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Pathology > Pathology of Immunologic Injury 2: the systemic autoimmune diseases > Flashcards

Pathology of Immunologic Injury 2: the systemic autoimmune diseases Flashcards

1
Q

central tolerance happens

A

mostly before birth

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2
Q

central tolerance T cells and b cells

A

Autoreactive T cells are killed

autoreactive B cells have their receptors modified

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3
Q

The idea that autoimmunity may have to do with failure of ____ has some empirical support

A

central tolerance

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4
Q

Autoimmune polyendocrinopathy type I is a fortunately uncommon autosomal ______ illness in which patients tend to suffer from

A

recessive
several different T cell mediated autoimmune diseases *as well as candidiasis and epidermal problems

  • hypothyroidism, chronic hepatitis, vitiligo, pernicious anemia, type I diabetes
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5
Q

Autoimmune polyendocirnopathy type one locus is ath

A

AIRE (autoimmune related), which is a key protein in deleting auto reactive T cells before birth

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6
Q

after birth, auto reactive lymphocytes may be kept from causing trouble by several ________mechanisms

A

peripheral tolerance

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7
Q

Peripheral tolerance: lymphocytes recognizing self antigen may be

A

rendered no responsive by the ways in which they are presented.

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8
Q

mutations in CTLA-4 and PD-1 - immune response brakes are each linked to

A

risk for autoimmune disease

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9
Q

Regulatory t cells are distinguished by

A

CD25/IL-2 a chain and foxp3/scurfin

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10
Q

Regulatory t cell function

A

suppress immunity to self

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11
Q

mutations in Treg cells (in either the CD25 or Foxp3) cause

A

a rare, grisly autoimmune disease that may require marrow transplantation

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12
Q

When auto reactive T cells activate, they undergo

A

apoptosis.

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13
Q

mice with defective fas or fasL get a ____ illness

A

lupus like

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14
Q

Thinking about autoimmunity focuses on

A

polygenic inheritance with some environmental stimulus as well

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15
Q

The most often mutated gene in autoimmuniity is

A

PTPN22, a tyrosine kinase involved in lymphocyte responses

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16
Q

Other mutations in autoimmunity are

A

NOD2 (a microbe sensor)
CD25 (Treg)
IL-17 a chains

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17
Q

actual triggers of autimmunity focus on

A

molecular mimicry by infections

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18
Q

molecular mimicry

A

many known examples, the most famous is sensitization to strep cross-reacting with heart - rheumatic fever

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19
Q

rheumatic fever

A

molecular mimicry

sensitization to strep cross reacting with the heart

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20
Q

mutated lymphocytes

A

before birth, we destroy our auto reactive lymphocytes. are the lymphocytes of people with autoimmune disease mutants that form after birth?

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21
Q

Microchimerism

A

everyone carries a few of their mother’s cells
all mothers keep a few of the cells of the children they have carried
rumored to be more abundant in those with autoimmune disease

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22
Q

Antinuclear antibodies- rim pattern.

A

Think classic system lupus, anti ds-DNA

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23
Q

Detect ANAs by

A

applying patient serum to normal cells then lighting up Abs that stick

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24
Q

antinublear antibodies: homogenous pattern

A

think drug-lupus

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25
Q

antinuclear antibodies: speckled pattern

A

think anything

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26
Q

antinuclear antibodies: centromere pattern

A

think CREST

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27
Q

antinublear antibodies: nucleolar pattern

A

think Scleroderma

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28
Q

anti-dsDNA

A

sensitive and specific for systemic lupus.

rim pattern on ANA

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29
Q

anti ss DNA

A

think of drug induced lupus

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30
Q

anti histone

A

think of drug induced lupus

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31
Q

anti-Sm (Smith)

A

only about 1/3 of systemic lupus patients have this autoantibodie, but it essentially makes the diagnosis

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32
Q

anti-Ro/SSA (Sjorgren’s A)

A

Most Sjogren’s patients, and many other folks. Much hated as the cause of neonatal lupus (heart block) when the mother has the antibody

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33
Q

anti-La/SSB (Sjogren’s B)

A

most Sjogren’s patients and many other folks

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34
Q

anti U1RNP (U1 ribonucleoprotein)

A

if this is your predominant autoantibody, you have mixed connective tissue disease

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35
Q

anti-Scl70 (anti topoisomerase)

A

common in patients with scleroderma, fairly specific

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36
Q

anti-RNA polymerase III:

A

common and fairly specific in scleroderma,

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37
Q

anti-centromere

A

the antibody for CREST, the “milder version of scleroderma”

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38
Q

Anti Jo (Jo, transfer RNA synthetase):

A

seen in many patients with polymyositis/dermatomyositis. Runs with severe disease, lung damage, arthritis, and mechanic’s hand

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39
Q

Anti-Mi2 (against a helices that remodels nucleosomes)

A

seen in dermatomyositis, especially when the rash is prominent

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40
Q

Anti-SRP (signal recognizing polypeptide)

A

may define a sever polymyositis variant with severe necrotizing myopathy

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41
Q

anty P1-55 TIF1g and anti P140/MJ

A

juvenile and paraneoplastic dermatomysoitis

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42
Q

Lupus, scleroderma, Sjogrens, and polymyositis/ dermatomyositis may all be accompanied by a ______. unlike rheumatoid arthritis, there is usually no _____

A

synovitis

major damage to the joints

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43
Q

Butterfly rash

A

lupus

wolf-like.

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44
Q

systemic lupus erytematosus

A

susceptibility genes +external triggers

persistent high level antinuclear IgG antibody production

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45
Q

Lupus

A

is a systemic AI dz characterized primarily by autoantibodies, primarily against nuclear antigens

is a dz of exacerbations and remissions. UV light triggers flareups. XX and XXYs are much more likely to be affected than XYs and XOs. Identical twins are around 30% concordant; dizygotic twins only about 2%. People of european ancestry are at relatively less risk

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46
Q

Etiology of lupus

A

unknown. the linked genetic loci are involved with interferons and lymphocyte responses

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47
Q

who is immune to new onset SLE

A

no one

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48
Q

Models for lupus include several

A

drug reactions
(hydralazine and procainamide, and anti-aTNF)

genetic deficiencies in certain complement components
(perhaps these percent clearance of immune complexes; C1q is important in removing apoptotic bodies

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49
Q

the recent 30 new susceptibility loci for SLE are related to

A

interferons

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50
Q

Most of the tissue injury in SLE seems to be related to

A

deposition of immune complexes (typeIII).

the destruction of RBC, neutrophils and/or platelets can be due to antibodies against these blood components (TypeII)

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51
Q

lupus anticoagulants

A

antibodies against phopholipid complexes produce lupus anticoagulants (they actually make the blood clot more in people, though less in the lab) and cause false positive syphilis screening tests

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52
Q

You need ___ of 11 criteron

A

4

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53
Q

Criterion for SLE, you need for of 11

A

malar rash
discoid rash
photosensitivity
oral ulcers
arthritis
serositis
Renal disorder (proteinuria or celluar casts)
Neuro disorder (seizures or psychosis)
Hematologic disorder (hemolysis, low WBC/lymphs/pl’s)
Immune (anti-DNA/anti-Sm/antiphopholipid/false syph)
antinuclear antibodies

54
Q

what triggers lupus

A

apoptoti bodies, alfalfa, much more

55
Q

Hematoxylin bodies

A

stripped nuclei acted on by ANA’s in lupus

56
Q

LE cell

A

a stippped nucleus opsonized by anti nuclear antibodies and now being devoured by phacocytes

57
Q

Similar homogenized nuclei produce

A

hematoxylin bodies

58
Q

lupus in kidney

A

lupus glomerulopathy

59
Q

lupus vasculitis

A

immune complex mediated

60
Q

spleen in lupus

A

adventitial onion skinning in spleen

61
Q

In the kidney with lupus, we see

A

lupus glomerular immune complex deposits
glomerulonephritis
segmental necrotizing lupus glomerulonephritis can happen

62
Q

wire loop

A

a massive immune complex deposit in a glomerulus

63
Q

interstitial nephritis in lupus

A

autoantibodies against tubules

64
Q

if you were to biopsy the butterfly rash, you will find

A

hydropic change of the basal layer and vasculitis

65
Q

lupus band test:

A

granular immunoglobulin and complement at the dermal -epidermal junction

66
Q

mouth ulcers in lupus are

A

infarcts from vasculitis (so are the familiar canker sores that many of you have had)

67
Q

Lupus pericarditis

A

fibrin on the heart surface

68
Q

libman sacks endocarditis in lupus

A

sterile thrombi on the lines of closure

all surfaces of leaflets

69
Q

neonatal lupus

A

child has rash. more ominous is heart block caused by mom’s autoantibodies, famously anti-Ro

70
Q

Blood

A

polyclonal gammopahty (increased Igs of many kinds)
AI lysis of red cells (Ab against your own RBCS) “coombs positive hemolysis)
AI destruction of neutrophils
AI destruction of platelets
low total lymphocyte count (

71
Q

______ kill many lupus patients

A

DVT/ pulmonary emboli

72
Q

Brain

A

CNS involvement in its with SLE can produce physiological problems that include personality disorders and frank psychoses. Grand mal seizures may be seen, as well as strokes

73
Q

behavioral changes in lupus

A

may range from mild to florid psychosis

74
Q

Lupus patients feel _____ than they look

A

much worse

75
Q

subacute cutaneous lupus

A

Anti-Ro is usually seen. many patients with this rash have lupus; others have Sjogren’s or rheumatoid arthritis. (leave diagnosis to Derm. Stay out of sun, and treat with topical glucocorticoids)

76
Q

Discoid lupus

A

the rash is familiar - patches with scales and lost hairs
“wolf bites?”
many its with systemic lupus have discoid lupus as well
just discoid=as common as systemic.
keep out of sun

77
Q

can see patches of hyperkeratosis as plugs. hydropic change, lymphocytes in patches of

A

discoid lupus

78
Q

Drug induced lupus

A

historically hydralazine, procainamide, and isoniazid.

anti-histone antibodies and a homogenous pattern

79
Q

Drug induced lupus - where do we see it

A

today 1 in 250 its receiving anti -a TNF rx will develop a rash and anti dsDNA antibodies

80
Q

incomplete lupus

A

some ppl will seem to have lupus (feeling terrible most of the time) and have some of the symptoms/signs/labs, but not meet the official 4/11.

the prognosis is generally good

81
Q

Sjoren’s

A

the salivary and lacrimal glands fill with very angry lymphocytes

82
Q

Sjorens syndrome

A

is a very poorly understood syndrome in which autoimmunity damages the salivary and lacrimal glands

HLA linkages are known, Viral syndromes (HIV, HTLV-1, Hep C) can mimic it, it tends to run with other systemic AI diseases (lupus, scleroderma, RA, PM.DM, MCTD) and most patients have a pari of autoantibodies (anti -SSA/Ro and anti SSB/La)

83
Q

Sjogrens and teeth

A

having no saliva is bad for the teeth

84
Q

Kerotoconjunctivitis sicca

A

sever dry eyes of sjorens

85
Q

xerostomia

A

severe dry mouth (sjorens)

86
Q

Mikulicz’s syndrome

A

much enlarged salivary and lacrimal glands, usu in the setting of dry eyes/moutn (sjorens)

87
Q

treatment of Sjogrens

A

symptomatic

88
Q

Schirmer test

A

how far does the water go on the filter paper

may be required to “prove the diagnosis of dry eyes” take a history instead.

89
Q

Other organs that may be involved in Sjogren’s

A

vasculitis with encephalopathy
lymphocytic pneumonitis
interstitial nephritis
pancreatic involvement with maldigestion may be missed easily.

90
Q

most feared complication of Sjogrens

A

malignant lymphoma, much more frequent in Sjogren’s pts

91
Q

Scleroderma aka

A

systemic sclerosis

92
Q

scleroderma

A

relatively common dread AI disease in which transient inflammation is followed by dense fibrosis, and the intimal layers of small blog vessel often undergo fibrous thickening

93
Q

sclerodactyly

A

in which the hands come to resemble those of a mannequin in scleroderma

94
Q

scleroderma almost always begins with

A

Raynaud’s (although most Raynaud’s its never get anything else)

95
Q

Scleroderma mechainsm

A

early the involved areas become inflamed, with fibrosis following as the inflammation moves on. Biopsy would show abundant CD4 T cells at this stage

96
Q

you can also see ____ deposits in scleroderma

A

calcium

97
Q

telangiectasias at the nail bases. many vessel are gone, those that remain are dilated tufts

A

scleroderma

98
Q

Face changes in scleroderma can include

A

telangiectasias on the face; thick skin

99
Q

lungs in scleroderma

A

plumonary fibrosis. Death in scleroderma is often due to pulmonary fibrosis/ pulmonary vascualar narrowing “pulmonary ypertension:

100
Q

gut involvemen in scleroderma

A

may include fibrosis of the esophagus (difficulty swallowing, strictures) and/or the small bowel (malabsorption)

not the hidebound crowding of the valvulae conniventes

101
Q

skin in scleroderma

A

dense dermis, flattened dermal-epidermal junction. lost adnexal structures. adenoma gone except fro arrestor pili muscles. patches of lymphocytes

102
Q

scleroderma in artery .

A

onionskining, lumen greatly narrowed (renal artery)

intimal fibrosis in finger artery (hence reynauds)

103
Q

limited scleroderma includes several entities that do not have the ________ and ________ of the diffuse dz.

A

rapid onset

generalized distribution

104
Q

CREST syndrome (anti centromere antibody disease) features

A

Calcinosis (calcium deposits in the skin)
Raynauds
Esophageal fibrosis
Sclerodactyly (and maybe involvement of the face)
Telangiectasias

105
Q

morphea

A

scleroderma-like changes in a patch
the histopathology, with an initial CD4 lymphocyte rich inflammation, then fibrosis and vessel changes, is like slceroderma

106
Q

en coup de sabre/ saber cut

A

localized linear scleroderma

107
Q

Parry Romberg

A

scar contracts over 1/2 of face, the underlying skull is likely to become deformed. mystery illness

108
Q

eosinophilic fasciitis

A

tight skin with many eosinophils in the subcutis and fascia

109
Q

why should eosinophilic fasciitis not be called a scleroderma variant

A

there is no raynauds, little involvement of the skin, little else to suggest systemic illness, and non antinuclear or specific antibodies

110
Q

Cause of eosinophilic fasciitis, and tx

A

cause - unknown

glucocorticoid treatment–> remits

111
Q

______ and _____ are a pair of dz.’s that are distinct but are usually discussed together

A

polymyositis and dermatomyositis

112
Q

similarities of polymositis and dermatomyositis

key difference?

A

clinical pictures are similar.

key difference= only dermatomyositis has the characteristic rash

113
Q

what is different between polymyositis and dermatomyositis

A

the muscle pathology and what we know of the immunopathology

114
Q

presentation of polymyositis and dermatomyositis

A

usually present as gradual (weeks to months) onset of weakness in the proximal muscles, both are clearly systemic AI dz’s

115
Q

Findings in polymyositis and dermatomyositis

A

creatine kinase and aldolase levels are likely to be very high
muscle and skin can calcify
although mm dz is usually the most prominent feature, there may be inflammatory arthritis and/or lung damage (usu interstitial fibrosis)
involvement of the upper esophagus may make swallowing a problem and sometimes the heart muscle is involved and weakened

116
Q

in polymyositis the distinctive lesion is

A

extensive infiltration of mm by Cd8 lymphocytes with obvious injury to the mm cells themselves
the lymphocytes are in endomysium around the mm cells

the vessels do not seem to be involved

117
Q

damaged mm. lymphocyte infiltrate throughout , cd8 cells

A

polymyositis

118
Q

polymyositis can cause

A

atrophy and calcification of the affected muscle

119
Q

in dermatomyositis, the lymphocytic infiltrate is

A

within or near the perimysium and around the blood vessels

the infiltrate is a mix of Cd4 T cells, B cells, and plasma cells

MAC complex is often seen in vessel walls, unlike polymositis

120
Q

most atropied muscle fibers in dermatomyositis

A

muscle fibers at the edges of the fascicles

121
Q

Grotton’s sign on the knuckles

A

marker for dermatomyositis

122
Q

mechanic’s hands

A

a poorly understood sign in polymyositis/ dermatomyositis/ anti-synthetase (Jo1) syndrome

123
Q

new onset of polymyositis/dermatomyositis in an adult, especially ______ can warn of

A

dermatomyositis

an occult cancer, usually one of the common ones

124
Q

MCTD=

A

mixed connective tissue dz

125
Q

MCTD is the best known of the overlap syndromes, combining

A

features of lupus, scleroderma, RA, and polymyositis.

anti-U1-RNP is the high titer antibody

126
Q

MCTD pathology

A

usually starts as synovits of the fingers plus Raynaud’s. Sclerodactyly may occur as in scleroderma.

skin changes range from rash to calcification

epicardial fibrosis, muscle atrophy and fibrosis

fibrosis of the gut wall,

There’s often a good response to glucocorticoids and the kidneys tend to be spared. - the only kidney lesion is vascular

127
Q

if MCTD progresses

A

(if the dz proves aggressive), death is usu due to pulmonary vascular sclerosis (pulmonary hypertension)/ pulmonary fibrosis

128
Q

IgG4-related disease is a newly recognized group of several previously mysterious _____ diseases

A

fibroinflammatory

whether this is AI disease, and best how to treat is still unanswered

129
Q

IgG4 related diseases include

A 
Riedel's fibrosing thyroiditis
AI pancreatitis
Retroperitoneal fibrosis/periaoritis
Fibrosis of the salivary and/or lacrimal glands
about a dozen others
130
Q

The characteristic features of IgG4-related diseases are

A

fibrosis with a cartwheel pattern
plasma cells making IgG4 in the lesion(s)
Increased IgG4 in the blood (about 2/3 of patients)

Pathology (15 decks)

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