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IHO Week 5 (MJ) > Immune Diseases 2 > Flashcards

Immune Diseases 2 Flashcards

1
Q

Define autoimmune diseases

A

immune mediated inflammatory diseases in which tissue and cell injury are due to immune reactions to self antigens (autoimmunity)

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2
Q

What mediates autoimmunity (3)

A

Immune complexes
autoantibodies
T lymphocytes

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3
Q

Why does autoimmunity occur?

A

Results from a loss of self tolerance

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4
Q

What is self tolerance?

A

The phenomenon of unresponsiveness to an individual’s own antigens as a result of exposure of lymphocytes to that antigen.

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5
Q

What two factors lead to auto immune disease?

A
  1. The inheritance of susceptibility genes

2. Environmental triggers

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6
Q

How do infections lead to auto immune disease? (4)

A
  • Upregulate the expression of co-stimulators on APCs - break down of anergy
  • Express same aa sequence as self antigens (molecular mimicry)
  • Polyclonal B lymphocyte activation produces autoantibodies
  • Tissue injury alters self antigens
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7
Q

How can infections protect against autoimmune disease?

A

Hygiene hypothesis

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8
Q

Autoimmune disease once initiated…

A

tend to be progressive

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9
Q

Autoimmune diseases directed at a specific organ

A

organ specific disease

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10
Q

Autoimmune diseases with widespread antigen

A

systemic/generalized disease

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11
Q

Describe SLE

A

autoimmune disease involving multiple organs

characterized by the formation of autoantibodies (ANAs)

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12
Q

Why are anti-nuclear antibodies (ANA) harmful?

A

they cause injury via deposition of immune complexes and bind to various cells and tissues

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13
Q

What is the course of SLE?

A

Typically chronic, remitting and relapsing

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14
Q

How does SLE present

A

febrile illness characterized by injury to skin, joints, kidney and seosal membranes

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15
Q

What is the hallmark of SLE?

A

production of autoantibodies

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16
Q

How does once detect ANAs?

A

immunoassay (ANA test) and indirect immunofluorescence

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17
Q

What causes the systemic lesions of SLE?

A

deposition of immune complexes, a type III hypersensitivity reaction

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18
Q

SLE autoimmune cytopenia

A

Type II hypersensitivity

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19
Q

Why is the presence of anti-phospholipid antibody a complication in SLE?

A

Patients with SLE have autoantibodies that react with phospholipids
May produce false positive syphilis test
prolong the partial thromboplastin time (anticoagulant)
Patients can get hypercoagulability resulting in vounous and arterial thrombosis

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20
Q

SLE and hypercoagulablility

A

result from autoantibodies reacting with phospholipids
May produce venous and arterial thrombosis
Results in spontaneous miscarriages and cerebral ischemia

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21
Q

cerebral ischemia and lupus

A

secondary anti-phospholipid antibody syndrome

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22
Q

Possible model/mehanism for lupus

A

susceptibility genes interfere with the maintainance of self tolerance and external triggers lead to persistence of nuclear antigens. This results in an antibody response against self nuclear antigens which is amplified by the action of nucleic acids on DCs and B cells.

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23
Q

Pathologic findings of SLE (general)

A

Acute necrotizing vasculitis

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24
Q

SLE kidney

A

Lupus nephritis - immune complex deposition in the glomeruli, tubular or peritubular capillary basement membranes, or larger blood vessels.
‘wire loop’ look

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25
Q

SLE skin

A

erythema in light exposed areas (butterfly rash)

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26
Q

SLE joints

A

nonerosive, nondeforming small joint involement

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27
Q

SLE CV system

A

fibrinous pericarditis, non bacterial verrucous endocarditis, accelerated atherosclerosis

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28
Q

SLE and spleen

A

splenomegaly

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29
Q

SLE and lungs

A

pleuritis, pleural effusion, interstital fibrosis

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30
Q

What is the mnemonic used for SLE pathology

A

SOAP BRAIN MD

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31
Q

SOAP

A

S: serositis
O: oral ulcers
A: arthritis
P: photosensitivity, pulmonary fibrosis

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32
Q

BRAIN

A 
B: Blood cells
R: Renal, Raynauds
A: ANA
I: Immunologic (anti-sm, anti-ds DNA)
N: neuropsych
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33
Q

MD

A

M: malar rash
D: discoid rash

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34
Q

Most common cause of death in lupus

A

infection due to immunosuppression

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35
Q

Chronic discoid lupus erythematosis

A

Limited to skin - chronic photosensitive dermatosis with atrophy and scarring.

Usually negative to antibodies for dsDNA

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36
Q

Subacute cutaneous lupus erythematosus

A

limited to skin - mild systemic lesions present.

antibodies to SS-A and HLA-DR3

37
Q

Drug Induced Lupus erythematous

A

procainamide and hydralazine bind to histones - become immuno geneic.

38
Q

What else can positive ANA mean?

A

connective tissue diseases
RA and MCTD
Medication induced

39
Q

Screening / diagnosis of SLE?

A

ANA

Follow up positive ANA with anti-ds and anti Sm

40
Q

Rheumatoid arthritis (RA)

A

chronic systemic inflammatory disorder attacking the joints producing a nonsupprative proliferative and inflammatory synovitis that progresses to destruction of articular cartilage and ankylosis.

41
Q

What is thought to trigger RA?

A

exposure to an arthritogenic antigen in a genetically predisposed individual that results in a breakdown of self tolerance and inflammation.

42
Q

What destroys the joints in RA?

A

The continual autoimmune reaction, with activation of CD4+ helper T cells and the releaes of inflammatory mediators and cytokines that ultimately destroy the joint.
There is also a b cell response

43
Q

Genetic markers for RA?

A

HLA-DRB1, PTPN-22

44
Q

What is used to diagnose RA?

A

presence of antibodies to cyclic citrullinated proteins (CCPs)

45
Q

Is rheumatoid factor specific for RA?

A

No, seen in 1-5% of healthy people.

46
Q

Lots of plasma cells

A

can be indicative of RA

47
Q

Sjogren syndrome

A

Chronic disease characterized by: dry eyes and dry mouth

Autoimmune destruction of lacrimal and salivary glands

48
Q

Sjogren most commonly associated with..

A

RA

49
Q

primary Sjogren syndrome

A

isolated disease

50
Q

secondary Sjogren syndrome

A

associated with another autoimmune disorder

51
Q

Patients with Sjogren syndrome have antibodies to

A

SS-A and SS-B

52
Q

There is an increased risk of patients with Sjogren syndrome to develop _______

A

Lymphoma

about 5%

53
Q

Scleroderma

A

chronic disease characterized by chronic inflammation of small blood vessels and progressive interstitial and perivascular fibrosis of the skin and multiple organs.

54
Q

Diffuse scleroderma

A

widespread skin involvement at onset, rapid progression and visceral involvement

55
Q

Limited scleroderma

A

skin involvement confined to the fingers, forearms, and face, with late visceral involvement.

56
Q

CREST

A

calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasia

57
Q

Patients with crest have __________ antibodies

A

anti-centromere

58
Q

Patients with scleroderma may have antibodies to

A

Scl-70

59
Q

Raynaud’s

A

vasospastic response to cold or emotional stress

60
Q

15-25% of patients with dermatomyositis have…

A

an underlying malignancy

61
Q

Dermatomyositis

A
  • autoimmune disease with immunologic injury and damage to small vessels and capillaries in skeletal muscle
  • Skin rash
62
Q

Polymyositis

A

Like dermatomyositis without the rash

63
Q

patients with polymyositis will have elevated (lab)

A

creatinine kinase

64
Q

Treatment for polymyositis?

A

immunosuppressive agents

65
Q

List the 6 main autoimmune diseases

A
  1. SLE
  2. RA
  3. Systemic sclerosis (scleroderma)
  4. Polymyositis
  5. Dermatomyositis
  6. Secondary Sjogren syndrome
66
Q

Define mixed connective tissue disease (MCTD)

A

The overlap of main autoimmune disease and presence of the distinctive anti U1-RNP antibody

67
Q

Primary immunodeficiency

A

congenital or genetically determined
can effect either the T or B lymphocyte functions in adaptive immunity
Manifests in infancy between 6 months and 2 years of life

68
Q

Primary immunodeficiencies are in which populations?

A

Often X linked - Agammaglobulinemia

69
Q

What are two questions you should always ask in clinic?

A

Is my patient immunosuppressed?

Has my patient traveled?

70
Q

3 ways to suspect an immunodeficiency

A
  1. Clinical history
  2. presents with an opportunistic infection from a ‘single organism’
  3. presents with repeated infections
71
Q

Laboratory test to assess B cell function (ab deficiencies)

A

immunoglobulin levels

72
Q

Assess T cell function (cellular immunity)

A

CBC, flow cytometry, Skin testing to assess delayed hypersensitvity

73
Q

Assess phagocytic function

A

CBC, peripheral smear (giant azurophilic granules in neutrophils, eosinophils, other granulocytes), genetic tests, specific tests of neutrophil function

74
Q

Assess complement

A

total serum complement (CH50)

75
Q

Secondary immunodeficiencies

A

secondary to another underlying disorder, more common than primary

76
Q

Causes of secondary immunodeficiency

A

Immunosuppressive therapy, microbial infection (HIV), Malignancy, Disorders of biochemical homeostasis, Autoimmune disease, Burn, Exposure to radiation, Asplenia, Age

77
Q

Asplenia and immunodeficiency

A

Loss of spenic macrophages post splenectomy can lead to increased risk of bacterial infection with encapsulated organisms, particularly with Streptococcus pneumoniae

78
Q

Bruton’s agammaglobulinemia defect

A

Failure of pre b cells to become mature
mutation in Btk
X linked

79
Q

Bruton’s agammaglubulinemia clinical presentation

A

SP infections
Maternal ab protect at birth, presents at 6 months
Decreased immunoglobulin levels

80
Q

IgA deficiency defect

A

Failure of IgA to mature into plasma cells

81
Q

IgA deficiency presentation

A

SP infections; giardiasis

Anaphylaxis if exposed to blood products that contain IgA

82
Q

Common variable immunodeficiency presentation

A

Defect in B cell maturation to plasma cells
Adult immunodeficiency disorder
Sinopulmonary infections
GI infections, pneumonia, autoimmune diseases, malig.
Decreased immunoglobulins

83
Q

DiGeorge Syndrome

A

Thymus fails to develop from 3rd and 4th pouch

84
Q

DiGeorge Presentaiton

A

Hypoparathyroidism; absent thymus shadow
GVH reaction
T lymphocyte disorder

85
Q

SCID

A

Adenosine deaminase deficiency

Autosomal recessive

86
Q

Wiskott Aldrich

A

progressive deletion of B and T cells

X linked

87
Q

Triad of Wiskott Aldrich

A

eczema, thrombocytopenia, SP infections

Associated with malignant lymphoma

88
Q

Ataxia Telangiectasia

A

Mutation in DNA repair enzymes
Thymic hypoplasia
Autosomal recessive disorder

89
Q

Ataxia Telangiectasia presentation

A

Cerebellar ataxia, telangiectasia of eyes and skin
increased risk of lymphoma and leukemia/adenocarcinoma
Increaesd serum a-fetoprotein
Decreased IgA, IgE and IgM
Decreased T cell function

IHO Week 5 (MJ) (4 decks)

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