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PB+T > Autoimmune Diseases > Flashcards

Autoimmune Diseases Flashcards

0
Q

Central versus peripheral tolerance

A

Central is based on negative selection, peripheral is based on clonal anergy, but both are designed to remove self antigen T cells from the body.

  • central: AIRE
  • peripheral: lack of costimulatory signals CD80/CD86
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1
Q

AIRE

A

Autoimmune regulator gene: mutation -> autoimmune polyendocriopathy

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2
Q

IPEX

A

Mutated FOXp3

- FOXp3 induced by CD25 (basilixumab) txn factor

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3
Q

Sequestered Abs

A

In immunopreviliged sites, may be expressed during trauma and manifest autoimmune diseases

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4
Q

Susceptibility genes: 4 with assoc conditions

A

HLA sites
PTPN-22: RA T1DM
NOD-1: crohns
IL-2/7: MS

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5
Q

CCL19/21

A

Lymphocyte endothelial migration signal that binds CCR7

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6
Q

TH1,2 and 17: signaling; cytokines

A

Macs->IL-12->TH1-> IFNg->macs (intracellular bacteria)
TH2->IL4/5/6/13->Eosinophils
TH17->IL17->macs/neutrophils (extracellular killing)

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7
Q

SLE: susceptibility

A

Asian/Black/Hispanic

  • 30-40
  • will always occur with cytopenias and lymphadenopathy
  • 4-11 criteria for SLE diagnosis
  • anti-nuclear Abs non specific, but highly sensitive
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8
Q

Rewires Tcells in SLE

A

CD3g -> FcRg

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9
Q

Antiphospholipid Antibody syndrome

A

Often accompanies SLE, and give false positive for syphilis

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10
Q

SLE classes

A

I - III: mild clinically, renal glomeruli not involved in disease process
IV: involves renal glomeruli, hyper cellular
V: mysangial/GBM thickening

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11
Q

Cardio SLE manifestations

A
  • Arrythmias/heart blocks

- Vegetations on both sides of valve leaflets (lineman sacks lesions)

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12
Q

Chronic discord lupus

A

Same as Normal SLE except no anti-nuclear antibodies

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13
Q

Sjorgrens syndrome

A
  • Dry eyes and mouth (Autoimmune destruction of the salivary and lacrimal glands)
  • women 40-70
  • May involve extra glandular fibrosis, Peripheral neuropathy/synovitis or Tubulointerstitial disease of the kidneys
  • LIP BIOPSY
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14
Q

Cellular mechanism of SLE et al.

A

Tolerance fails and T cells with self antigen recognition Get into circulation. On Destruction of cells self antigens are released And anti-self antibodies are then processed and made Precipitating the autoimmune cascade.

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15
Q

SSA/SSB

A

Their presence supports diagnosis of Sjorgrens syndrome,

- SSA assoc. with longer duration

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16
Q

Scleroderma

A

Progressive perivascular fibrosis

  • Limited involves skin of fingers hands, forearms and face; Late visceral involvement
  • diffuse: Widespread skin involvement; Early Visceral involvement. Anti-SCL 70 associated with this form.
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17
Q

Crest syndrome

A

Calcinosis, Raynaud’s, esophageal dysmotility, Sclerodactyly, talengectasia

  • similar to SLE -> deposits in BVs
  • Nodules often seen on extremities: Dystrophic calcification
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18
Q

Anti-SCL 70 antibodies

A

Associated with diffuse scleroderma

- Anti-DNA topoisomerase 1

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19
Q

Mixed connective tissue disease

A

Not clinically valuable diagnosis but may progress to SLE

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20
Q

Direct versus indirect transplant rejection

A

Direct: CD4/CD8 initiate direct cellular attack
Indirect: CD4’s initiate cell and antibody mediated injury

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21
Q

Hyperacute graft Rejection

A

Resultant from preformed antibodies

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22
Q

Acute graft rejection

A

Happens over 1 to 3 months
CD8 and CD4: Cell mediated injury
CD4: Humoral mediated injury

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23
Q

Chronic graft rejection

A

Most common and most clinically difficult to manage, Many drugs meant to avoid acute graft rejection enable chronic graft rejection
- Injury mediated by fibrosis causing scarring and ischemia

24
Q

Clinical suspicion of autoimmune condition

A

Repeated, opportunistic, not easily cleared, age of onset (eg 3 mo = something Ig related) X-linked in males

25
Q

Complement deficiency

A

C-5 through C-9: Increased susceptibility to Neisseria species
Meningococcal sepsis: Purpera with defused ecchymosis

26
Q

Chronic granulomatous disease

A

Decreased oxidative burst.
Resultant caseous necrosis of the liver often causes mortality.
Neutrophils and bugs with the ring of macrophages surrounding trying to wall Infection off in a fibrous capsule.

27
Q

Nitroblue tetrazolium test

A

Colorless dye is added to cellular sample, Oxidized dye turns blue. Thus partial oxidation reveals heterozygous mutant for chronic granulomatous disease, Lack of oxidation reveals homozygous mutant.

28
Q

Most common CGD mutations

A

gp91phox: 75% < Poorer prognosis
gp47phox: 25%

29
Q

XLA

A

Deficient Bruton’s tyrosine kinase. BT K precipitates the progression from pro to pre-B cell.
- Decreased IgG,IgM and A
- Susceptible to encapsulated bacteria and extracellular bugs
: Staph aureus, strep pneumo, H. influenzae, Echo, polio, Cox, Giardia
- Never give live attenuated virus Vaccine

30
Q

DiGeorges syndrome

A

Catch-22: 22q11.2 Microdeletion
Results in failure of migration of the third and fourth pharyngeal arches, Resulting in abnormal facies and thymic aplasia as well as parathyroid agenesis.

31
Q

SCID

A

Gamma chain most common, (50 to 60%) ADA deficiency (40%)

Survival requires BMT

32
Q

Secondary immunodeficiencies

A 
Malnutrition: # 1
Burn: #2
Diabetes Milletus
Uremia
Glucocorticoids
Pregnancy
AIDS
33
Q

Amyloid

A

Excessive deposition of miss folded proteins (Cross beta-pleated fibrils)
Cells accumulate miss folded proteins and die

34
Q

Amyloidosis diagnosis

A

Congo red stain appears Applegreen Under polarized light In the presence of ammo

35
Q

Familial amyloidosis

A

Transthyretin: mutated protein

Never threaten the family

36
Q

Hemodynamically associated amyloidosis

A

AB2 Microglobulin: Chronic renal failure

Globulin - blood

37
Q

Alzheimer’s disease associated amyloidosis

A

AB Amyloid precursor protein: Sporadic or familial

38
Q

Immune dyscrasias

A

AL Ig light chains: Monoclonal B cell proliferation

Immune: bence jones protein, light chain dimers

39
Q

Reactive systemic or secondary amyloidosis

A

Alpha amyloid Serum amyloid protein: Chronic inflammation

Do an AASA to determine systemic or secondary

40
Q

Features of systemic amyloidosis

A

Amyloid deposits in various Visceral tissues

  • microglossia: Amyloid deposits in tongue
  • Carpel tunnel: Amyloid deposits in joints
41
Q

Lip biopsy

A

Gold standard for diagnosing Sjorgrens

42
Q

CD80/CD86

A

Costimulatory signals resulting in positive selection

43
Q

Echo, cox, giardia, polio

A

Extracellular viruses That individuals with XLA are susceptible to

44
Q

gp91Phox/gp47Phox

A

Most common CGD mutations

  • 91: 75% ~ poor prognosis
  • 47: 25%
45
Q

XL - Hyper IgM: etiology, labs

A 
Increased or normal IgM with all other Igs decreased
- cd40 ligand deficiency (no class switching)
46
Q

Type I HSN: BEEM

A

IL4: B cell stimulation
IL5: eosinophil recruitment
IL13: IgE class switching/mucus production

47
Q

Anticentromere antibodies

A

Specific to limited type scleroderma

48
Q

Anti SM, antihistone antibodies

A

Specific for SLE

49
Q

Scl 70 antibodies

A

Specific marker for Diffuse scleroderma

50
Q

AIRE/CTLA-4

A

Central vs. peripheral tolerance mechanisms

51
Q

Leukotrienes C/D

A

SpasmoDiC

- vasoconstriction, bronchospasm

52
Q

Perivascular cuffing

A

Associated with the type 4 hypersensitivity

53
Q

CD 25

A

Treg ligand

54
Q

Type IV vs. type V SLE glomerulonephritis

A

Type IV is a capsular hypercellular state

Type V is a capsular hyperproliferation of membranes

55
Q

HLA-DR3

A

is associated with susceptibility for SLE subcutaneous

56
Q

Normal - increased IgM, decreased IgG,A and E without lymphocytopenia

A

CVID

57
Q

Normal IgG/M; decreased IgE/A

A

Wieskott-Aldrietch syndrome

  • defective WASP protein - migration and transduction
  • BMT is only treatment

PB+T (20 decks)

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