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56 Cards in this Set
- Front
- Back
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What is the basic definition of Vasculitis?
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Inflammation of the blood vessels
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What is Vasculitis often accompanied by?
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Necrosis and Occlusion
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What is Vasculitis often the result of (2 things)?
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-Infections
-Autoimmune disease |
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What is the most common type of Vasculitis?
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Giant Cell (Temporal) Arteritis
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What vessels are affected in Giant cell arteritis?
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-Small
-Medium and Large! |
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Where are the most common vessels affected by giant cell arteritis found?
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In the head
-Temporal -Vertebrals -Ophthalmics |
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What type of inflammation is seen in Giant cell arteritis?
In what part of the vessel? |
Granulomatous inflammation in the media
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What is the most common pattern of granulomatous inflammation seen in Giant cell arteritis?
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Nodular thickening and narrowing of the lumen
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What happens to the internal elastic lamina as a result of giant cell arteritis?
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It becomes fragmented
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What is the cause of Giant cell arteritis?
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Unknown
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How is Giant cell arteritis treated?
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With high dose steroids
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What age patients usually get Giant cell arteritis?
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>50 yr old
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What is the clinical presentation of Giant cell arteritis?
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Vague:
-Headache -Ocular symptoms (vision loss or diplopia) |
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The 2 microscopic morpholigies seen in GIant cell arteritis are:
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-Granulomas
-Giant cells |
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What is the "pulseless" disease?
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Takayasu's arteritis
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What size vessels are afflicted in Takayasu's arteritis?
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Medium and large
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What location of vessels are afflicted in Takayasu's arteritis?
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-Aortic arch
-Coronaries -Renals -Carotids |
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Why is Takayasu's arteritis called the pulseless disease?
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Because the upper extremity pulses are weakened.
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What 2 pathologic processes are seen in Takayasu's arteritis?
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-Fibrous thickening of the aortic arch
-Narrowing of the vessels |
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In what patient population is Takayasu's mostly seen?
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Women younger than 40
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What microscopic morphology is seen in Takayasu's arteritis?
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-Mononuclear inflammation
-Giant cells in the adventitia and media |
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Can Takayasu's arteritis be distinguished from Giant cell/temporal arteritis?
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no, not based on morphology
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How is Takayasu's arteritis differentiated from Giant cell arteritis, then?
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Based on clinical presentation -AGE
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What is Systemic necrotizing vasculitis?
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Polyarteritis Nodosa PAN
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What size vessels are affected in PAN?
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Small to medium sized
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What is the course of Polyarteritis nodosa like?
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-Focal
-Random -Episodic |
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What are the clinical symptoms of PAN a result of?
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Ischemia and infarction of downstream organs
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What are the 2 phases of PAN?
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-Acute phase
-Chronic phase |
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What is the acute phase histology in PAN?
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Transmural inflammation with fibrinoid necrosis in the arterial wall
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What is the Late phase histology in PAN?
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Fibrous thickening of the wall
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What is the CHARACTERISTIC of PAN?
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All stages of the disease may coexist at the same time!!
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What is the classic age of PAN patients?
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Young adults
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What does the remittent, episodic, and variable nature of the disease result in?
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Puzzling signs and symptoms
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What is a prominent and major cause of death in patients with polyarteritis nodosa?
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Renal arterial involvement
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What is the mortality rate of untreated PAN? What is the cure rate with steroids?
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Mortality = almost always if untreated
Remission = 90% |
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What is the syndrome that commonly involves coronary arteries in children?
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Kawasaki syndrome
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Why is Kawasaki syndrome concerning?
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It is the leading cause of ACQUIRED heart disease in children in North America and Japan!
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With what other syndrome is Kawasaki syndrome associated?
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Mucocutaneous lymph node syndrome
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What are the clinical symptoms of Mucocutaneous lymph node syndrome?
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-Fever
-Oral/conjunctival redness and edema -Erythyma and desquamation of palms/soles |
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What is the inflammation in Kawasaki syndrome similar to?
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PAN
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How is Kawasaki syndrome inflammation different from that in PAN?
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Less fibrinoid necrosis
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What is Wegener's Granulomatosis?
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A necrotizing vasculitis
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What is the triad of Wegener's Granulomatosis?
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1. Upper resp tract has Acute necrotizing granulomas
2. Vasculitis in the small-med vessels of upper resp tracts - necrotizing/granulomatous. 3. Renal disease |
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What serum antibody is pos in 90% of patients with Wegener's granulomatosis?
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c-ANCA
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What is the death rate of WEgener's granulomatosis?
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80% within the first year
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What sex is Wegener's associated with?
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Men>Women
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What is Thromboangiitis Obliterans?
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Buerger disease
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What vessels are affected in Buerger disease?
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The med/small arteries of the lower extremities - Radial and Tibial
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What is the typical age and sex of patients presenting with Buerger's disease?
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Male Smokers
~35 yr old |
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What is the clinical presentation of Buerger disease?
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Severe Pain!
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Why is Buerger disease seen with such severe pain?
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Because of ischemia and NEURAL involvement
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What makes Buerger syndrome unique from the other vasculitidies?
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The inflammation can spread into continuous veins and nerves
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What causes ischemia in Buerger disease?
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Thrombosis
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What do the thrombi in Buerger disease often contain?
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Neutrophilic microabscesses surrounded by granulomatous inflammation.
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What is often essential for Buerger disease regression?
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Stopping smoking
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What are the 2 most common causes of infectious vasculitis?
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Aspergillus
Mucormycoses |
