Vascular Pathology (Pathoma) Flashcards
Vasculitis
Inflammation of blood vessel (arteriole wall)
Etiology is usually unknown
Most causes are not infectious
Nonspecific sx and sx of organ ischemia (key to sx of particular vasculititidies). Ischemia from thrombis or fibrosis.
Large vessel (aortic branches) ; medium vessel (muscular arteries) ; or small vessel (arterioles, capillaries, and venules)
Temporal (Giant Cell) Arteritis
MC form of vasculitis in older adults (>50); usually females
Branches of carotid artery
Symptoms: Headache (temporal); Visual disturbances (opthalmic); jaw claudication
Flu like sx w/ joint and muscle pain (PMR)
ESR is elevated ( rx if any suspicion before biopsy confirmation)
Takayasu Arteritis
Spectrum of Giant Cell (Granulomatous vasculitis in adults classic pt. is young asian female)
Affects major vessels coming off aortic arch.
Sx:
Visual and neurologic symptoms; Weak or absent pulse in an upper extremity (‘pulseless disease’)
ESR is elevated
Rx = steroids
Polyarteritis nodosa
Necrotizing vasculitis that can involve any organ except lung.
Presents in young adults w/:
HTN (renal artery); Abdominal pain w/ melana (mesenteric artery); Neurologic disturbances; skin lesions
Associated w/ serum HBsAG
Lesions are present in varying stages. “String of pearls” appearance on imaging. –> transmural fibrinoid necrosis (highlighter pink) and subsequent fibrosis. Combination of fibrosis and small aneurysm (nodes)
Rx: Corticosterois and Cyclophosphamide
Kawasaki disease
Classically affects Asian children thrombosis w/ MI or aneurysm w/ rupture.
Rx: Aspirin and IVIG (key ddx –> reye syndrome). Disease is self limited.
Remember kid sitting on motorcycle (palms and soles to drive). Heart goes up while driving motorcycle.
Buerger’s Disease
Necrotizing vasculitis involving digits
Presents w/ ulceration, gangrene, and autoampuation of fingers and toes.
Raynaud phenomenon is often present (white –> blue –> red)
Highly associated w/ smoking –> rx = cessation**
Weg(C)ener’s Granulomatosis (Granulomatosis w/ polyangitis)
Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys*
Presents w/ middle aged male w/ sinusitis or nasopharyngeal ulceration, hemoptysis w/ bilateral lung infiltrates, and hematuria due to RPGN
Key serum marker = Cytoplasmic anti neutrophil cytoplasmic antibody c-ANCA (levels correlate w/ disease activity)
Biopsy: Large Necrotizing Granulomas w/ adjacent necrotizing vasculitis
Rx: Corticosteroids and Cyclophosphamide. Relapses are common.
Necrotizing Polyangiitis
Necrotizing vasculitis involving multiple organs, especially the lung and kidney
Similar to Wegener’s, but no nasopharyngeal involvement or granulomas
Serum perinuclear- anti neutrophili cytoplasmic antibody (p-ANCA) levels correlate w/ disease activity
Rx: Cyclophosphamide and corticosteroids w/ common relapses
Churg-Strauss Syndrome
Necrotizing granulmatous vasculitis w/ eosinophils
Involves multiple organs, especially lungs and heart
Asthma and peripheral eosinophilia*
p-ANCA levels correlate w/ disease activity
Henoch Schonheim Purpura
Vasculitis due to IgA immune complex deposition
MC vasculitis in children
Presents w/ palpable purpura (bleeding and inflammation) on buttocks and legs, GI bleeding and pain, Hematuria (IgA nephropathy –> IgA in mesangium)
Usually post URI or enteritis (IgA production increased)
Disease is self limited and can recur. Steroids if severe.
Systemic HTN
BP >140/90. 20% of US population
Divided into primary or secondary
Primary HTN
95% of HTN; no known etiology
Risk factors: Age, race (blacks increased; asians decreased), obesity, stress, lack of physical activity, high salt diet (Na affects both both volume (systolic) and TPR (diastolic)).
Secondary HTN
HTN due to identifiable cause.
MC is Renal artery stenosis
Important to rule out.
Renal Artery Stenosis
Increased plasma renin –> RAAS –> increased blood volume and TPR ( caused by decreased blood flow hitting JGA), unilateral atrophy of affected kidney.
If bilateral –> ACEI’s contraindicated
Key causes:
Atherosclerosis (elderly males)
Fibromuscular dysplasia (young females)
Fibromuscular dysplasia
Cause of secondary HTN often in young females.
Due to congenital defect resulting in irregular thickening of medium sized vessels (especially renal artery)
Strings on peal appearance on CT w/ contrast
Benign HTN
Mild or moderate elevation of bp. MC form of
Clinically silent; vessels and organs are damaged over time
Hyaline arteriolosclerosis
Malignant HTN
Severe elevation of bp (>200/120)
May arise from preexisting HTN or de novo
Present w/ acute renal failure, headache, and papilledema.
Fibrinoid arteriolosclerosis (Onion skinning)
Medical emergency
Rx: Sodium nitroprusside.
Arteriosclerosis
Three pathologic patterns:
- )Atherosclerosis -thickening of intima* of blood vessel walls. Usually in medium and large vessels.
- ) Arteriolosclerosis - Thickening of arterioles either due to protein (hyaline) or thickening of smooth muscle (fibrinoid)
- ) Monckeberg medial sclerosis - Calcification of media of vessel walls. Not super clinically signifcant.
Atherosclerosis
Intimal plaque that obstructs blood flow. Mostly consists of necrotic lipid core (cholesterol that can be dystrophically calcified) w/ smooth muscle cap.
Invovles large/medium sized arteries. 4 MC are Abdominal aorta, coronary, popliteal, and internal carotid.
Risk factors:
Modifiable: HTN, Hypercholestemia, smoking, DM
Nonmodifiable: Age, gender (males; estrogen is protective; genetics (family hx is key)
Pathogenesis: Oxidized lipids deposit in intima and are phagocytosed by mphages –> fatty streak (teenagers) –> inflammation and healing –> fibromuscular cap –> narrowed lumen.
Complications: Account for >50% of disease in western world.
Stenosis of medium sized vessels –> peripheral vascular disease; angina; ischemic bowel disease. No sx until >70% stenosis ***
Plaque rupture (at neck) w/ thrombosis; MI, stroke
Plaque rupture w/ embolization. Results in atherosclerotic emboli (hallmark is cholestrol clefts in emboli)
Weakening of vessel wall (thick wall –> ischemia of wall beyond intima)–> aneurysm
Arteriolosclerosis
Narrowing of small arteries
Divided into hyaline and hyperplastic types:
Hyaline - caused by proteins leaking into the vessel wall –> thickening –> pink protein deposits on bx. Due to DM (NEG of BM) and benign HTN (pressure pushes proteins in wall)***
Results in reduced vessel caliber w/ end-organ ischemia. Classically produces glomerular scarring. Progresses to CKD ( thus DM and HTN are 2 MCC of CKD).
Presents w/ shrunken kidney and scarring of cortex.
Hyperplastic - involves thickening of smooth muscle creating onion-skin appearance. Due to malignant HTN.*
Results in reduced vessel caliber w/ end organ ischemia as well. May lead to fibrinoid necrosis of vessel wall. Classically causes ARF w/ “flea bitten” appearance (petichiae)
Hyaline arteriolosclerosis
Caused by proteins leaking into the vessel wall –> thickening –> pink protein deposits on bx. Due to DM (NEG of BM) and benign HTN (pressure pushes proteins in wall)***
Results in reduced vessel caliber w/ end-organ ischemia. Classically produces glomerular scarring. Progresses to CKD ( thus DM and HTN are 2 MCC of CKD).
Presents w/ shrunken kidney and scarring of cortex.
Hyperplastic atherosclerosis
involves thickening of smooth muscle creating onion-skin appearance. Due to malignant HTN.*
Results in reduced vessel caliber w/ end organ ischemia as well. May lead to fibrinoid necrosis of vessel wall. Classically causes ARF w/ “flea bitten” appearance (petichiae)
Monkeberg Medial Calcific Sclerosis
Calcification of media; non-obstructive.
Not clinically significant.
Incidental finding on XR or Mammography*.
Aortic Dissection
Intimal tear w/ dissection of blood through the media of the aortic wall.
Occurs in proximal 10cm of aorta w/ preexisting weakness of media.
Need both weakness and stress –> HTN is key for both. (HTN can cause hyaline arterilosclerosis of vasovasorum –> weaking of medium).
Also associated w/ inherited defects of connective tissue (marfans and ehlers-danlos)
Presentation is sharp tearing chest pain radiating to the back. MC COD is pericardial tamponade. Can also have obstruction of arch vessels and other aortic arteries (i.e. renal artery). Can also result in fatal hemorrhage.
Thoracic aneurysm
Balloon like dilatation of thoracic aorta
Need weakness in aortic wall.
Classically seen in tertiary syphillis (syphillis arteritis of vasovasorm) –> atrophy in wall of blood vessel –> risk for aneursym.
Results in ‘tree-bark’ appearance of aorta due to scarring from end arteritis.
Complications: Dilatation of aortic valve w/ insufficiency. Can also get compression of mediastinal structures. Also rupture and hemorrhage. Can cause get thrombosis or embolus (blood hemostasis and endothelial damage in aneurysm).
AAA
Balloon like dilatation of AA. Usually arises below renal arteries, but above aortic bifurcation (lack of vasovasorum in this region).
Primarily due to atherosclerosis (less O2 to tissue w/o vasovasorum) –> atrophy of wall –> weakness –> aneurysm
Classically in a male smoker > 60 w/ HTN.
Present w/ pulsatile mass that grows w/ time.
Major complication is rupture (esp. if >5cm*). Presents w/ triad of hypotension, pulsatile flank mass, and flank pain.
Hemangioma
Benign tumor comprised of blood vessels
Commonly present at birth; *often regresses during childhood (don’t remove, esp. on face)
Most often involves skin and liver.
Blanching (vs. bleed into skin)
Angiosarcoma
Malignant proliferation of endothelial cells
Highly aggressive
Common sites include skin, breast, and liver
*Liver angiosarcoma is associated w/ exposure to PVC, arsenic , and Thorotrast
Kaposi Sarcoma
Low grade malignant proliferation of enothelial cells
Caused by HHV-8
Presents /w purple patches, placques, or nodules on skin (purple due to blood in the endothelial proliferation –> non-blanching!)
May involve visceral organs.
3 patients:
Older eastern european males (surgical resection for rx), AIDS (rx is antiretrovirals –> recover), and Transplant recipients (decrease immunosuppression).
