Path-myocarditis, pericarditis, vasculitis Flashcards

1
Q

In myocarditis, inflammation is the (cause/response) of the injury

A

Cause

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2
Q

Myocarditis

Active phase

A

Interstitial inflammatory infiltrate
Focal myocyte necrosis
+/- myocyte hypertrophy
Diffuse mononuclear infiltrate is most common (mostly lymphocytes)

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3
Q

In acute myocarditis, numerous ____ are associated with mononuclear cell infiltrates, including ___ whereas in chronic myocarditis, inflammatory cells such as ___ are mainly present in areas with ___.

A
  1. Necrotic myocytes
  2. CD3+ T cells
  3. CD68+ macrophages
  4. Fibrosis
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4
Q

In the chronic phase of myocarditis, ___ resolves and one of these develops:

A

Inflammation

  1. Resolution of lesions
  2. Progressive fibrosis
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5
Q

10 Most common viral causes of myocarditis

A
  1. Parvovirus B19
  2. HHV6
  3. Coxsackie A and B
  4. Other enteroviruses
  5. Hep C
  6. Adenovirus
  7. Echovirus
  8. CMV
  9. HIV
  10. Influenza A and B
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6
Q

Most common bacterial causes of myocarditis

A

C. diphtheria
N. meningitidis
Borrelia burgdorferi (Lyme disease)
Ehrlichia chaffeensis

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7
Q

The leading cause of dilated cardiomyopathy in Central and South America is ___. Myocarditis occurs in the majority of these infected patients. What is pathognomonic for this disease?

A

T. cruzi (Chagas disease)

Left ventricular apical aneurysms

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8
Q

The most common helminth to cause myocarditis is ___

A

Trichinella spiralis (undercooked or raw pork, or wild game)

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9
Q

poststreptococcal myocarditis (rheumatic fever) results from immune responses to ___ strep. Antibodies directed against ___ proteins of strep cross-react with self-antigens in the heart. ___ cells specific for strep peptides react with self-antigens and produce ____. RF typicallay arises ____ after strep pharyngitis infections.

A
  1. Group A
  2. M
  3. CD4+ T
  4. Cytokines that activate macrophages
  5. 10 days-6 weeks
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10
Q

____ are composed of lymphocytes, few plasma cells and activated macrophages (called ___) which are pathognomonic for RF and have round-oval nuclei with chromatin dispersed around a central wavy ribbon (hence the nickname “___”)

A
  1. Aschoff bodies
  2. Anitschkow cells
  3. Caterpillar cells
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11
Q

Diffuse inflammation and Aschoff bodies may be found in the ____ during acute RF. This is called ___

A
  1. Endo-, myo-, or pericardium

2. Pancarditis

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12
Q

Myocarditis with many non-necrotizing epithelioid granulomas

Negative for organisms

A

Sarcoidosis

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13
Q

Rapid onset of chest pain, fever; and hemodynamic compromise
Often with V tach or AV block
Unknown cause
Poor prognosis
Often fatal without transplant
Widespread inflammatory infiltrate with lymphocytes, plasma cells, macrophages, eosinophils, and multinucleated giant cells

A

Giant cell myocarditis

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14
Q

Sudden intense emotional or physical stress due to production of catecholamines, which cause myocardial stunning

A

Pheochromocytoma

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15
Q

Diagnosis of myocarditis includes:

A
ECG
Cardiac biomarkers
CXR
Labs often normal
Recommend testing BNP or NT-proBNP if HF is suspected
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16
Q

Differential dx for myocarditis

A

Ischemic or valvular heart disease
Congenital heart disease
Pulmonary disease

*echo is helpful in distinguishing

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17
Q

True or false:
Cardiomyopathies are major structural abnormalities that are limited to the myocardium and are secondary to ischemia, valvular disease, or hypertensive heart disease.

A

False. First half is true. But they are NOT secondary to any of those

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18
Q

3 types of cardiomyopathies

A
  1. Dilated (DCM)
  2. Hypertrophic (HCM)
  3. Restrictive (RCM)
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19
Q

Definition of DCM

A

Dilation and impaired contraction of one or both ventricles, with systolic dysfunction.

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20
Q

Most patients with DCM present between the ages of ___. Symptoms vary

A

20-60

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21
Q

Causes of DCM

A
  1. Idiopathic (~50%)
  2. Infectious myocarditis
  3. Infiltrative disease (sarcoidosis, carcinoma)
  4. Periosteum state
  5. Toxins (ethanol, cocaine, cobalt, anthracyclines)
  6. CT disease
  7. Metabolic disorders
  8. End-stage renal disease
  9. Genetic abnormalities
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22
Q

Gross morphology of DCM

A

Heart is large and flabby
Poor contractile function
Valves and coronary arteries are usually normal

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23
Q

Prognosis of DCM

A

Indolent and progressive CHF

Fewer than 50% survive more than 5 yrs after onset of symptoms unless they receive a transplant

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24
Q

HCM is characterized by __ and is not caused by ___

A

Unexplained septal or LV hypertrophy that is NOT caused by chronic pressure overload (not caused by systemic HTN, congenital heart defects, or aortic stenosis)

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25
Q

Gross morphology of the heart in HCM

A
Heavy
Muscular
Hypercontractile
Stiff 
Poor diastolic relaxation 
Ventricular outflow obstruction is seen in 1/3 of cases 
Asymmetric thickening of IV septum 
Banana like shape of LV cavity
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26
Q

Microscopic morphology of HCM

A
  1. Cardiac myocyte hypertrophy or disarray
  2. Enlarged hyperchromatic nuclei
  3. Interstitial replacement fibrosis of myocardium
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27
Q

A majority of HCM cases involve a mutation of the ___ gene, typically with ___ inheritance. Other genes involved encode ___ and ___

A
  1. Myosin heavy chain
  2. Autosomal dominant
  3. Troponin T and myosin binding protein C
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28
Q

Symptoms of HCM

A
  • most patients are asymptomatic
  • sometimes v fib and sudden cardiac death
  • symptomatic progression is usually slow
  • symptomatic disease usually presents in young adults with dyspnea, angina, and/or syncope
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29
Q

Complications of HCM

A
  1. A fib
  2. Mural thrombus
  3. Embolization
  4. Congestive heart failure
  5. Infectious endocarditis
  6. Sudden cardiac death
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30
Q

___ is the most common cause of sudden cardiac death in young athletes

A

HCM

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31
Q

RCM is characterized by __

A

Abnormally rigid ventricles that are usually of normal thickness and are not dilated.
Impaired diastolic filling
Systolic function is usually normal
Non-specific interstitial myocardial fibrosis

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32
Q

RCM usually results from ___

A

Either fibrosis of the endomyocardium or infiltration of the myocardium by an abnormal substance

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33
Q

___ is the most common cause of RCM in non tropical countries

A

Amyloidosis

34
Q

Mainly a disease of children and young adults in tropical regions.
Fibrosis of ventricular endocardium and subendocardium

A

Endomyocardial fibrosis

35
Q

Associated with peripheral blood eosinophilia and eosinophilic infiltration of organs. Endomyocardial fibrosis, typically with large mural thrombus. Can be rapidly fatal

A

Loeffler endocarditis

36
Q

Endocardial fibroelastosis is most common in ___

A

Children under 2

37
Q

Other cardiomyopathies

A
  1. Arrhythmogenic right ventricular CM
  2. Stress-induced CM
    - transient left ventricular apical ballooning, takotsubo CM, and broken heart syndrome CM
38
Q

True or false:

Arrhythmogenic right ventricular CM is autosomal recessive

A

False. Autosomal dominant

39
Q

Gross morphology of Arrhythmogenic right ventricular CM

A
  1. RV wall is thinned due to loss of myocytes
  2. Extensive fatty infiltration
  3. Fibrosis
40
Q

Acute coronary syndrome, usually with ST elevation, with focal systolic dysfunction of the LV, occurring in the absence of critical CAD

A

Stress-induced CM

41
Q

Stress-induced CM occurs mostly in ___

A

Older women

42
Q

Symptoms of Stress-induced CM

A
Acute substernal chest pain (most common)
Dyspnea
Syncope
HF
Arrhythmias
Cardiogenic shock
43
Q

Lab findings for Stress-induced CM

A

Elevated troponin
Normal or slightly elevated CK
BNP or NT-proBNP usually elevated

44
Q

Prognosis of Stress-induced CM

A

Even in severe cases, almost all patients recover within 1-4 weeks

45
Q

___ and ___ are the most common pericarditis

A

Fibrinois and serofibrinous

46
Q

Caseous pericarditis is ___ until proven otherwise

A

Tuberculosis

47
Q

___ obliterates the pericardial sac and leaves the parietal layer tethered to mediastinal tissue.
___ is marked by thick, dense, fibrous obliteration of the pericardial sac, often with calcification. This limits diastolic expansion and restricts CO

A

Adhesive mediastinopericarditis

Constrictive pericarditis

48
Q

In pericardial effusions, inflammation typically results in ___ while non-inflammatory conditions typically result in ___

A

Exudates

Transudates

49
Q

Chylous effusions occur in response to ___

A

Lymphatic obstruction of pericardial drainage by tuberculosis or a tumor

50
Q

Transudate vs. exudate

A
Transudates:
clear
low cell counts
specific gravity <1.015
protein <3 g/dL
Fluid:serum protein ratio <0.5
Fluid:serum LDH ratio <0.6
Fluid:serum glucose ratio >1

*Exudates are all opposite

51
Q

The most common cause of pericardial effusion is ___

A

Congestive heart failure

52
Q

Too much fluid causes restriction of heart motion, leading to ___, where pericardial fluid accumulates under high pressure, compresses the cardiac chambers, and severely limits filling. This can lead to hypotensive shock and death

A

Cardiac tamponade

53
Q

Takayasu arteritis is vasculitis of ___ vessels while Kawasaki disease is vasculitis of ___ vessels.
What is Wegener’s disease?

A

Large
Medium
Granulomatis is with polyangiitis (small vessels)

54
Q

The two most common pathogenic mechanisms of vasculitis are ___ and ___

A

Immune-mediated inflammation and direct invasion of vascular walls by pathogenic organisms

55
Q

Non-infectious vasculitis is mainly mediated by ___

A

Immune complex

56
Q

What is the most common form of vasculitis in the elderly?

Which is most commonly seen in young Asian or African females?

A

Temporal (Giant Cell) Arteritis

Takayasu Arteritis

57
Q

Chronic vasculitis of medium to large sized arteries. It is a systemic disease that mainly involves the cranial vessels, but can involve the aortic arch and its branches

A

Temporal (Giant Cell) Arteritis

Giant Cell aortitis

58
Q

Etiology for Temporal Arteritis

A

Immune response against vascular wall antigens

59
Q

Morphology of Temporal Arteritis

A

Patchy, medial granulomatous inflammation centered on the internal elastic lamina of medium-small arteries

60
Q

Chronic granulomatous vasculitis of unknown etiology that involves the aorta and its major branches

A

Takayasu Arteritis

61
Q

Progression of Takayasu Arteritis is characterized by ___

A

Ocular disturbances, neurological symptoms, and upper extremity pulselessness (pulseless disease)

62
Q

How can you distinguish between Takayasu Arteritis and Giant Cell Arteritis histologically?

A

You can’t

63
Q

Systemic disease characterized by necrotizing vasculitis of small-medium sized arteries of kidney, heart, liver, and GI tract. Not pulmonary circulation

A

PAN

Polyarteritis Nodosa

64
Q

PAN usually affects ____. It is fatal if untreated but with treatment 90% may remit or be cured

A

Middle aged and older adults, but can affect any age

65
Q

1/3 of PAN cases are associated with ___ and ____, the rest are ___. There is no association with ____.

A
  1. Chronic hep B
  2. immune complex deposition
  3. Idiopathic
  4. ANCA
66
Q

Acute, febrile, usually self-limited illness of infancy and childhood. Results from arteritis affects my large to medium sized or even small vessels. May follow a viral illness

A

Kawasaki disease aka

Mucocutaneous lymph node syndrome

67
Q

Kawasaki disease primarily affects ___

A

Children under 4 (80%)

68
Q

Kawasaki disease is the most common cause of ___

A

Acquired heart disease in children in the U.S.

69
Q

Treatment for Kawasaki disease

A

Aspiring

IV gamma globulin

70
Q

Necrotizing vasculitis of Arteritis les, capillaries, and venules; unlike PAN, all lesions are at the same stage and are distributed more widely

A

Microscopic polyangitis

71
Q

Necrotizing granulomas of upper resp tract, lungs, or both. Of medium or small vessels, often crescentic, glomerulonephritis. Clinically resembles PAN

A

Granulomatosis with Polyangitis aka

Wegeners Granulomatosis

72
Q

Granulomatosis with Polyangitis aka

Wegeners Granulomatosis commonly affects ___

A

Males over 40

73
Q

If untreated, ___% of people with Granulomatosis with Polyangitis die within 1 year.

A

80%

74
Q

90% of cases of active generalized Granulomatosis with Polyangitis have ___ and 60% of limited disease have ____. ____ is usually negative, unless there is ____

A

c-ANCA (PR3-ANCA)
c-ANCA
P-ANCA
Crescentic glomerulonephritis

75
Q

Acute and chronic segmental thrombosing inflammation of medium m and small vessels (mainly tibial and radial) with occasional extension into veins and nerves of extremities

A

Thromboangiitis obliterans aka

Buergers disease

76
Q

Thromboangiitis obliterans is most common in ___

A

Far and Middle East, strong association with cigarettes, almost exclusively in young adults (usually under 35)

77
Q

Paroxysmal cyanosis of the extremities, particularly the fingers and toes; also tip of nose, earlobes, or lips. Exaggerated vasoconstriction of arteries or arterioles.

A

Raynaud phenomenon

78
Q

Which disease is described as patriotic? Why?

A

Raynaud phenomenon
red, white, and blue color change from most proximal to distant, correlating with proximal vasodilation, central vasoconstriction, and distal cyanosis

79
Q

Primary Raynaud Phenomenon

A

Young women
Symmetrically affects extremities
Severity is static over time
Usually benign
Chronicity can lead to atrophy of skin, subcutaneous tissues, and muscle
Ulceration and ischemic gangrene are rare

80
Q

Secondary Raynaud Phenomenon

A

Asymmetric involvement of extremities
Progressively worsens
Ulceration and ischemic gangrene are more common