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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
Kawasaki. We'll describe this under
vessels
26
Henoch-Schonlein
purpura and IgA nephropathy | also arthritis, GI bleeding. A vasculitis with predominantly IgA pin the deposits. Mild in children severe in adults
27
Cryoglobinemia
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
28
Churg-strauss
"allergic granulomatosis" "eosinophilic granulomatosis with polyangiitis" another treacherous vasculitis
29
The hallmark of Churg Strauss is
abundant eosinophils in the peripheral blood and asthma - often new onset the vasculitis makes its appearance later, eosinophil are abundant in the vessel lesions
30
Churg Struss is thoug to have been precipitated in some cases by
leukotriene antagonist Rx for asthma
31
_____ is very common in Churg-Strauss. nobody know why
mononeuritis multiplex
32
Churg-strauss often kills by
occluding the microvasculature of the heart
33
Churg strauss morphology
abundang eosinophils with vasculitis. lung involvement, small vessels vasculitis, usually ANCA-pos with eosinophils
34
Behcet's Disease
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 ```
35
Behcet's you'll see
mouth ulcers, the rest of the picture is widely variable genital ulcers eye involvement is common, brain less so but does happen
36
Behcet's classic exam question
the link to HLA-B51
37
Behcet's involves what kind of vessels
usually small and medium vessels, though any vessel can be involved
38
Behcet's pathergy test
an ulcer will often develop at the site of a sterile needle prick
39
amyloid
the proteins that accumulate in beta pleated form | amyloid is beta pleated anything
40
Can stain stain amyloid with
congo red
41
congo red with "apple green birefringence"
amyloid
42
classic photo pair:
amyloid in a node, Congo Red
43
Amyloid AA
serum amyloid associated protein
44
serum amyloid associated protein (amyloid AA)
prolonged inflammation with macrophages, osteomyelitis, TB, leprosy, rheumatoid arthritis, etc
45
Amyloid AL
immunoglobulin light chains
46
immunoglobulin light chains (amyloid AL)
plasma cell neoplasm, not necessarily obrious
47
Amyloid ATTR (AF)
Transthyretin
48
Transthyretin (amyloid ATTR, AF)
the bad forms have a mutation - may get a liver transplant
49
amyloid B2m (AH)
Beta-two microbloulin
50
Beta two microgloculin (amyloid b2m AH)
HLA light chains, of course; longstanding kidney failure
51
Amyloid: glomeruli
are black on iodine H2SO4 treatment
52
Amyloid: atrium
many older people have a little amyloid
53
Amyloid in kiney __________ is common with glomerular involvement
nephrotic range prteinuria
54
Carpal tunnel syndrome often occurs in _____. Looks like _____ when stained
amyloidosis | apple green birefringence in flexor retinaculum
55
the first dz managed successfully by infusion of inhibitory RNA
transthyretin amyloidosis (AF)
56
people with defects in innate immunity (neutrophils, complement) usually have the most difficulty with
bacteria
57
Three syndromes with absent _______ are now known. The blood neutrophil count is very high bc they cannot leave the vessels
leukocyte adhesion molecules
58
the best known defect in phagolysosome function is
Chediak- Higashi
59
Chediak Higashi
the same lysosomal trafficking protein problem that prevents proper processing of lysosomes also interferes with melanosome formation and causes a neuropathy
60
defects in oxygen depenent bacterial killing mechanisms require the _______ system to handle common bacteria such as staph. They are called
the macrophage system | chronic granulomatous diseases
61
Complement component deficiencies SHOULD interfere with the ability to fight
bacteria
62
Deficiencies of C2, C4, and especially C1q mimic
lupus The alternate pathway works fine and handles the bacteria
63
C3 deficiency causes serious problems with ______ and _____
bacteria and with immune complex membranoproliferative glomerulonephritis
64
Deficiency in C5, C6, C7, C8 or C9 causes diffuculty dealing with ____ esp_____
bacteria | nisseria
65
C1 (esterase) inhibitor produces
hereditary angioedema. We don't really understand how this works, but its by far the most common of the complement protein deficiencies
66
T cell problems
Candida ("yeast"), later CMV, pneumocystis, etc
67
B cell problems
bacterial infections once mom's antibodies clear
68
Sever combined immunodeficiency
thymus with few T cells | slide - baby has left arm paralyzed after receiving oral polio vaccine
69
Most common scid, usually due to a mutation in _____
X -linked SCID, usually due to a mutation in the common g-chain (gc) of the interleukin receptors (IL2Rg)
70
The first dz to be cured by gene therapy
SCID: adenosine deaminase. The successful cure of a dz by gene therapy marked the beginning of overwhelming public acceptance of biotechnology within medicine
71
Burtons
X linked agammaglobulinemia
72
x linked agammaglobulinemia (cause)
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
Most brutons patins live happy healthy lives due to the development of
intravenous immunoglobulin
74
DiGeorge syndrome / thymic dysembriogenesis
lack of parathyroids lack of thymus midline cardiac defects
75
DiGeorge syndrome can have fatal
RSV
76
Hyper IgM
Pts are unable to switch over from making IgM to making IgG, IgA, and IgE
77
Hyper IgM mutation
most often, the gene encoding CD40 on the X chromosome is mutated (CD40LG)
78
Hyper IgM immunodeficiency
mixed immunodeficiency, often with low neutrophils and platelets, mf dysfunction and pneumocystis infetions
79
hyper IgM Tx
stem cell transplants are now in use
80
Common variable immunodeficienciy
many dz.'s, low Ig's | this time it's lots of lymphoid tissue, with poor germinal centers
81
Isolated IgA deficiency
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
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
sex linked lymphoproliferative syndrome
83
cure for sex linked lymphoproliferative syndrome
stem cell transplant
84
Tiny platelets, eczema, deficient T cells, X linked, WASp locus. Stem cell Rx; gene therapy under study
Wiskott-Aldrich
85
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
ataxia-telangiectasia
86
Hyper IgE (Job's syndrome)
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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