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immune Disease Flashcards

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1
Q

Muscle biopsy of Polymyiositis will show

A

Endomysial inflammation with CD8+ T cells

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2
Q

Polymyositis/dermatomyositis positives Antibodies

A

⊕ANA, ⊕anti-Jo-1, ⊕anti-SRP, ⊕anti-Mi-2 .

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3
Q

Pt with Gottron papules and “mechanic’s hands”

A

dermatomyositis

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4
Q

heliotrope rash , “shawl and face” rash are for this myopathy

A

dermatomyositis

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5
Q

Gottron papules

A

Small purple or red flat papules on extensor surfaces, particularly the elbows and joints of the hand (Gottron papules).

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6
Q

Gottron papules biopsy will show

A

acanthosis, hyperkeratosis, focal vacuolar alteration of the basal cell layer, and perivascular inflammatory infiltrat

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7
Q

Bilious vomiting in an infant should be considered diagnostic of _______ until proven otherwise.

A

Midgut malrotation w/ volvulus until proven otherwise

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8
Q

keratoderma blennorrhagica, a papulosquamous skin rash on the palms and soles, is seen in which dz

A

Reactive Arthritis

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9
Q

Thymomas occur in approximately __% of patients with MG.

A

10%

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10
Q

This paraneoplasic syndrome is associated with small cell lung cancer.

A

Lambert-Eaton myasthenic syndrome (LEMS).

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11
Q

Pathogenesis of Lambert-Eaton myasthenic syndrome (LEMS).

A

Pre-synaptic Voltage-gated calcium channel antibodies

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12
Q

Myasthenia gravis epidemiology according sex

A

Women teens-20s, Men after age 60

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13
Q

Antigliadin antibodies are found in patients with:

A

celiac disease

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14
Q

Bullous pemphigoid (BP) is associated with a ? Nikolsky sign

A

Negative. the blisters do not exfoliate when rubbed.

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15
Q

BP is caused by an autoimmune attack against

A

hemidesmosomes,

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16
Q

Pemphigus bulgaris is caused by an autoimmune attack against

A

Desmosomes

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17
Q

Pemphigus vulgaris, which has a ? Nikolsky sign

A

Positive

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18
Q

Dermatomyositis criteria (5)

A

Symmetric proximal muscle weakness

Characteristic heliotrope rash (shown in the image)

Elevated serum muscle enzymes

Myopathic changes on electromyography

Muscle biopsy abnormalities with the absence of histopathologic signs of other myopathies.

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19
Q

Anti-desmoglein (anti-desmosomes) seen in

A

Pemphigus vulgaris

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20
Q

Criteria for Churg Strausss (4/6)

A
  1. asthma
  2. eosinophilia (complete blood count with differential eosinophilia >10%)
  3. paranasal sinusitis
  4. pulmonary infiltrates
  5. histologic proof of vasculitis with extravascular eosinophils
  6. mononeuritis multiplex or polyneuropathy.
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21
Q

anti-neutrophil cytoplasmic antibodies (ANCA) are for:

A

allergic (eosinophilic) granulomatosis and angiitis which is a small-vessel vasculitis
Churg Strauss.

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22
Q

IL-12 deficiency: cannot trigger differentiation T cells to:

A

Th1 cells

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23
Q

If Th1 is defficient, Th1 will not produce

A

IFN gamma

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24
Q

In IL-12 deficiency, pts have ↑ susceptibility to disseminated …

A

mycobacterial and Salmonella infections

also disseminated BCG after vaccination

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25
Q

Treatment of IL-12 deficiency is

A

IFN gamma

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26
Q

Terminal deficiency of complement (c5-c9) put you at risk for what type of infections?

A

Prone to Neisseria infections.

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27
Q

Early complement deficiency put you at risk for what type of infections?

A

Pyogenic infections. Increased risk of SLE

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28
Q

> 80% of patients with MPGN II are positive for:

A

Serum C3 nephritic factor (C3NeF), an autoantibody directed against C3bBb, the convertase of the alternative pathway of complement

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29
Q

Hepatitis B antibody complexes are involved frequently in which Vasculitis?

A

PAN

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30
Q

in RA 70% of pts are positive for this haplotype

A

HLA-DR4 haplotype

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31
Q

X-linked agammaglobulinemia (Bruton’s disease) is caused by a mutation in what gene?

A

Bruton Tyrosine Kinase gene

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32
Q

Pathogenisis of (Bruton’s disease)

A

B cells fail to mature. Males with XLA have a total or almost total absence of B lymphocytes and plasma cells.

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33
Q

The genes for the HLAs are located in the MHC

on chromosome ___

A

6

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34
Q

What cells mediate most graft rejections?

A

CD81 cytotoxic T cells and macrophages (activated by CD41 T cells)

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35
Q

When proto-oncogenes become altered or damaged, they are termed:

A

oncogenes

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36
Q

Name the tissue resident form of macrophages in

  1. Liver
  2. Dermis
  3. Lung
  4. Brain
  5. Bone
A 
Kupffer cells (liver), histiocytes (dermis), alveolar
macrophage (lung), microglia (brain), and osteoclasts (bone).
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37
Q

Molecular defect in Omenn Syndrome

A

Missense mutations in RAG genes. The RAG enzymes only have partial activity

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38
Q

Molecular defect in Severe Combined Immunodeficiency

A

No RAG enzyme activity. Total lack of B and T cells

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39
Q

Wiskott-Aldrich syndrome triad:

A
  1. thrombocytopenic purpura
  2. multiple infections
  3. eczema
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40
Q

How will you expect immunoglobulins in Wiskott-Aldrich syndrome

A

Decreased IgM, increased IgE/IgA

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41
Q

Wiskott-Aldrich syndrome is a B and T cell immunodeficiency, which is caused by a defect in what gene ?

A

WASP gene in X chromosome

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42
Q

Delayed separation of the umbilical cord (> 4 weeks) is a classic finding, as are recurrent bacterial infections and the absence of pus formation

A

LAD

Leukocyte adhesion deficiency

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43
Q

How do you expect the nitroblue tetrazolium (NBT) test in chronic granulomatous disease?

A

Negative

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44
Q

What would you see in a peripheral blood smear of a patient with leukocyte adhesion deficiency type 1 (LAD-1)?

A

Neutrophilia is observed due to the inability of neutrophils to exit the bloodstream.

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45
Q

What is observed in neutrophils in Chediak-Higashi syndrome?

A

Giant granules

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46
Q

Patients with tuberculoid leprosy have granulomas that have elevated amounts of :

A

IL-2, IFN-γ, and

TNF-β.

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47
Q

CD25-positive TReg cells have been shown to have

a role in maintenance of

A

Self tolerance. They secrete IL-10 which is anti infammatory. Defects in TReg are associated with autoimmune disease

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48
Q

Describe Direct Coombs test

A

Adding Coombs Serum (antihuman gammaglobulin) to the baby’s RBCs will cause aglutination. If the child is developing hemolytic disease of the NB, then his RBCs will be coated with maternal anti Rh antibodies.

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49
Q

Lab shows Normal levels of pro B cells but decreased levels of all classes of immunoglobulins. Dx

A

Bruton´s agammaglobulinemia

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50
Q

Absent tonsils think in ____

A

Bruton´s agammaglobulinemia

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51
Q

You need to give washed RBCs transfusion to pts with which type of Immunodeficiency?

A

Selective IgA Deficiency

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52
Q

Anaphylaxis after tranfusion, increased susceptibility to Giardiasis, think in _______

A

Selective IgA Deficiency

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53
Q

This immunodeficiency can present in the 2nd/3rd decade of life and

A

CVID

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54
Q

This pathology arises from the failure of the 3rd and 4rd pharyngeal pouches to develop

A

DiGeorge syndrome

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55
Q

Mode of Inheritance of Job Syndrome

A

AD (hyper IgE)

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56
Q

Mutation of Job Syndrome is in which gene?

A

STAT3

57
Q

Chronic Mucocutaneous Candidiasis is tipically a manifestation of dysruption in IL- _____

A

IL-17 or IL-17 receptor

58
Q

Severe Combined immunodeficiency findings (LAB/ CXR)

A

Absence of thymic shadow, and T cells on flow cytometry

59
Q

Child presents w/ FTT, chronic diarrhea, thrush and recurrent infections, think in

A

Severe Combined immunodeficiency

60
Q

Mention the 3 possible defects causing Severe Combined immunodeficiency

A

R A M

  1. MOST common is a mutation of the common γ chain of IL-2 Receptor (X chromosome)
  2. ADA (adenosine Deaminase) deficiency
  3. MHC II
61
Q

Severe Combined immunodeficiency mode of inheritance

A

X linked 50% of pts are male

other is AR

62
Q

Triad of ataxia telengactasia

A

IgA deficiency

63
Q

This condition is characterized for a defect in the CD40 ligand on Th cells:

A

Hyper IgM syndrome (no class switching)

64
Q

Severe pyogenic infections, Cryptosporidium, pneumocystis early in life and Increased level of IgM, dx is

A

Hyper IgM syndrome

65
Q

Defect in LFA-1 integrin protein also known as CD18 (normally on phagocytes) gives rise to which immunodeficiency

A

LAD

66
Q

Absent pus formation in …

A

LAD

67
Q

Chediak- Higashi syndrome inheritance is

A

AR `

68
Q

Name catalase positive organisms

CGD pts are prone to this infections

A 
Staphylococci. aureus 
Pseudomonas aeroginosa.
Aspergillus fumigatus.
Candida albicans.
Enterobacteriaceae (Klebsiella, Serratia)
69
Q

nitroblue tetrazolium test is negative in CGD, why?

A

bc the absence of superoxide prevents reduction of the dye. Fails to turn Blue
Nl people turn Blue

70
Q

What is the most modern test to dx CGD?

A

Abnl Dihydrorhodamine, neg flow cytometry leads to decreased green fluorescence

71
Q

This SLE antibodies are specific but not prognostic

A

anti-Smith

72
Q

Anitbodies that are specific for SLE and indicate a poor prognosis:

A

anti-dsDNA

73
Q

This antibodies are considered sensitive but not specific for SLE;

A

antinuclear antibodies,

74
Q

antiphospholipid antibodies are present in close to half of patients with_____

A

SLE

75
Q

What ca give you a false-positive result in the VDRL test?

A

antiphospholipid autoantibodies that are seen in roughly 40% of SLE patients can react to the cardiolipin antigen used in syphilis serology, thus giving a false positive test

76
Q

Wiskott-Aldrich syndrome. Immunoglobulins and platelet level expected?

A

low to normal IgG, IgM.

increased IgE, IgA.

Fewer and smaller platelets.

77
Q

IF Immuno Fluorescence findings in Acute poststreptococcal glomerulonephritis

A

(“starry sky”) granular appearance (“lumpy-bumpy”) due to IgG, IgM, and C3 deposition along GBM and mesangium

78
Q

Finding of Rapidly progressive (crescentic) glomerulonephritis in IF (immunofluorescence)

A

Linear IF due to antibodies to GBM and alveolar basement membrane: Goodpasture syndrome—hematuria/hemoptysis
Granular IF—PSGN or DPGN

79
Q

Finding of Rapidly progressive (crescentic) glomerulonephritis in LM

A

LM—crescent moon shape . Crescents consist of fibrin and plasma proteins (eg, C3b) with glomerular parietal cells, monocytes, macrophage

80
Q

Diffuse proliferative glomerulonephritis often seen in

A

SLE

81
Q

LM findings in DPGN

A

LM—“wire looping” of capillaries

82
Q

EM—“Basket-weave” think in

A

Alport syndrome “can’t see, can’t pee, can’t hear a bee”

83
Q

Treatment of ITP can include:

A

steroids, IVIG, and in refractory cases, splenectomy.

84
Q

Pappenheimer bodies are seen in???

A

sideroblastic anemia

85
Q

rare granulomatous vasculitis affecting small vessels associated w/ p-ANCA

A

Churg-Strauss

86
Q

c-ANCA is associated with

A

Granulomatosis with polyangiitis (previously known as Wegener granulomatosis).

87
Q

Is the cause of more than half the deaths that occur in patients with Churg-Strauss syndrom

A

Cardiomyopathy

88
Q

Sinusitis/nasopharyngeal ulceration, hemoptysis, and hematuria; a petechial rash is not characteristic of this condition.

A

granulomatosis with polyangiitis (formerly called Wegener granulomatosis)

89
Q

Is a medium-vessel vasculitis that is not pANCA positive. It typically involves the kidneys and other visceral organs, but spares the lungs. It is associated with abdominal pain, foot drop, fever, muscle and joint aches, and decreased appetite.

A

Polyarteritis nodosa

90
Q

Goodpasture sx is a type II hypersensitivity reaction in which autoantibodies cross-react with the α-3 chain of ___ in both the pulmonary alveolar and glomerular b.m.

A

type IV collagen

91
Q

This graft rejection is mediated by the recipient’s cytotoxic T lymphocytes destroy the graft within weeks to months of the transplant.

A

Acute

92
Q

GVHD can occur after _____/______transplantation

A

bone marrow or liver

93
Q

______is due to grafted donor immunocompetent T-cells that reject host cells.

A

GVHD

94
Q

GVHD is which type of HS ??

A

Type IV

95
Q

This disease is the most common form of systemic vasculitis in children. Most patients make a complete recovery.

A

Henoch-Schönlein purpura

96
Q

Spike and dome” on electron microscopy is associated with

A

membranous glomerulonephritis

97
Q

Foot process effacement on electron microscopy is associated with

A

minimal change disease.

98
Q

Linear immunofluorescence on electron microscopy is consistent with

A

Goodpasture syndrome.

99
Q

Classic triad of Henoch-Schönlein purpura

A

Skin: palpable purpura on buttocks/legs
Arthralgias
GI: abdominal pain (associated w- intussusception)

100
Q

Guillain-Barré syndrome (GBS) is also known as

A

acute inflammatory demyelinating polyradiculoneuropathy (AIDP).

101
Q

in Guillain-Barré syndrome Antibodies against C. jejuni inappropriately target the ???? of peripheral nerves

A

endoneurium

102
Q

Autoimmune hemolytic anemia can be caused by which 2 drugs

A

Penicillin and Methyldopa

103
Q

This infection induces antibodies vs RBCs and can induce Autoimmune hemolytic

A

Mycoplasma pneumonia

104
Q

Rheumatic fever is HS type:

A

II (strep antibodies cross react with cardiac myocytes)

105
Q

Instances where you can see Serum sickness nowadays (rare)

A

When people are passively immunized with Rabies or Tetanus’antitoxin
Monoclonal antibodies Rituximab-infliximab

106
Q

PAN in HS type….

A

Type III HS

107
Q

Multiple sclerosis is type HS

A

type IV

108
Q

SLE and RA are very common 20-30% in what immunodeficieny?

A

selective IgA def

109
Q

which immunodef gives you a false positive beta hcg test?

A

selective IgA def up to 30% pts with def IgA will test ¿ due to heterophile antibodies

110
Q

This immunodef is often sporadic-no family history

A

SCID

111
Q

Pathology is SCID is

A

defective B cell maturation, no plasma cells no antibodies. Normal B cell count but no antibodies

112
Q

Defective CD4 + Th17 cells that fail to produce IL-17

Loss of attraction of neutrophils

A

Jobs Syndrome Hyper IgE, loss of INF gamma

113
Q

CV eczema in first days of life, staph abscesses that are cold, recurrent sinusitis and otitis and facial deformities (retained 1ry teeth) seen in which immunodef

A

obs Syndrome Hyper IgE,

114
Q

Chronic Mucocutaneous Candidiasis is associated with defects in which genes

A

AIRE genes (Dectin-1)

115
Q

primary T cell problem, fail to react w/ candida albicans, also associated w/ which pathologies?

A

Chronic Mucocutaneous Candidiasis with hypo parathyroidism and adrenal insufficiency

116
Q

newborn screening for SCID is called

A

TRECS (t cell recombinant excision circles)

117
Q

Ataxia telangiectasia gene defective is ___ on chromosome

A

ATM gene on ch 11 — failure to repair DNA mutations Non-homologous end joining (NHEJ) is a pathway that repairs double-strand breaks in DNA

118
Q

usually pts w Ataxia telangiectasia die from

A

Malignancy

119
Q

WAS protein is necessary for T cell ___________

A

cytoskeleton maintenance

120
Q

Mutation in Chediak Higashi Syndrome:

A

LYST gene Lysosomal Trafficking Regulator -causes microtuble dysfunction

121
Q

systemic juvenile idiopathic arthritis occurs in children younger than____
When the disease occurs in pts older than 16 yo is known as ____

A

16 years old

Still disease

122
Q

in sJIA the fever must be present for

A

more than 2 weeks

123
Q

in sJIA the arthritis must be present for more than

A

6 weeks

124
Q

In warm reactive autoimmune hemolytic anemia, which antibodies attach to RBCs with maximal reactivity at 37 C???

A

IgG

125
Q

What is the role of corticosteroids in warm reactive autoimmune hemolytic anemia

A

Corticosteroids inhibit the Fc receptor-mediated clearance of sensitized erythrocytes. Steroids may also diminish the production of the autoantibodies

126
Q

What is Rho(D) immune globulin IV (RhoGAM)?

A

An antibody to the Rh antigen, it is given to the mother and will help destroy Rh-positive cells (from the first newborn) that the mother may become exposed to, so it prevents the mom to mount an immune response against future fetos.

127
Q

In hemolytic disease, which antibodies can cross the placenta *2nd preganncy) and enter the fetal circulation, where they cause hemolysis??

A

anti-Rh IgG antibodies

128
Q

The best immediate treatment for an infant with hemolytic disease of the newborn is

A

to exchange the Rh-positive blood with Rh-negative blood.

129
Q

in IgA nephropathy (also called Berger disease)a renal biopsy specimen will show:

A

Mesangial deposits of IgA

130
Q

This disorder is the most common cause of primary glomerulonephritis.

A

IgA nephropathy (also called Berger disease)

131
Q

Classic presentation of Rheumatoid arthritis

A
  • joint pain & morning stiffness that lasts > 30 min
  • improves with activity
  • symmetric presentation
  • typically involves the metacarpophalangeal joints
132
Q

Classic finding in this dz is eosinophils in urine

A

Acute interstitial nephritis

133
Q

Acute interstitial nephritis biopsy is

A

usually nl

134
Q

Acute interstitial nephritis is an allergic reaction to

A

Drugs

135
Q

The classic pentad of thrombotic thrombocytopenic purpura (TTP) is

A
  1. microangiopathic hemolytic anemia (MAHA),
  2. thrombocytopenia
  3. neurologic
  4. renal abnormalities
  5. fever,
136
Q

microangiopathic hemolytic anem ia:

A

schistocytes, elevated LDH, and indirect hyperbilirubinemia

137
Q

Child w/ type 1 DM. Screen for:

A 
Autoimmune thyroiditis and Celiac disease. 
Autoimmune thyroiditis (HLA-DR3) and celiac disease (HLA-DR3 and HLA-DR4).
138
Q

Anticentromere antibodies are associated with the CREST variant of scleroderma

A

CREST variant of scleroderma

139
Q

Anti-Scl-70 (anti-DNA topoisomerase I)

A

Scleroderma

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