Necrotic Lipid core w/ fibromuscular cap
- Often undergoes dystrophic calcification
- Abdominal aorta, Coronary artery, Popliteal artery, Internal carotid artery are commonly affected
- (4) Modifiable risk factors: HTN, Hypercholesterolemia (LDL increase, HDL decrease), Smoking, Diabetes
- Nonmodifiable: Age, Gender (Males and Postmenopausal), Genetics (History)
- Lipids oxidized –> macrophage laden –> Fatty Streaks (arise in teenagers) involves PDGF and FGF prolif.
Atherosclerosis
Complications of Atherosclerosis
- Peripheral vascular disease (extremity arteries)
- Angina (coronary arteries)
- Ischemic bowel disease (mesenteric arteries)
- Thrombosis –> myocardial infarction (coronary)
- Thrombosis –> stroke (middle cerebral artery)
- Plaque rupture w/ embolization –> embolus
- Weakening can result in aneurysm
- Proteins leaking into the vessel wall producing vascular thickening (pink on microscopy)
- Consequences of long-standing:
- Benign HTN (‘Systemic HTN’)
- Diabetes
- Associated with Nephrosclerosis –> Renal Failure
- Reduced vessel caliber w/ end-organ ischemia producing glomerular scarring (arteriolonephrosclerosis) that slowly progresses to Chronic Renal Failure
Hyaline Arteriolosclerosis
- Thickening of the vessel wall by hyperplasia of smooth muscle (‘onion skin’ appearance)
- Acute w/in 1-2 years
- Consequence of Malignant HTN
- Reduced vessel caliber with end-organ ischemia
- May lead to fibrinoid necrosis of the vessel wall w/ hemmorrhage –> acute renal failure w/ “flea-bitten’ appearance
- Life threatening organ damage: Brain, Kidneys, Heart –> Medical emergency
Hyperplastic arteriolosclerosis
- Calcification of the media of the arteries, especially the Radial or Ulnar (medium sized) arteries
- Nonobstructive
- Usually benign; “pipestem” arteries on x-ray
- Intima not involved
- Not clinically significant; seen as an incidental finding on x-ray or mammography
Mönckeberg medial calcific sclerosis
- Dissection through the media of the aortic wall
- Proximal 10 cm of the Aorta w/ preexisting weakness
- HTN (older adults), Bicuspid aortic valve
-
Connective tissue defects (younger individuals)
- Marfan Syndrome –> defective Fibrillin –> cystic medial necrosis of the Aorta
- Ehlers-Danlos Syndrome –> defective Collagen
- Sharp, tearing chest pain that radiates to the back
- Complications: Pericardial tamponade (death), Rupture w/ fatal hemorrhage, Obstruction of branching arteries (coronary and renal) –> end-organ ischemia
Aortic Dissection
- Balloon-like dilation of Thoracic Aorta
- A/w Cystic medial degeneration due to HTN (elderly)
- Tertiary syphilis –> Obliterative Endaorteritis of the vasa vasorum results in luminal narrowing, decreased flow, and atrophy of the vessel wall
- ‘tree-bark’ appearance of the Aorta
- Complication: Dilation of the Aortic valve root → Aortic valve insufficiency → pain
- Compression of the mediastinal structures (airway or esophogus) → shortness of breath
- Thrombosis / embolism
Thoracic Aortic Aneurysm
- Balloon-like dilation arises below the Renal arteries but above the Aortic bifurcation
- Primarily due to Atherosclerosis: Males, Smokers, > 60 years old, HTN
- Atherosclerosis increases diffusion barrier to the media
- -> atrophy and weakness of the vessel wall
- Pulsatile abdominal mass that grows with time
- Complications: when > 5 cm –> triad of hypotension, pulsatile abdominal mass, and flank pain
- Compression of structures (ureter), Thrombosis/emboli
Abdominal Aortic Aneurysm (AAA)
- Benign tumor comprised of blood vessels, Red papules or nodules
- Blanches when pressed
- Commonly present at birth
- Regresses during childhood
- Involves the Skin and Liver
Hemangioma
- Maliginant proliferation of Mesenchymal endothelial cells
- Highly aggressive, poorly demarcated, variegated, fleshy masses, atypical cells lining vascular lumen
- Older Adults (M = F), CD 31 / 34 Postiive
- Common sites: Skin, Breast, and Liver
- a/w Rx therapy after mastectomy
- Polyvinyl chloride (PVC) exposure to Liver
- Arsenic exposure
- Thorotrast exposure (radiocontrast agent)
- Poor prognosis
Angiosarcoma
- Low-grade malignant proliferation of endothelial cells
- Locally aggressive
- Purple patches, plaques, and nodules on the skin
- May involve visceral organs
- HHV-8 virus infects endothelial cells
- Seen in:
- Older Eastern European Males –> Surgery
- AIDS –> antiretroviral agents
- Transplant recipients –> decreasing immunosuppresion
Kaposi Sarcoma
- Directed toward neutrophil azurophilic granules (to increase PMN activity) a/w microbial peptides (polyangiitis)
- Primarily involves intracellular component –> plasma membrane expression –> leads to vascular injury
Anti-proteinase-3 (PR3-ANCA)
Non-infectious Vasculitis
- Directed toward lysosomal granules (oxygen free radical formation)
- Drug to cross-reactive microbial antigens
- Infection (elicit cytokines: TNF) & endotoxic exposure
- Autoantibodies (direct endothelial cell injury or reactive neutrophils
- Leads to vascular injury
Anti-myeloperoxidase
(MPO-ANCA)
Non-infectious Vasculitis
Benign Vascular Tumors
- Ectasias
- Telangiectasias
- Hemangiomas
- Pyogenic granulomas
- Lymphangiomas
- Glomangiomas
- Bacillary angiomatosis
Intermediate-grade Vascular Tumors
- Kaposi Sarcoma
- Hemangioendothelioma
Malignant Vascular Tumors
- Angiosarcoma
- Hemangiopericytoma
- Plaques or nodules composed of Lipid-laden Histocytes in the skin
- Especially the eyelids
- Looks like a rash of raised red nodules on the skin and fatty orange / yellow plaques on the eylids
Xanthomas
- Lipid deposits in Tendon, especially Achilles
Tendinous Xanthoma
-
Lipid deposit in Cornea
- Appears early in life w/ Hypercholesterolemia
- Cholesterol deposits in or hyalinosis of the corneal stroma
- Common in elderly (Arcus Senilis) and fades in infants
- A/w decreased blood flow to eye
- Appears as a white, grey, or blue opaque ring in the corneal margin (peripheral corneal opacity), or white ring in front of the periphery of the iris.
Corneal Arcus
- Systolic BP > 140 mmHg and/or Diastoloic > 90 mmHg
- Age, Obesity, Diabetes, Smoking, Genetics, Black > White > Asian
- 90% is Essential due to CO and TPR
- 10% is Secondary; Renal disease, including Fibromuscular dysplasia in young pts.
- A/w Atherosclerosis, LVH, Stroke, CHF, Renal failure, retinopathy, and Aortic Dissection
Hypertension (HTN)
- Common
- Two types:
- Hyaline (thickening of small arteries in essential HTN or Diabetes mellitus)
- Hyperplastic (‘onion skinning’) as seen in severe HTN
Arteriolosclerosis