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CP2 7.4 path PAD, vasculitis, dz of aorta

fisch

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image: scheme cyanosis
what is vasculitis?
inflammation and necrosis of blood vessels, including arteries, veins, and capillaries.
what are the causes of vasculitis?
infectious agents, mechanical trauma, radiation or toxins, often idiopathic.
What is the pathogenesis of the Vasculitides?
involve immune mechanisms, including (1) the deposition of immune complexes, (2) a direct attack on the vessels by circulating antibodies, and (3) various forms of cell-mediated immunity. The start of the reaction is unknown, but sometimes linked to viral infection.
How do viruses interact with blood vessels?
viral antigen antibody immune complexes are deposited in the vessel wall
Which viruses are known to cause vasculitis?
Hepatitis B virus is associated with some cases of Polyarteritis Nodosa, others include Herpes simplex, CMV, and Parvovirus.
What are ANCAs?
anti-neutrophil cytoplasmic antibodies are associated with Small vessel vasculitides, like Wegener granulomatosis and Polyarteritis Nodosa.
How is damage accomplished with ANCAs?
they activate neutrophil.
How are ANCA's detected?
indirect immunoflourescence assays using the patients serum and ethanol-fixed neutrophils.
What are the different kinds of titers?
Perinuclear immunoflourescence (P-ANCA) and a more general Cytoplasmic immunoflourescence (C-ANCA). each indicating different vasculatides.
What is Polyarteritis Nodosa?
acute, necrotizing vasculitis that affects medium and smaller-sized muscular arteries.
What population is susceptible to developing PAN?
white men
What is the pathology of Polyarteritis Nodosa?
lesions are patchy in distribution, and may extend to renal, splenic or coronary arteries. fibrinoid necrosis in which vessel layers are fused into a eosinophilic mass of fibrin. acute inflammation surrounds the area of necrosis and extends into all the other layers of the vessel.
which immune cells are involved in PAN?
Neutrophils, plasma cells, and lymphocytes
Which ANCA titer in PAN associated with?
P-ANCA
What is found on autopsy with PAN?
Thrombosis of the smaller arteries and infarcts in the involved organs. Small aneurysms in larger vessels, particularly in the branches of the renal, coronary, and cerebral arteries.
How does PAN cause death?
rupture of aneurysm, thrombosis causing organ failure.
What if patients survives aneurysm rupture?
healing with fibrosis of the media and gaps in the elastic laminae, especially if corticosteroids have been administered.
What is the clinical presentation of PAN?
kidneys, heart, skeletal muscles, skin and mesentery are most frequently involved, fever and weight loss.
How can PAN be treated?
corticosteroids and Cyclophosphamide
What is Temporal Arteritis?
focal, chronic, granulomatous inflammation of the temporal arteries. can also involve additional cranial arteries, the aorta and its branches, and other arteries.
Which age range most commonly develops TA?
70+. can happen as young as 50 yoa
What is the cause of Temporal Arteritis?
genetic component, activated CD4+ T-cells, suggests an immunological reaction, and a cell-mediated response to arterial antigens.
How does the artery appear grossly?
vessel is cord-like and exhibits nodular thickening. The lumen is reduced to a slit or may be obliterated by a thrombus.
How does the artery appear microscopically?
granulomatous inflammation of the media and intima, consisting of aggregates of macrophages, lymphocytes, and plasma cells with varying amounts of neutrophils and eosinophils. Giant cells are distributed near the elastic laminae along with necrosis and progressive fragmentation of the elastic lamina.
How does the artery appear microscopically in late stages?
The intima is thickened and the media is fibrotic. Thrombosis may obliterate the lumen after which organization and canalization occurs.
Is TA dangerous?
benign and self-limited, with the symptoms subsiding in 6-12 months.
What are the S/S of TA?
H/A, and throbbing temporal pain, malaise, fever, weight loss, and generalized muscular aching in the shoulders and hips (polymyalgia rheumatica). Visual symptoms occur in almost 1/2 of the patients and may proceed from transient to permanent blindness.
What is found on PE with TA?
swelling, tenderness, and redness in the skin overlying the vessel.
How is the diagnosis of TA made?
biopsy is unreliable, diagnosis from history
What is the treatment for TA?
corticosteroids help dramatically
What is Kawasaki Disease?
AKA Mucocutaneous Lymph Node Syndrome. acute, necrotizing vasculitis of infancy and early childhood.
What are the S/S of Kawasaki's?
high fever, rash, conjunctival and oral lesions, lymphadenitis, and peeling skin on fingertips, soles and palms.
Is Kawasaki's dangerous?
yes- this form of vasculitis affects coronary arteries in 70% of cases forming aneurysms. This is the cause of death in 2% of cases.
What is the cause of kawasaki's?
unknown, some cases are associated with Parvovirus B19, and various bacterial infections.
What is Takayasu Arteritis?
inflammatory disorder of large arteries, classically the aortic arch and its major branches.
Which populations develop takayasu arteritis?
primarily affects young women (90%), the large majority of whom are younger than 30 years of age.
What is the cause of Takayasu?
unknown, but an autoimmune basis has been proposed.
What is the gross pathology that leads to its other name?
aorta is thickened and the intima exhibits focal, raised plaques, branches of the aorta exhibit localized stenosis or occlusion- causing "Pulseless Disease" when the subclavian arteries are affected. thoracic and abdominal aorta commonly show aneurysms
How do the arteries appear microscopically?
panarteritis, with infiltrates of neuts, lymphs, and giant cells (granulomatous inflammation).
What are the S/S of takayasu?
dizziness, visual disturbance, cardiac symptoms appear with claudication of the arms and legs.
How does takayasu affect blood pressure?
asymmetrical differences in the BP may occur, and the pulse in one extremity may be absent.
What is the treatment for takayasu?
steroids, eventually srugery
What is Wegener Granulomatosis?
systemic, necrotizing vasculitis of the respiratory tract and renal glomerular disease
what is the cause of wegener's?
unknown cause, no infectious agent known
Which ANCA's are involved with Wegener's?
C-ANCA
What is the microscopic pathology in Wegener's?
parenchymal necrosis, vasculitis, and a granulomatous inflammation composed of neuts, lymphs, plasma cells, macrophages, and eosinophils. Differentiate these lesions from TB
What organs are involved most often in Wegeners?
respiratory tract, kidney and spleen.
How do the vessels appear grossly in Wegener's?
medial thickening, intimal proliferation and narrowing of the lumen.
What are the most prominent pulmonary features in Wegerners?
persistent bilateral pneumonia with nodular infiltrates that undergo cavitation. Chronic granulomatous sinusitis and ulcerations of the nasopharyngeal mucosa are common.
What are the most prominent kidney features?
focal necrotizing glomerulonephritis, which progresses to Crescentic Glomerulonephritis (Rapidly Progressive Glomerulonephritis)
what is the clinical presentation of Wegener's?
pneumonia and sinusitis, Hematuria and proteinuria, acute renal failure, Rashes, muscular pains, joint involvement, and neurologic symptoms
What does testing show in Wegener's?
On CXR, multiple cavitating pulmonary infiltrates are prominent.
What is the treatment for Wegener's?
cyclophosphaminde.
Is Wegener's dangerous?
mean survival is five months
What is another name for Churg-Strauss syndrome?
Allergic granulomatosis and Angiitis
What is Churg-Strauss syndrome?
systemic vasculitis with prominent eosinophilia that occurs in young people with asthma. necrotizing vascular lesions of the small and medium-sized arteries, arterioles, and veins of the lungs, spleen, kidney, heart, liver, and central nervous system.
What are the microscopic findings in Churg-Strauss?
granulomas with an intense eosinophilic infiltrate in and around blood vessels, resulting in fibrinoid necrosis and thrombosis.
Which ANCAs are involved?
C-ANCA and P-ANCA is demonstrated in 2/3 of the patients.
What is the prognosis of Churg-Strauss?
corticosteroids, untreated has poor prognosis
What is another name for Thromboangiitis Obliterans?
Buerger Disease
What is Thromboangiitis Obliterans?
occlusive, inflammatory disease of medium and small arteries in the distal arms and legs.
Which populations are pre-disposed to developing Thromboangiitis Obliterans?
middle-aged heavy smokers.
How does smoking contribute to developing Thromboangiitis Obliterans?
mechanism unknown, but ceasation of smoking causes disease to go to remission. byproducts of tobacco elicit antibodies that can induce inflammation.
Is there a genetic component to Thromboangiitis Obliterans?
greater frequency in Japan, Israel, and India, suggesting possible predisposing genetic factors.
What is the microscopic pathology in Thromboangiitis Obliternans?
inflammation of medium-sized and small arteries with PMN infiltrates. Small microabscesses of the vessel wall, with neutrophils and giant cells are prominent.
What are the complications in Thromboangiitis Obliternans?
endothelium in the inflamed areas leads to thrombosis and obliteration of the lumen. early lesions can be severe enough to result in gangrene of the extremities, the only treatment being amputation.
What is the clinical presentation of Thromboangiitis Obliterans?
symptoms usually start between the ages of 25 and 40 years and include claudication and painful ulcerations of the digits. Those who continue to smoke may slowly lose both hands and feet.
What is the etiology of Varicose Veins?
venous valves easily become incompetent when the veins dilate.
What happens to the leg veins when they become varicose?
dilated and tortuous, aka varicose.
Are varicose veins dangerous?
slow flow of blood in the veins predisposes an individual to clotting.
Which diseases allow development of varicose veins?
heart failure.
who is more likely to get varicose veins?
people with professions that require long hours of standing.
What causes varicose veins?
multi-factorial disease with a genetic and environmental component. more likely to occur in individuals from families known to have a connective tissue weakness.
Describe varicose veins and pregnancy.
results from the increased blood pressure in the pelvic veins compressed by the pregnant uterus and the subsequent stagnation of blood in the leg veins.
What is thrombophlebitis, and how does it contribute o varicose veins?
Thrombotic occlusion of the veins prevents the outflow of venous blood allows for venous distention and valve incompetence.
What are the complications of Varicose Veins?
clotting, embolus to lungs, leaking of blood from capillaries
What does the leakage of blood into the tissues cause?
Brownish discoloration of the skin, skin is dry and scaly and shows small, pinpoint hemorrhages, ischemia causes the skin to necrotize and stasis ulcers may form.
What is hemosiderin?
pigment in RBCs
What is "Stasis Dermatitis?"
pinpoint hemorrhages
image: varicose veins

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