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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
SLE skin
erythema in light exposed areas (butterfly rash)
26
SLE joints
nonerosive, nondeforming small joint involement
27
SLE CV system
fibrinous pericarditis, non bacterial verrucous endocarditis, accelerated atherosclerosis
28
SLE and spleen
splenomegaly
29
SLE and lungs
pleuritis, pleural effusion, interstital fibrosis
30
What is the mnemonic used for SLE pathology
SOAP BRAIN MD
31
SOAP
S: serositis O: oral ulcers A: arthritis P: photosensitivity, pulmonary fibrosis
32
BRAIN
``` B: Blood cells R: Renal, Raynauds A: ANA I: Immunologic (anti-sm, anti-ds DNA) N: neuropsych ```
33
MD
M: malar rash D: discoid rash
34
Most common cause of death in lupus
infection due to immunosuppression
35
Chronic discoid lupus erythematosis
Limited to skin - chronic photosensitive dermatosis with atrophy and scarring. Usually negative to antibodies for dsDNA
36
Subacute cutaneous lupus erythematosus
limited to skin - mild systemic lesions present. antibodies to SS-A and HLA-DR3
37
Drug Induced Lupus erythematous
procainamide and hydralazine bind to histones - become immuno geneic.
38
What else can positive ANA mean?
connective tissue diseases RA and MCTD Medication induced
39
Screening / diagnosis of SLE?
ANA | Follow up positive ANA with anti-ds and anti Sm
40
Rheumatoid arthritis (RA)
chronic systemic inflammatory disorder attacking the joints producing a nonsupprative proliferative and inflammatory synovitis that progresses to destruction of articular cartilage and ankylosis.
41
What is thought to trigger RA?
exposure to an arthritogenic antigen in a genetically predisposed individual that results in a breakdown of self tolerance and inflammation.
42
What destroys the joints in RA?
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
Genetic markers for RA?
HLA-DRB1, PTPN-22
44
What is used to diagnose RA?
presence of antibodies to cyclic citrullinated proteins (CCPs)
45
Is rheumatoid factor specific for RA?
No, seen in 1-5% of healthy people.
46
Lots of plasma cells
can be indicative of RA
47
Sjogren syndrome
Chronic disease characterized by: dry eyes and dry mouth | Autoimmune destruction of lacrimal and salivary glands
48
Sjogren most commonly associated with..
RA
49
primary Sjogren syndrome
isolated disease
50
secondary Sjogren syndrome
associated with another autoimmune disorder
51
Patients with Sjogren syndrome have antibodies to
SS-A and SS-B
52
There is an increased risk of patients with Sjogren syndrome to develop _______
Lymphoma | about 5%
53
Scleroderma
chronic disease characterized by chronic inflammation of small blood vessels and progressive interstitial and perivascular fibrosis of the skin and multiple organs.
54
Diffuse scleroderma
widespread skin involvement at onset, rapid progression and visceral involvement
55
Limited scleroderma
skin involvement confined to the fingers, forearms, and face, with late visceral involvement.
56
CREST
calcinosis, raynaud's, esophageal dysmotility, sclerodactyly, telangiectasia
57
Patients with crest have __________ antibodies
anti-centromere
58
Patients with scleroderma may have antibodies to
Scl-70
59
Raynaud's
vasospastic response to cold or emotional stress
60
15-25% of patients with dermatomyositis have...
an underlying malignancy
61
Dermatomyositis
- autoimmune disease with immunologic injury and damage to small vessels and capillaries in skeletal muscle - Skin rash
62
Polymyositis
Like dermatomyositis without the rash
63
patients with polymyositis will have elevated (lab)
creatinine kinase
64
Treatment for polymyositis?
immunosuppressive agents
65
List the 6 main autoimmune diseases
1. SLE 2. RA 3. Systemic sclerosis (scleroderma) 4. Polymyositis 5. Dermatomyositis 6. Secondary Sjogren syndrome
66
Define mixed connective tissue disease (MCTD)
The overlap of main autoimmune disease and presence of the distinctive anti U1-RNP antibody
67
Primary immunodeficiency
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
Primary immunodeficiencies are in which populations?
Often X linked - Agammaglobulinemia
69
What are two questions you should always ask in clinic?
Is my patient immunosuppressed? | Has my patient traveled?
70
3 ways to suspect an immunodeficiency
1. Clinical history 2. presents with an opportunistic infection from a 'single organism' 3. presents with repeated infections
71
Laboratory test to assess B cell function (ab deficiencies)
immunoglobulin levels
72
Assess T cell function (cellular immunity)
CBC, flow cytometry, Skin testing to assess delayed hypersensitvity
73
Assess phagocytic function
CBC, peripheral smear (giant azurophilic granules in neutrophils, eosinophils, other granulocytes), genetic tests, specific tests of neutrophil function
74
Assess complement
total serum complement (CH50)
75
Secondary immunodeficiencies
secondary to another underlying disorder, more common than primary
76
Causes of secondary immunodeficiency
Immunosuppressive therapy, microbial infection (HIV), Malignancy, Disorders of biochemical homeostasis, Autoimmune disease, Burn, Exposure to radiation, Asplenia, Age
77
Asplenia and immunodeficiency
Loss of spenic macrophages post splenectomy can lead to increased risk of bacterial infection with encapsulated organisms, particularly with Streptococcus pneumoniae
78
Bruton's agammaglobulinemia defect
Failure of pre b cells to become mature mutation in Btk X linked
79
Bruton's agammaglubulinemia clinical presentation
SP infections Maternal ab protect at birth, presents at 6 months Decreased immunoglobulin levels
80
IgA deficiency defect
Failure of IgA to mature into plasma cells
81
IgA deficiency presentation
SP infections; giardiasis | Anaphylaxis if exposed to blood products that contain IgA
82
Common variable immunodeficiency presentation
Defect in B cell maturation to plasma cells Adult immunodeficiency disorder Sinopulmonary infections GI infections, pneumonia, autoimmune diseases, malig. Decreased immunoglobulins
83
DiGeorge Syndrome
Thymus fails to develop from 3rd and 4th pouch
84
DiGeorge Presentaiton
Hypoparathyroidism; absent thymus shadow GVH reaction T lymphocyte disorder
85
SCID
Adenosine deaminase deficiency | Autosomal recessive
86
Wiskott Aldrich
progressive deletion of B and T cells | X linked
87
Triad of Wiskott Aldrich
eczema, thrombocytopenia, SP infections | Associated with malignant lymphoma
88
Ataxia Telangiectasia
Mutation in DNA repair enzymes Thymic hypoplasia Autosomal recessive disorder
89
Ataxia Telangiectasia presentation
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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