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Pathology > Pathology of Immunologic Injury 3: Immune mediated vasculitis, amyloidosis, and non-HIV related immunodeficiency > Flashcards

Pathology of Immunologic Injury 3: Immune mediated vasculitis, amyloidosis, and non-HIV related immunodeficiency Flashcards

1
Q

the _____ family is an especially treacherous group of dz.’s with various immune based etiologies

A

vasculitis

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2
Q

vasculitis

A

vessels rupture and/or undergo thrombosis due to damage from inflammation. They are famously deadly and famously easy to miss. ALl can be managed with todays medications

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3
Q

Tipoffs to vasculitis

A

mononeuritis multiplex
palpable purpura
anybody who you just can’t tell why they are sick

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4
Q

polyarteritis Nodosa - large vessel type

A 
patych inflammation of all 3 layers of larger arteries
veins are spared
Remember hep B, hep C and cyoglobulins as etiologies
migrane
stroke
vague aches and pains
lungs spared
heart attack 
liver infarcts
bowel infarcts
hypertension
gangrene
peripheral nerve damage
usually no hematuria
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5
Q

today, polyarteritis nodosa means

A

larger arteries, ANCA negative

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6
Q

polyarteritis nodosa

A

true aneurysms.
The process perpetuates itself locally and is patchy
the weakened wall tends to ballon out. These may burst, or more often may undergo thrombosis

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7
Q

polyarteritis - the pink necrotic stuff is

A

fibrinoid.

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8
Q

polyarteritis nodosa classic illustration

A

you’ll see lesions of different ages at the same location

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9
Q

polyarteritis nodosa findings in elastic stain

A

can show old damage to elastin, damaged elastic.

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10
Q

polyarteritis nodosa

A

damage to elastin
aneurysms,
small aneurysms with thrombus
fibrinoid and organized thrombus

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11
Q

polyarteritis nodosa - when it’s not the Hep B vasculitis, ____ often helps

A

immunosupression

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12
Q

microscopic polyangiitis (small vessel polyarteritis)

A

smaller veins and arteries show patchy 3-layer inflammation
positive anti neutrophil cytoplasm test (p-ANCA)

vague aches and pains
stroke 
heart attack
hemptysis and inflitrates
bowel infarcts
nephritis/kidney failure
gangrene
peripheral nerve damage
hematuria
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13
Q

microscopic polyangiitis characterized by

A

fibrinoid necrosis

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14
Q

microscopic polyangiitis/small vessel polyarteritis

this is the vasculitis most likely to involve

A

the capillaries

capillaritis and alveolar hemorrhage in lung
nuclear dust from dead neutrophils “leukocytoclastic vasculitis”
RPGN

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15
Q

segmental necrosis in a glomerulus suggests

A

vasculitis

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16
Q

microscopic polyangiitis/ small vessel polyarteritis morphology

A

broken basement membrane; crescent in the kidney

in skin, can’t tell from drug rash, Henoch=Schonlein, lupus, others

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17
Q

p-ANCA (perinuclear patter, anti-neutrophil cytoplasmic antibody)

A

think P

Polyarteritis, small vessel type. Often anti-myeloperoxidase

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18
Q

Wegener’s Granulomatosis

A

Wegener’s is infamous for it’s sublet presentation, and its lethality if (and only if) missed.
Caused by autoantibodies against proteinase 3
positive antineutrophil cytoplasm test c-ANCA
granulomas and patchy necrosis in arteries and veins

sore eye, 
sore ear
stuffy nose
chest x ray blip
sore joints 
traces of blood in urine
destruction of the face
lung cavities and bleeds
permanent kidney failure
gangrene
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19
Q

Looked on x ray like a lung cancer

A

wegener’s granulomatosis

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20
Q

new name for wegner’s granulomatosis

A

granulomatosis with polyangiitis

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21
Q

Wegener’s morphology

A

Granulomatous vasculits and necrotic areas
Kidney: glomerulonephritis with segmental necrosis. Crescent (RPGN) and broken GBM; red cell cast

granulomas and vasculitis. cavitating lesions

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22
Q

wegener’s granulomatosis - eye

A

episcleritis

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23
Q

wegener’ facial features

A

“saddle nose” from necrosis of the nasal septum

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24
Q

C-ANCA

A

think wegeners (cytoplasmic pattern of the anti neutrophil cytoplasmic antibodies). Anti proteinase 3

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25
Q

Kawasaki. We’ll describe this under

A

vessels

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26
Q

Henoch-Schonlein

A

purpura and IgA nephropathy

also arthritis, GI bleeding. A vasculitis with predominantly IgA pin the deposits. Mild in children severe in adults

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27
Q

Cryoglobinemia

A

marginally soluble protein (hep C antibody and antigen complexes, rheumatoid factors, other) preciptiate in the cooler places and/ or where blood is concentrated (i.e. kidney)

another cause of vasculitis, stay warm if you have it

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28
Q

Churg-strauss

A

“allergic granulomatosis”
“eosinophilic granulomatosis with polyangiitis”
another treacherous vasculitis

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29
Q

The hallmark of Churg Strauss is

A

abundant eosinophils in the peripheral blood and asthma - often new onset
the vasculitis makes its appearance later, eosinophil are abundant in the vessel lesions

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30
Q

Churg Struss is thoug to have been precipitated in some cases by

A

leukotriene antagonist Rx for asthma

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31
Q

_____ is very common in Churg-Strauss. nobody know why

A

mononeuritis multiplex

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32
Q

Churg-strauss often kills by

A

occluding the microvasculature of the heart

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33
Q

Churg strauss morphology

A

abundang eosinophils with vasculitis.
lung involvement, small vessels
vasculitis, usually ANCA-pos with eosinophils

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34
Q

Behcet’s Disease

A

AI against heat shock proteins produces one of the truly great mimics
Pathergy test 48 hr after a sterile needlestick, an ulcer or blister.
affects arteries and veins of all sizes

mouth ulcers (always)
eye lesions
genital ulcers
skin lesions
Neurologic syndromes
infarcts of anything
thrombosis of anything
amyloidosis
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35
Q

Behcet’s you’ll see

A

mouth ulcers, the rest of the picture is widely variable
genital ulcers
eye involvement is common, brain less so but does happen

36
Q

Behcet’s classic exam question

A

the link to HLA-B51

37
Q

Behcet’s involves what kind of vessels

A

usually small and medium vessels, though any vessel can be involved

38
Q

Behcet’s pathergy test

A

an ulcer will often develop at the site of a sterile needle prick

39
Q

amyloid

A

the proteins that accumulate in beta pleated form

amyloid is beta pleated anything

40
Q

Can stain stain amyloid with

A

congo red

41
Q

congo red with “apple green birefringence”

A

amyloid

42
Q

classic photo pair:

A

amyloid in a node, Congo Red

43
Q

Amyloid AA

A

serum amyloid associated protein

44
Q

serum amyloid associated protein (amyloid AA)

A

prolonged inflammation with macrophages, osteomyelitis, TB, leprosy, rheumatoid arthritis, etc

45
Q

Amyloid AL

A

immunoglobulin light chains

46
Q

immunoglobulin light chains (amyloid AL)

A

plasma cell neoplasm, not necessarily obrious

47
Q

Amyloid ATTR (AF)

A

Transthyretin

48
Q

Transthyretin (amyloid ATTR, AF)

A

the bad forms have a mutation - may get a liver transplant

49
Q

amyloid B2m (AH)

A

Beta-two microbloulin

50
Q

Beta two microgloculin (amyloid b2m AH)

A

HLA light chains, of course; longstanding kidney failure

51
Q

Amyloid: glomeruli

A

are black on iodine H2SO4 treatment

52
Q

Amyloid: atrium

A

many older people have a little amyloid

53
Q

Amyloid in kiney __________ is common with glomerular involvement

A

nephrotic range prteinuria

54
Q

Carpal tunnel syndrome often occurs in _____. Looks like _____ when stained

A

amyloidosis

apple green birefringence in flexor retinaculum

55
Q

the first dz managed successfully by infusion of inhibitory RNA

A

transthyretin amyloidosis (AF)

56
Q

people with defects in innate immunity (neutrophils, complement) usually have the most difficulty with

A

bacteria

57
Q

Three syndromes with absent _______ are now known. The blood neutrophil count is very high bc they cannot leave the vessels

A

leukocyte adhesion molecules

58
Q

the best known defect in phagolysosome function is

A

Chediak- Higashi

59
Q

Chediak Higashi

A

the same lysosomal trafficking protein problem that prevents proper processing of lysosomes also interferes with melanosome formation and causes a neuropathy

60
Q

defects in oxygen depenent bacterial killing mechanisms require the _______ system to handle common bacteria such as staph. They are called

A

the macrophage system

chronic granulomatous diseases

61
Q

Complement component deficiencies SHOULD interfere with the ability to fight

A

bacteria

62
Q

Deficiencies of C2, C4, and especially C1q mimic

A

lupus

The alternate pathway works fine and handles the bacteria

63
Q

C3 deficiency causes serious problems with ______ and _____

A

bacteria and with immune complex membranoproliferative glomerulonephritis

64
Q

Deficiency in C5, C6, C7, C8 or C9 causes diffuculty dealing with ____ esp_____

A

bacteria

nisseria

65
Q

C1 (esterase) inhibitor produces

A

hereditary angioedema. We don’t really understand how this works, but its by far the most common of the complement protein deficiencies

66
Q

T cell problems

A

Candida (“yeast”), later CMV, pneumocystis, etc

67
Q

B cell problems

A

bacterial infections once mom’s antibodies clear

68
Q

Sever combined immunodeficiency

A

thymus with few T cells

slide - baby has left arm paralyzed after receiving oral polio vaccine

69
Q

Most common scid, usually due to a mutation in _____

A

X -linked SCID, usually due to a mutation in the common g-chain (gc) of the interleukin receptors (IL2Rg)

70
Q

The first dz to be cured by gene therapy

A

SCID: adenosine deaminase.
The successful cure of a dz by gene therapy marked the beginning of overwhelming public acceptance of biotechnology within medicine

71
Q

Burtons

A

X linked agammaglobulinemia

72
Q

x linked agammaglobulinemia (cause)

A

is fairly common and is caused by lack of burton’s tyrosine kinase, which lets B cells mature

especially after the loss of maternal antibodies by around six months, these kids have troule with bacteria (H flu, pneumococcus, staph) and with giardia and enteroviruses

they do need to avoid the live polio and MMR vaccines

73
Q

Most brutons patins live happy healthy lives due to the development of

A

intravenous immunoglobulin

74
Q

DiGeorge syndrome / thymic dysembriogenesis

A

lack of parathyroids
lack of thymus
midline cardiac defects

75
Q

DiGeorge syndrome can have fatal

A

RSV

76
Q

Hyper IgM

A

Pts are unable to switch over from making IgM to making IgG, IgA, and IgE

77
Q

Hyper IgM mutation

A

most often, the gene encoding CD40 on the X chromosome is mutated (CD40LG)

78
Q

Hyper IgM immunodeficiency

A

mixed immunodeficiency, often with low neutrophils and platelets, mf dysfunction and pneumocystis infetions

79
Q

hyper IgM Tx

A

stem cell transplants are now in use

80
Q

Common variable immunodeficienciy

A

many dz.’s, low Ig’s

this time it’s lots of lymphoid tissue, with poor germinal centers

81
Q

Isolated IgA deficiency

A

you may never know until you have your second blood transfusion
anaphylaxis occurs and workup reveals that you are allergic to the donors IgA

82
Q

Mutant SH2D1A makes a portion of a signal transduction protein that is defective. The poor response to viruses eventually allow the Epstein Barr virus to produce a deadly lymphoma-like overgrowth of lymphocytes, or destroy liver and/or marrow

A

sex linked lymphoproliferative syndrome

83
Q

cure for sex linked lymphoproliferative syndrome

A

stem cell transplant

84
Q

Tiny platelets, eczema, deficient T cells, X linked, WASp locus. Stem cell Rx; gene therapy under study

A

Wiskott-Aldrich

85
Q

The reasons for lab abnormalities aren’t well understood:

Tcell counts and/or low antibody levels are often, but not always, part of the picture.

Despite this illness being described as an immunodeficiency, opportunistic infections are rarely seen AI and cancers are common

A

ataxia-telangiectasia

86
Q

Hyper IgE (Job’s syndrome)

A

at least three illnesses with lots of IgE and too little of other antibodies, with faulty neutrophil movement. There may be other birth defects. Staph is the most troublesome of the bacteria

Pathology (15 decks)

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