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IHO week 5 > Autoimmune disease and Immnunodeficiency Syndromes > Flashcards

Autoimmune disease and Immnunodeficiency Syndromes Flashcards

1
Q

Key underlying immune defects of autoimmune disease?

A

Immune reaction to self antigen mediated by:

autoantibodies

immune complexes

T-cells

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2
Q

Two key factors leading to autoimmune disease?

A
  1. inheritance of susceptibility genes

2. environmental triggers

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3
Q

Typical clinical course of untreated autoimmune disease:

A

Progressive, inexorable tissue damage

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4
Q

Self tolerance means?

A

Unresponsiveness to an individual’s own antigens.

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5
Q

Gene defects most commonly associated with autoimmunity?

A

HLA

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6
Q

Multiple organs

ANA positive

Immune complexes

A

SLE

Systemic Lupus Erythematosis

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7
Q

Two ways to detect ANA in SLE?

A
  1. immunoassay

2. indirect immunofluorescence

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8
Q

Specific antibodies for:

dsDNA

Smith (Sm) antigen

A

SLE

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9
Q

Anti-phospholipid antibodies = ?

A

SLE

**think hypercoagulation –> thrombosis/ischemia

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10
Q

How does the environment play into SLE triggering?

A

Cell apoptosis (sunburn, estrogen, meds, etc.) –> increased nuclear antigen floating around for ANA’s to bind and cause reaction

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11
Q

Main type of hypersensitivity associated with SLE?

A

Type III

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12
Q

Pathology finding for SLE:

A

Acute necrotizing vasculitis –> can affect virtually any organ

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13
Q

SOAP BRAIN MD stands for:

A

Serositis

Oral ulcers

Arthritis

Photosensitivity, Pulmonary fibrosis

Blood cells

Renal, Reynaud’s

ANA

Immunologic (anti-Sm, anti-dsDNA)

Neuropsych

Malar rash (classic BUTTERFLY rash)

Discoid rash

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14
Q

Immune complex deposits on glomerulus of SLE look how? In contrast to what?

A

Lumpy

vs smooth in Goodpasture’s where Ig’s are specific for kidney?

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15
Q

LE limited to skin?

A

Chronic Discoid LE

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16
Q

Predominantly skin related LE with some systemic involvement

SS-A and HLA-DR3 genotype

A

Subacute Cutaneous LE

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17
Q

Mainly attacks joints

Destroys cartilage

ankylosis

A

Rheumatoid Arthritis

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18
Q

HLA-DRB1, PTPN22 genotype?

A

RA

19
Q

Autoantibodies for CCP?

A

RA

20
Q

dry eyes

dry mouth

middle age women

lymphocytic infiltration involving salivary and parotid glands

A

Sjogren Syndrome

21
Q

Autoantibodies to ribonucleoproteins SS-A and SS-B:

A

Sjogren Syndrome

22
Q

MALT lymphoma associated with?

A

Sjogren Syndrome

23
Q

Progressive interstitial FIBROSIS of the skin and multiple organs:

A

Systemic sclerosis

24
Q

Antibodies to Scl-70 (DNA Topo-1):

A

Systemic sclerosis

25
Q

Anti-centromere antibodies:

**on the test?

A

CREST syndrome

Calcinosis
Raynaud's
Esophageal dysmotility
Sclerodactyly
Telangiectasia
26
Q

Skeletal muscle + skin involvement:

A

Dermatomyositis

27
Q

ALWAYS screen pts with dermatomyositis for:

A

Underlying malignancy

28
Q

Muscle and systemic involvement LACKING skin involvement?

A

Polymyositis

29
Q

Antibodies for Jo1?

A

Polymyositis

30
Q

What is Jo1?

A

histidyl t-RNA synthetase

31
Q

anti-U1-RNP antibody:

A

Mixed Connective Tissue Disease (MCTD)

32
Q

Failure of B-cell precursor development

X-linked (mostly see in males)

Btk

A

X-linked Agammaglobinemia

aka

Bruton’s

33
Q

Significant blood smear finding of XLA?

A

No B-cells

34
Q

Sx onset in XLA?

A

6 months (when mom’s Abs wear off)

sinopulmonary bacterial infx

35
Q

Failure of B-cells to differentiate into plasma cells

hyperplastic germinal centers in lymph nodes

low Ig production

A

Common Variable Immunodeficiency

36
Q

Failure of B-cells to differentiate into IgA plasma cells

A

Isolated IgA deficiency

37
Q

failure of development of 3rd and 4th pharyngeal puches

22q11 deletion

no T-cells

A

DiGeorge Syndrome

Thymic hypoplasia–>no thymus = no T-cell maturation

38
Q

Defect in Ig class switching

X-linked mutation of CD40

risk of Pneumocystis jiroveci

A

Hyper IgM syndrome

39
Q

Defects in both humoral and cell-mediated immunity

rapidly fatal if no stem cell transplant

autosomal recessive deficiency of ADA

A

SCID

40
Q

X-linked recessive

WASP deficiency

depleted T-cells

low IgM

A

Wiskott-Aldrich Syndrome

41
Q

Inability to eliminate EBV

severe Infectious Mono

B-cell lymphoma

SLAM protein

A

X-linked Lymphoproliferative syndrome

42
Q

CHS1/LYST

defective phagocyte function

giant cytoplasmic granules in lymphocytes

autosomal recessive

A

Chediak-Higashi Syndrome

43
Q

Deficiency in MAC (complement system) increases risk for?

A

Neisseria infx

44
Q

Loss of what organ is big risk for secondary immunodeficiency?

A

Spleen

  • *particularly encapsulated organisms
  • Strep pneumo
  • Hib
  • N. meningitidis

IHO week 5 (8 decks)

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