Blood Vessels Pt. 2 Flashcards
1
Q
What are the most common vessels to be affected by vasculitis?
A
arterioles, capillaries and venuoles
2
Q
What is the difference between non-infectious and infectious vasculitis?
A
infectious: immune mediated inflammation
- can indirectly induce a noninfectious vasculitis
3
Q
What is the major cause of noninfectious vasculitis?
A
immune response (local or systemic) KNOW these: - giant cell (teporal) arteritis - polyarteritis nodosa (PAN) - granulomatosis with polyangitis (Wegener granulomatosis) - thromboangitis obliterans (Buerger dz)
4
Q
What is immune complex vasculitis?
A
autoantibody production and formation of immune complexes
- deposition of Ag-Ab complexes in vascular walls
- seen in systemic immunologic diseases (SLE), and drug hypersensitivity (PCN acts as hapten binding to serum proteins, streptokinase acts as a foreign protein)
- secondary exposure to infectious agent
5
Q
What is Antineutrophil cytoplasmic antibodies (ANCA)?
A
a heterogeneous group of antibodies reactant with cytoplasmic enzymes found in neutrophil granules, monocytes and endothelial cells
6
Q
What is PR3-ANCA?
A
anti-proteinase-3: associated with polyangiitis
7
Q
What is MPO-ANCA?
A
anti-myeloperoxidase: induced by Rx, propylthiouracil
- associated with microscopic polyangiitis and Churg-Strauss syndrome
8
Q
What do ANCAs do?
A
they activate neutrophils, which then release ROS
- “pauci-immune” due to ANCE Ab directed against cellular constituents and do NOT form circulating immune complexes
9
Q
What is Giant cell (temporal) arteritis and aortitis?
A
most common vasculitis among older patients
- may present with constitutional symptoms (fever, fatigue, wt loss), facial pain, headache
- chronic T cell-mediated (CD4>CD8) inflammation of arteries in the head, especially temporal arteries
10
Q
What happens in chronic inflammation of the arteries?
A
- granulomatous inflammation, often with multinucleated giant cells
- fragmentation of the elastic lamina and intimal thickening
- sites of involvement within artery may be patchy and focal
- double vision* or involvement of opthalmic artery may lead to vision loss -> hallmark
11
Q
What is Takayasu arteritis?
A
characterized by ocular disturbances and marked weakening of pulses of upper extremities (pulseless disease is hallmark)
- sx: weak pulse and low BP in upper extremities
- granulomatous vasculitis of medium and larger arteries
- similar histologically to giant cell arteritis EXCEPT”
- > INVOLVES THE AORTIC ARCH (AORTITIS) AND MAJOR BRANCH VESSELS
12
Q
What are the symptoms of Takayasu arteritis?
A
initially nonspecific
- later, vascular sx dominate: decrease in BP, weak carotid and UE pulses, visual deficits, renal hemorrhages, total blindness and neuro deficits
distal aorta sx: claudication (pain) in legs, pulm art -> pulm HTN, coronary art -> MI, renal art -> systemic HTN
13
Q
What is Polyarteritis nodosa (PAN)?
A
systemic vasculitis, likely immune complex mediated
involves: renal vessels, heart, liver, GI tract
- > pulmonary vessels are spared
classically seen in young adults, 1/3 of pt have chronic hepatitis B*** (hallmark)
- HBsAg-HBsAb complexes are found in involved vessels
14
Q
When do you see segmental transmural necrotizing inflammation?
A
In PAN. it has a predilection for branch points
15
Q
What are the first signs of PAN?
A
ulcerations, infarcts, ischemic atrophy or hemorrhage
16
Q
What are the inflamed vessel walls susceptible to in PAN?
A
- thrombus
- aneurysm
- rupture
17
Q
What is the classic presentation of PAN?
A
typically involves some combination of
- rapidly accelerating HTN due to renal artery involvement
- abdominal pain and blood stools caused by vascular gastrointestinal lesions
- diffuse myalgias and peripheral neuritis mostly affecting motor nerves
- renal involvement is often prominent and a major cause of mortality
18
Q
What happens in PAN goes untreated?
A
Often fatal! Immunosuppression can yield remission or cure in 90% of cases
19
Q
What is Kawasaki disease?
A
acute arteritis of infants and small children** (80% <4 y/o)
20
Q
What is usually involved in Kawasaki disease?
A
coronary arteries
- affected sites may form aneurysms -> thrombosis or rupture -> acute MI
21
Q
What are clinical findings in Kawasaki’s?
A
- erythema of the conjunctiva, oral mucosa, palms and soles, desquamative rash
- cervical lymph node enlargement “mucocutaneous lymph node syndrome”
NOTE: usually self-limiting, but IV-immunoglobulins and aspirin are indicated to lower risk of coronary event
22
Q
What is microscopic polyangiitis?
A
necrotizing vasculitis involving arterioles, capillaries and venules
23
Q
What is the difference between PAN and microscopic polyangiitis?
A
unlike PAN, all lesions of microscopic polyangiitis tend to be of the same age in any given patient and are distributed more widely
24
Q
What vessels does microscopic polyangiitis affect?
A
vessels of many organ systems, but renal glomeruli and lung capillaries are most common (90% of necrotising glomerulonephritis)
25
What are most cases of microscopic polyangiitis associated with?
MPO-ANCA
26
What is seen histologically in microscopic polyangiitis?
segmental necrotizing inflammation with fibrinoid necrosis
- usually see many apoptotic neutrophils
- leukocytoclastic vastilitis, or hypersensitivity vasculitis
27
What are the major symptoms of microscopic polyangiitis?
hemoptysis, hematuria, proteinuria, bowel pain or bleeding, muscle pain or weakness, **palpable** cutaneous purpura
28
What is Churg-Strauss syndrome?
aka allergic granulomatosis and angiitis
- is small vessel necrotizing vasculitis associated with asthma, allergic rhinitis, hypereosinophilia, lung infiltrates, extravascular granulomas
29
What can the inflammation of Churg-Strauss resemble?
PAN or microscopic polyangiitis, ***with the addition of eosinophils and granulomas***
30
What organ systems are involved in Churg-Strauss?
multisystem disease with cutaneous involvement (palpable purpura), GI tract bleeding, and renal disease
- myocardial involvement may give rise to cardiomyopathy, heart is involved in 60% of patients and accounts for half of deaths in the syndrome
31
What is Behcet disease?
vasculitis of small-medium vessels with classic triad of sx:
1. aphthous ulcers (canker sores)
2. genital ulcers
3. uveitis (eye inflammation, reddening)
32
What HLA gene is Behcet associated with?
HLA-B51
33
What is disease mortality related to in Behcet disease?
severe neurologic involvement or rupture of aneurysms
34
What is granulomatosis with polyangiitis?
Aka Wegener granulomatosis, is a necrotizing vasculitis featuring:
- necrotizing granulomas of upper/lower respiratory tracts
- necrotizing or granulomatous vasculitis most prominently in the respiratory tract
35
How would you characterize necrosis of granulomatosis with polyangiitis?
focal necrotizing, often crescentic glomerulonephritis
36
What is granulomatosis with polyangiitis associated with?
PR3-ANCA (up to 96% of cases)
37
What is a form of T cell-mediated hypersensitivity response to normally "innocuous" inhaled microbial or environmental agents
granulomatosis with polyangiitis
38
What are the clinical features of granulomatosis with polyangiitis?
M>F, average age 40
sx: persistent pneumonitis and **sinusitis**, renal disease, nasopharyngeal ulceration
- rashes, myalgias, articular involvement, neural inflammation, fever
39
What happens is granulomatosis with polyangiitis is left untreated?
rapidly fatal, 80% mortality at one year
| tx: steriods, cyclophosphamide, TNF agonists
40
What is the morphology of granulomatosis with polyangiitis?
- upper respiratory tract: sinonasal and pharyngeal inflammation with granulomas and vasculitis (granulomas with geographic patterns of central necrosis, accompanying vasculitis)
- lower resp tract: multiple necrotizing granulomas, which may coalesce and cavitate**
41
What is Thromboangiitis obliterans (Buerger disease)?
- segmental, thombosing vasculitis of small and medium vessels
- especially affects tibial and radial arteries
- may lead to vascular insufficiency of the extremities (chronic ulcerations can lead to gangrene)
- pt almost always heavy smokers, <35 y/o, most have hypersensitivity to intradermally injected tobacco products
42
What ethnic groups are associated with Buerger disease?
Israeli, Indian subcontinent, Japanese
43
What are the symptoms of Thromboangiitis obliterans?
** severe pain, even at rest**, chronic extremity ulcerations leading to gangrene
early manifestations: cold-induced Raynaud phenomenon, leg pain induced by exercise that is relieved on rest (intermittent claudication), instep foot pain, ***superficial nodular phlebitis (venous inflammation)***
44
What are the agents of direct invasion of infectious vasculitis?
pseudomonas, aspergillus, mucor
45
Why are mycotic aneurysms a concern in infectious vasculitis?
because vascular infections weaken the arterial walls, or induce thrombosis/infarction
46
What is the Raynaud phenomenon?
excessive vasospasm of small arteries and arterioles, especially fingers and toes
- "red, white, and blue" = proximal vasodilation, central vasoconstriction, and distal cyanosis**
47
What is Primary Raynaud phenomenon?
Aka Raynaud disease, induced by cold or emotion, symmetric involvement of the digits
- estimated 3-5% of population, young women, benign course
48
What is secondary Raynaud phenomenon?
component of another arterial disease such as SLA, scleroderma, thromboangiitis obliterans, asymmetric involvement of digits
- worsens with time
49
What is myocardial vessel vasospasm?
Aka cardiac Raynaud, is excessive vasoconstriction of myocardial arteries or arterioles that may cause ischemia or infarct
50
What usually causes myocardial vessel vasospasm?
circulating vasoactive agents, which may be endogenous (Epi, pheochromocytoma), or exogenous (cocaine)
51
What is the outcome of myocardial vessel vasospasm?
sudden cardia death or Takotsubo cardiomyopathy (broken heart syndrome, assoc with emotional distress)
52
What causes varicose veins?
abnormal dilation of veins with valvular incompetence, secondary to sustained intraluminal pressure
- stasis, congestion, thrombus, edema, pain and ischemia of overlying skin (stasis dermatitis)
53
What is rare to be seen in varicose veins?
embolism from thrombi of superficial lower extremity veins (unlike DVT, which is not rare to see)
54
What is esophageal varices?
portal HTN (d/t cirrhosis) opens portosystemic shunts which direct blood to veins at the gastroesophageal junction
55
Why are esophageal varices clinically important?
because they may fatally rupture
56
What are hemorrhoids?
dilation of the venous plexus at the anorectal junction
| - cause pain, bleeding, may ulcerate
57
What is thrombophlebitis?
venous thrombosis and inflammation
| - almost always (>90%) involves deep veins in the legs, can be complete asymptomatic
58
What is the single most important risk factor for developing a deep venous thrombosis in the lower extremities?
prolonged inactivity/immobilization
NOTE: systemic hypercoagulability may also increase the risk of DVT
59
What is the most serious potential consequence of thrombophlebitis?
pulmonary embolism
60
What is migratory thrombophlebitis? (Trousseau sign)
patients with cancer may experience hypercoagulability as a paraneoplastic syndrome
61
Where is Trousseau sign particularly seen?
mucin-producing adenocarcinomas (music thought to be thrombogenic)
62
What is seen in a classic case of migratory thrombophlebitis?
venous thromboses appear at one site, disappear and reappear at a different site
63
What is thrombophlebitis associated with?
adenocarcinomas of the lung, ovary and pancreas
64
What is Superior Vena Cave syndrome?
neoplasms that compress of invade the SVC
| - bronchogenic carcinoma, mediastinal lymphoma, or aortic aneurysm
65
What is the characteristic clinical complex that SVC obstruction causes?
- marked dilation of the veins of the head, neck and arms with cyanosis
- pulmonary vessels can also be compressed -> respiratory distress***
66
What is Inferior Vena Cava syndrome?
neoplasms that compress or invade the IVC, or thrombosis of the hepatic, renal or lower extremity veins that propagate cephalad
67
What two types of carcinoma tend to grow in veins, occlude the IVC and extend into the right atrium?
Hepatocellular carcinoma and renal cell carcinoma
68
What does IVC syndrome cause?
induces marked lower extremity edema, distention of the superficial collateral veins of the lower abdomen
- renal involvement -> massive proteinuria***
69
What is lymphangitis?
accute inflammation and spread of bacterial infection into lymphatics
70
What is the most common infectious agent of lymphangitis?
group A b-hemolytic streptococci
71
What are the symptoms of lymphangitis?
red, painful subcutaneous streaks (inflamed lymphatics), and painful enlargement of draining LN (lymphadenitis)
72
What specific markers are used to detect malignant tumors?
CD31 and von Willebrand factor
73
What are hemangiomas?
common tumors, localized increase in neoplastic blood vessels
- common sites include skin and mucous membranes of the head and neck, and in the liver
- congenital (juvenile "strawberry") hemangiomas often regress
74
What is a capillary hengioma?
MOST COMMON, thin-walled capillaries, tightly packed together
75
What is a cavernous hemangioma?
irregular, dilated vascular channels that make a lesion with an indistinct border
- more likely to involve deep tissue, more likely to bleed d/t larger vessel involvement
76
What is a pyogenic granuloma?
Aka lobular capillary hemangioma
- type of capillary hemangioma
- rapidly growing, often in oral mucosa*** (may ulcerate), can develop following trauma
77
What is a granuloma gravidarum?
tumor that develops in the gingiva of pregnant women
78
What is a simple lymphangioma?
appear very similar to capillary hemangiomas, but WITHOUT RBCs
- subcutis of head/neck and axillae
79
What is a cavernous lymphangioma?
Aka cystic hygroma, in the neck or axilla of children
| - can be large, often seen in Turner syndrome***
80
What is a Glomus tumor?
Aka glomangioma, paraganglioma
- benign, painful tumor arising from glomus bodies (dermis layer of skin, but smooth muscle origin, not endothelial)
- most often appear in distal fingers
81
What is Bacillary angiomatosis?
a vascular proliferation in response to gram negative Bartonella bacilli
- occurs on the skin of the immunocoprimised
- lesions are localized, forming red papules
- Tx: macrolide antiobiotics
82
What does Bacillary angiomatosus look like histologically?
proliferation of capillaries with plump endothelial cells
83
What histo stain is used to identify Bacillary angiomatosis?
Warthin-Starry stain
84
What is epithelioid hemangioendothelioma?
neoplastic endothelial cells are plump and cuboidal, resembling epithelium. vascular channels may be difficult to recognize
- variable clinical behavior, with metastasis in 20-30%
85
What causes Kaposi Sarcoma?
human herpesvirus 8
86
What are the 4 distinct clinical forms of Kaposi Sarcoma?
1. AIDS-associated KS
2. Classic KS
3. Endemic African KS
4. Transplant-associated KS
87
What is AIDS-associated KS?
- Most common form in US
- most common AIDS-related malignant tumor
- may spread to lymph nodes and viscera
88
What is classic KS?
- older men from middle eastern, mediterranean or eastern european descent
- NOT associated with HIV
- tumors localized to skin
89
What is Endemic African KS?
- NOT associated with HIV
- patients ,40
- can involve lymph nodes
90
What is Transplant-associated KS?
- NOT associated with HIV, but with T-cell immunosuppression
| - can spread to lymph nodes and viscera
91
What is an angiosarcoma?
malignant endothelial tumor
- can be induced by radiation exposure, can arise in setting of lymphedema (upper extremity after radical mastectomy)
- may occur everywhere, but most common on skin, soft tissue, breast and liver
- hard to treat, 5 year survival 30%
92
What is associated with arsenic, pesticides, Thorotrast (contract agent) and polyvinyl chloride?
Hepatic angiosarcoma
93
What is a balloon angioplasty?
rupture of occluding plaque, and limited dissection produced
| - abrupt reclosure can result from extensive dissection, thus 90% of angioplasties followed by stent placement
94
What are drug-eluting stents?
leach antiproliferative drugs to block smooth muscle activation
- just a temporary fix
95
What vasculature is utilized in small bore grafts?
saphenous vein or left internal mammary arteries
