Cardio-Path-Vasculitis Flashcards Preview

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Flashcards in Cardio-Path-Vasculitis Deck (127)
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1
Q

Which type of vessel only has endothelium, with no subendothelial layer or other tunics.

A

capillaries

2
Q

In arteries the intima includes a thin layer, the composed of elastin, with holes allowing better diffusion of substances from blood deeper into the wall.

A

internal elastic lamina

3
Q

Variable amounts of which 4 components are scattered throughout the muscle fibers of the tunica media and all of which are produced by the SMCs?

A

elastic fibers

and elastic lamellae,

reticular fibers, and

proteoglycans,

4
Q

general term for vessel wall inflammation?

A

vasculitis

5
Q

Concerning vasculitis,

Classification schemes attempt to group them according to vessel role of immune complexes, presence of specific autoantibodies, granuloma formation, organ , and population demo­graphics.

A

diameter; specificity

6
Q

The granulomas of are found within the vessel wall as part of the inflammation comprising the vasculitis, but need not be present to render the diagnosis.

A

giant cell arteritis

7
Q

Not Giant cel arteritis, The granulomas of are larger, spanning between vessels, and associated with areas of tissue necrosis.

A

granulomatosis with polyangiitis

8
Q

Though the clinical manifestations are many, what do they largely depend on :

A

specific vascular bed that is affected

9
Q

Regarding vasculitis, besides findings referable to the affected tissue(s), there are usually also S and S of inflammation, eg, fever, myalgia, arthralgias, and malaise

A

systemic

10
Q

The 4 main immunologic mechanisms underlying noninfectious vasculitis are:

A
  • Immune complex deposition
  • Anti-neutrophil cytoplasmic antibodies (ANCAs)
  • Anti-Endothelial Cell antibodies
  • Autoreactive T cells
11
Q

Immune complex associated vasculitis is seen in immunologic disorders such as that are associated with autoantibody production

A

SLE

12
Q

Immune complexes involve both the antigen and :

A

antibody

13
Q

Many patients with vasculitis have circulating antibodies that react with neutrophil cytoplasmic antigens called:

A

“anti-neutrophil cytoplasmic antibodies (ANCAs).”

14
Q

What are a heterogeneous group of autoantibodies directed against constituents (mainly enzymes) of neutrophil primary granules, monocyte lysosomes, and ECs?

A

ANCAs

15
Q

What anti-neutrophil cytoplasmic Ab (ANCA) attacks PR3 is a neutrophil azurophilic granule constituent?

A

Anti-proteinase-3 (PR3-ANCA), previously called c-ANCA.

16
Q

Which type of vasculitis is associated with PR3-ANCAs?

A

granulomatosis with polyangiitis (WEGENERS)

17
Q

What is a lysosomal granule constituent involved in oxygen free radical generation.?

A

myeloperoxidase (MPO)

18
Q

Which ANCA is associated with microscopic polyangiitis and Churg-Strauss syndrome?

A

Anti-myeloperoxidase (MPO-ANCA), previously called p-ANCA.

19
Q

The close association between ANCA titers and disease activity suggests

a role for these antibodies

A

pathogenic

20
Q

ANCAs can directly/indirectly activate neutrophils, stimulating the release of reactive oxygen species and proteolytic enzymes; this may lead to EC injury associated with vasculitis.

A

directly

21
Q

In antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV), local infection, such as with Staphylococcus aureus, results in priming of via proinflammatory cytokines. This results in the surface expression of the ANCA antigens, allowing ANCAs to bind to and further activate that are rolling along the endothelium. Activation results in firm binding to the and release of lytic enzymes and reactive oxygen species (ROS), which damage the vessel wall

A

neutrophils; neutrophils; endothelium

22
Q

Antibodies to endothelial cells underlie certain vasculitides, for example in children:

A

Kawasaki disease (a medium vessel vasculitis)

23
Q

Autoreactive T cells cause injury in some forms of vasculitides characterized by formation of .

A

granulomas

24
Q

What is a chronic inflammatory disorder, typically with granulomatous inflammation, that principally affects large- to small-sized arteries in the head?

A

Giant cell (temporal) arteritis

25
Q

How is the diagnosis for Giant cell (temporal) arteritis established?

A

biopsy of the temporal arteries.

26
Q

As concerning biopsy of the temporal arteries, what are some other common affected arteries?

A

Vertebral and ophthalmic arteries, as well as the aorta (giant cell aortitis)

27
Q

Because ophthalmic artery vasculitis can lead to sudden and permanent affected individuals must be promptly diagnosed and treated.

A

blindness,

28
Q

What is the most common form of vasculitis among older adults in developed countries?

A

Giant cell (temporal) arteritis

29
Q

Which vasculitis likely occurs as a result of a T-cell–mediated immune response to an as-yet uncharacterized vessel wall antigen.

• Pro-inflammatory cytokines (especially TNF) and anti-EC antibodies may also contribute.

A

Giant Cell Arteritis

30
Q

Concerning giant cell arteritis, pathologic changes are not consistent throughout the affected vessel, these changes are considered .

A

patchy

31
Q

In Giant cell arteritis, Involved arterial segments exhibit intimal thickening (and occasional thromboses) that reduce the vessel diameter and cause distal .

A

nodular; ischemia.

32
Q

The vast majority of lesions in giant cell arteritis exhibit granulomatous inflammation within the inner centered on the internal elastic membrane; and of the internal elastic lamina as demonstrated in the slide.

A

media; fragmentation

33
Q

What is being demonstrated in this slide of giant cell arteritis?

A

Hematoxylin-eosin-stained section of a temporal artery showing giant cells near the fragmented internal elastic membrane (arrow), along with medial and adventitial inflammation.

34
Q

What is being demonstrated in this slide of giant cell arteritis?

A

Elastic tissue stain demonstrating focal destruction of internal elastic lamina (arrow) and intimal thickening (IT) characteristic of long-standing or healed arteritis.

35
Q

• Temporal arteritis is rare/common before 50 years of age.

A

rare

36
Q

Concerning giant cell arteritis, Other than Signs and symptoms that are vague and constitutional (eg, fever, fatigue, weight loss) what type of facial pain accompanies this disease:

A

most intense along the course of the superficial temporal artery, which is painful to palpation.

37
Q

What 2 common occular symptoms (associated with involvement of the ophthalmic artery) abruptly appear in about 50% of patients with giant cell cell arteritis?

A

diplopia to complete vision loss.

38
Q

What are 2 effective treatments for giant cell arteritis?

A

Corticosteroid or anti-TNF therapies are effective treatments.

39
Q

In Giant cell arteritis, the superficial temporal artery is often , nodular and thickened

A

pulseless

40
Q

Which vasculitis is a granulomatous vasculitis of medium- and large-sized arteries characterized principally by ocular disturbances, and marked weakening of the pulses in the upper extremities (hence the alternate name, “pulseless disease”)?

A

• Takayasu arteritis

41
Q

What manifests with transmural scarring and thickening of the aorta - particularly the aortic arch and great vessels - with severe luminal narrowing of the major branch vessels as is demonstrated in the slides?

A

Takayasu Arteritis

42
Q

What is the distinction between ginat cell arteritis and takayasu arteritis as they both share clinical and histologic features of giant cell aortitis?

A

basis of a patient’s age; those older than 50 years of age are said to have giant cell aortitis, and lesions that occur in those younger than 50 years of age are designated Takayasu aortitis.

43
Q

Takayasu arteritis classically affects the aortic and vessels.

A

arch; arch

44
Q

Concerning takayasu arteritis, what other arteries, other than aortic arch and arch vessels are invovled in 50% of patients?

A

pulmonary arteries; renal and coronary arteries can also be affected

45
Q

What explains upper-extremity weakness and faint carotid pulses in cases of takayasu arteritis?

A

The takeoffs of the great vessels can be markedly narrowed and even obliterated

46
Q

There is a histologic spectrum ranging from adventitial mononuclear infiltrates and perivascular cuffing of the vasa vasorum, to intense transmural mononuclear inflammation, to granulomatous inflammation, including giant cells and patchy medial necrosis.

• Inflammation is associated with irregular of the vessel wall, intimal , and adventitial

A

thickening; hyperplasia; fibrosis.

47
Q

Early signs and symptoms of takayasu arteritis are nonspecific, however, with progression, vascular signs and symptoms appear including reduced upper-extremity blood pressure and pulse strength; neurologic deficits; and ocular disturbances, including visual field defects, retinal hemorrhages, and total

A

blindness.

48
Q

The evolution of Takayasu Arteritis is fixed/variable?

A

variable

49
Q

In Takayasu arteritis, distal aorta disease can manifest as leg pain or :

A

claudication

50
Q

In Takayasu arteritis, pulmonary artery involvement can cause pulmonary

A

hypertension

51
Q

In 50% of patients with renal artery involvement with diagnosed Takayasu arteritis, can cause what systemic issue?

A

systemic hypertension

52
Q

Which vasculitis is a systemic vasculitis of small - or medium - sized muscular arteries that typically involves the renal and visceral vessels and spares the pulmonary circulation?

A

Polyarteritis Nodosa (PAN)

53
Q

One third of pts. with Polyarteritis Nodosa (PAN), have which type of chronic infection, which leads to the formation of immune complexes containing antigens that deposit in affected vessels.

A

Hepatitis B infection, and the Hep antigens are the antigens depositied in the affected vessels

54
Q

Other than HEP B being the cause of a third of the cases of • Polyarteritis Nodosa (PAN), what is the cause in the remaining cases?

A

unknown

55
Q

Which type of polyarteritis nodisa is a segmental transmural necrotizing inflammation of small-to medium-sized arteries, often with thrombosis?

A

Classic PAN

56
Q

What are the 4 main visceral arteries affected in decreasing order of frequency?

A

Kidney,

heart,

liver,

gastrointestinal tract vessels

57
Q

What are some of the effects of impaired perfusion related to polyarteritis nodosa?

A

ulcerations, infarcts, ischemic atrophy, or hemorrhages in the distribution of affected vessels

58
Q

When the inflammatory process related to polyarteritis nodosa weakens the arterial wall, it can lead to which condition?

A

aneurysm and rupture

59
Q

During which phase of polyarteritis nodosa, there is transmural mixed inflammatory infiltrate composed of neutrophils and mononuclear cells, frequently accompanied by fibrinoid necrosis and luminal thrombosis?

A

The acute phase

Note: the dark pink is fibrinoid necrosis and the black arrow demonstrates areas that are not involved

60
Q

In polyarteritis nodosa, all stages of activity (from early to late) in different vessels or even within the same vessel

A

coexist

61
Q

PAN is primarily a disease of adults, but can occur in all age groups.

A

young

62
Q

The clinical course of PAN typically is episodic/ persistent, with long symptom-free intervals.

A

episodic

63
Q

Why are the clinical manifestations of polyarteritis varied?

A

The systemic findings - malaise, fever, and weight loss - are nonspecific, and the vascular involvement is widely scattered,

64
Q

Concerning PAN, what is the classic presentation involving the renal arteries?

A

rapidly accelerating hypertension

65
Q

Concerning PAN, what is the classic presentation of GI arteries and lesions?

A

abdominal pain and bloody stools

66
Q

Concerning PAN, what is the classic presentation when the motor nerves are affected?

A

diffuse muscular aches and pains; and peripheral neuritis,

67
Q

Concerning PAN, which organ is often involved and is a majpr cause of death?

A

kidneys, renal involvement

68
Q

How is renal involved PAN treated?

A

With immunosuppression, 5-year survival is close to 80%.*

69
Q

Which vasculitis is an acute, febrile, usually self-limited illness of infancy and childhood, associated with an arteritis of mainly large- to medium-sized vessels.?

A

Kawasaki disease

70
Q

The clinical significance of Kawasaki disease stems from the involvement of which arteries?

A

coronary arteries

71
Q

What is the clinical significance of the involvement of coronary arteries in Kawasaki disease?

A

Coronary arteritis can result in aneurysms that rupture or thrombose, causing myocardial infarction.

72
Q

Concerning Kawasakin disease, most patients, approximately 80%, are under the age of:

A

4

73
Q

In genetically susceptible individuals, a variety of infectious agents (mostly ) may trigger the disease.

A

viral

74
Q

Which vasculitis results from a delayed-type hypersensitivity response directed against cross-reactive vascular antigen(s) that is usually a self-limited illness of infancy and childhood?

A

Kawasaki disease

75
Q

Describe the process of delayed-type hypersensitivity response directed against cross-reactive vascular antigen(s) as it pertains to Kawasaki disease?

A

After infection (usually viral), subsequent cytokine production and B cell activation result in auto-antibodies to endothelial cells (and SMC’s) that precipitate the vasculitis.

76
Q

What are the main clinical manifestations of Kawasaki disease?

A

conjunctival and oral erythema,

blistering,

edema of the hands and feet,

erythema of the palms and soles,

a desquamative rash,

and cervical lymph node enlargement.*

77
Q

Concerning Kawasaki dz, what are the cardiovascular sequelae that develop in 20% of patients?

A

asymptomatic coronary arteritis, to coronary artery ectasia (dilation), to large coronary artery aneurysms (7-8 mm in diameter).

78
Q

What is the treatement for Kawasaki disease?

A

intravenous immunoglobulin infusions (IVIG) and aspirin

Which decrease the incidence of symptomatic coronary artery disease

79
Q

What is a necrotizing vasculitis that generally affects capillaries, as well as small arterioles and venules?

A

Microscopic polyangiitis (leukocytoclastic vasculitis)

80
Q

Which 8 areas of the body can be inolved with Microscopic polyangiitis (leukocytoclastic vasculitis)?

A

skin,

mucous membranes,

lungs,

brain,

heart,

GI tract,

kidneys,

and muscle can be involved

81
Q

90% of patients with Microscopic polyangiitis (leukocytoclastic vasculitis) develop :

A

Necrotizing glomerulonephritis.

82
Q

Other than Necrotizing glomerulonephritis (90%), a common effect of Microscopic polyangiitis (leukocytoclastic vasculitis), what other effect is common?

A

pulmonary capillaritis

83
Q

In some cases of microscopic polyangiitis, there are antibody responses to antigens, such as:

A

drugs (eg, penicillin), microorganisms (eg, streptococci), or tumor proteins have been implicated

84
Q

Conceringin microscopic polyangiitis, , antibody responses to antigens such as drugs (eg, penicillin), microorganisms (eg, streptococci), or tumor protein reactions can lead to deposition or trigger secondary immune responses, eg, development of , that are pathogenic.

A

IC; ANCAs

85
Q

Most cases of microscopic polyangiitis associated with autoantiody response are asociated with which type of ANCA?

A

MPO-ANCA (p-ANCA).

86
Q

Like other diseases that involve ANCAs, microscopic polyangiitis works on Recruitment and activation of in the vascular beds is likely responsible for the disease manifestations

A

neutrophils

87
Q

Describ the pathogenic process of ANCAs and neutrophils in a disease like microscopic polyangiitis?

A

In antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV), local infection, such as with Staphylococcus aureus, results in priming of neutrophils via proinflammatory cytokines. This results in the surface expression of the ANCA antigens, allowing ANCAs to bind to and further activate neutrophils that are rolling along the endothelium. Activation results in firm binding to the endothelium and release of lytic enzymes and reactive oxygen species (ROS), which damage the vessel wall.

88
Q

Due to ANCA activation of more neutrophils and generation of ROS species, what is another manner by which ANCA activated neutrophils can damage the vessel wall in vasculitis?

A

Also, the alternative pathway of complement is activated with generation of the powerful neutrophil chemoattractant C5a. This amplification loop contributes to the necrotizing inflammation of the vessel wall.

89
Q

In microscopic polyangiitis, histologically, segmental fibrinoid of the media with focal necrotizing lesions are seen.

A

necrosis; transmural

Note: In some areas, only infiltrating neutrophils undergoing fragmentation are seen, giving rise to the term leukocytoclastic vasculitis (microscopic polyangiitis)

neutrophil fragements are seen in this image

90
Q

Concerning microscopic polyangiitis, if the renal vascular bed is involved, what signs and symptoms can be seen?

A

hemoptysis, hematuria, proteinuria,

91
Q

Concerning microscopic polyangiitis, if the gi vascular bed is involved, what signs and symptoms can be seen?

A

abdominal pain or bleeding

92
Q

Microscopic polyangiitis can lead to muscle pain or weakness, and palpable cutaneous , if it involves vascular beds in the muscles and skin.

A

purpura.

93
Q

What two methods of treatement can cause microcsopic polyangiitis to induce remission, With the exception of patients with widespread renal or CNS involvement?

A

immunosuppression and removal of the offending agent

94
Q

Granulomatosis with Polyangiitis (GPA) was previously called

A

Wegener granulomatosis

95
Q

Which vasculitis is characterized by a triad of:

Necrotizing granulomas of the upper-respiratory tract, the lower-respiratory tract or both. •

Necrotizing or granulomatous vasculitis affecting small- to medium- sized vessels, most prominently the lungs and upper airways, but other sites as well.

• Focal necrotizing, often crescentic, glomerulonephritis.*

A

Granulomatosis with Polyangiitis (GPA)

96
Q

Conversely, when widespread the disease may affect the eyes, skin, and other organs, most notably the heart; clinically, widespread GPA resembles PAN with the additional feature of involvement.

A

respiratory

97
Q

Granulomatosis With PolyangiitisIt is likely initiated as a cell-mediated hypersensitivity response to infectious or environmental AGs.

A

inhaled

98
Q

Which ANCA represents is involved with Granulomatosis With Polyangiitis, and is involved with 95% of cases?

A

PR3-ANCAs (c-ANCAs)

99
Q

What is a useful marker for disease activity in Granulomatosis With Polyangiitis?

A

PR3-ANCAs (c-ANCAs

100
Q

Examples of Granulomatosis With Polyangiitis in the upper respiratory tract can also be called granulomatous . There can also be ulcerative lesions of the nose, palate, or pharynx.

A

sinusitis

note: A. GAP inflammation B. necrosis C. giant cells with granulomas D. vasculitis

101
Q

What is the tissue invovled with this case of Granulomatosis With Polyangiitis:

A

Lung findings range from diffuse parenchymal infiltrates to granulomatous nodules.

note: notice cavity (black star) due to necrosis

102
Q
A

There is multifocal necrotizing granulomatous vasculitis with a surrounding fibroblastic proliferation.

note: notice the giant cells

103
Q

Describe this example of Granulomatosis With Polyangiitis

A

Multiple granulomata can coalesce to produce radiographically visible nodules with central cavitation

104
Q

WIth the respiratory involvement of Granulomatosis With Polyangiitis, descruction of vessels can lead to hemorrhage and bloody sputum called .

A

hemoptysis

105
Q

What is being demonstrated in this slide in this case of Granulomatosis With Polyangiitis

A

Focal and segmental glomerulosclerosis (periodic acid–Schiff stain), seen as a collection of scarred, obliterated capillaries and accumulations of matrix material in part of the affected glomerulus

-can lead to thrombosis

106
Q

What is being demonstrated in this slide in this case of Granulomatosis With Polyangiitis:

A

Crescentic glomerulonephritis (silver stain). Arrows indicate areas of necrosis and crescent formation. The segmental distribution in this case is typical of ANCA (anti-neutrophil cytoplasmic antibody)–associated crescentic glomerulonephritis.

107
Q

What is causing the cresent shape cells in this case of Granulomatosis With Polyangiitis

A

parietal cell proliferation forming epithelial crescents (crescentic glomerulonephritis).

108
Q

Who is the typical patient with Granulomatosis With Polyangiitis

A

middle aged man

109
Q

Classic presentatikons with Granulomatosis With Polyangiitis include (4):

A

bilateral pneumonitis with nodules and cavitary lesions (95%),

chronic sinusitis (90%),

mucosal ulcerations of the nasopharynx (75%),

and renal disease (80%)

110
Q

What are some more mild common symptoms that can also occur with Granulomatosis With Polyangiitis:

A

* Rash, myalgias, articular involvement, neuritis, and fever also may occur.

111
Q

Concerning Granulomatosis With Polyangiitis: what is the mortality rate at one year

A

80%

112
Q

What are the 4 treatments that have provent the outcome of Granulomatosis With Polyangiitis considerably:

A

steroids,

cyclophosphamide,

TNF inhibitors, and

anti-B-cell antibodies (rituximab)

113
Q

Of those that survive Granulomatosis With Polyangiitis, there is a high risk for relapses that may ultimately lead to what type of organ failure?

A

renal failure

114
Q

What vasculitis is a small-vessel necrotizing vasculitis classically associated with asthma, allergic rhinitis, lung infiltrates, peripheral eosinophilia, extravascular necrotizing granulomas, and infiltration of vessels and perivascular tissues by eosinophils

A

Churg-Strauss syndrome (allergic granulomatosis and angiitis)

115
Q

Churg-Strauss syndrome is a rare/common disorder with major associations with with Cutaneous involvement (with palpable purpura), gastrointestinal bleeding, and disease

A

rare; renal

116
Q

In regards to Churg-Strauss Syndrome (allergic granulomatosis and Cytotoxicity produced by the myocardial eosinophilic infiltrates often leads:

A

cardiomyopathy. (restrictive)

117
Q

Which vasculitis is characterized by segmental, thrombosing, acute and chronic inflammation of medium - and small - sized arteries, principally the tibial and radial arteries, with occasional secondary extension into the veins and nerves of the extremities?

A

Thromboangiitis Obliterans (Buerger Disease)

118
Q

Which vasculitis occurs almost exclusively in heavy tobacco smokers, and usually develops before 35 years of age

A

Thromboangiitis Obliterans (Buerger Disease)

119
Q

Which vasculitis has genetic predilection is suggested by an increased prevalence in certain ethnic groups (Israeli, Indian subcontinent, Japanese), and an association with certain HLA haplotypes?

A

Thromboangiitis obliterans (Buerger disease)

120
Q

Morphologicaly, Thromboangiitis obliterans (Buerger disease), has sharply segmental and transmural vasculitis of medium- and small-sized arteries, predominantly those of the u.

A

acute; chronic; extremities

121
Q

In the early stages of Thromboangiitis Obliterans, mixed inflammatory infiltrates are accompanied by thrombosis as is demonstrated in the slide.

A

Thromboangiitis Obliterans

note: luminal thrombosis with transmural inflammation (leukocytes infiltrates)

122
Q

Clinical features of which vasculitis include cold-induced Raynaud phenomenon, instep foot pain induced by exercise (instep claudication), and superficial nodular phlebitis?

A

Thromboangiitis Obliterans (Buerger Disease)

123
Q

With vascular insufficiency, such as in the case of Thromboangiitis Obliterans (Buerger Disease), it can lead to severe , even at rest

A

pain (from neural involvement)

124
Q

Concerning Thromboangiitis Obliterans, Chronic extremity ulcerations may develop, progressing over time to

A

gangrene

125
Q

What can prevent further attacks of vascular insufficiency and pain caused by Thromboangiitis Obliterans?

A

Smoking abstinence, however, the vascular lesions do not respond to smoking abstinence.

126
Q

What is the difference in location of the granulomas found in giant cell arteritis vs. granulomatosis with polyangiitis?

A

granulomas of giant cell arteritis are found within the vessel wall while granulomas of granulomatosis with polyangiitis are larger, spanning between vessels, and associated with areas of tissue necrosis.

127
Q
A