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Unit 3: Immune Related Diseases > Section 2: Systemic autoimmunity - Systemic Lupus Erythematosus > Flashcards

Section 2: Systemic autoimmunity - Systemic Lupus Erythematosus Flashcards

1
Q

Autoimmunity

A

failure of an organism to recognize self antigens, resulting in immune responses to that organism’s tissues
-if affects 5-7% of the population
-a variety of factors result in autoimmune diseases (genetics, environmental exposures, and infections)
-in general all result from the breakdown of self tolerance and the development of an adaptive immune response to self antigens
-more common in women than in men

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2
Q

central tolerance

A
  1. In the thymus, T cell progenitors give rise to billions of thymocytes, each with a different T cell receptor
  2. thymocytes are positively selected by epithelial cells in the cortex of the thymus
  3. positively selected thymocytes to survive and divide
  4. Positively selected thymocytes clones are negatively selected in the thymic medulla
  5. clones surviving negative selection leave the thymus for circulation (less than 1% are selected by pathogens)
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3
Q

Mechanisms that contribute to self-tolerance

A

-negative selection in bone marrow (B cell) and thymus (T cell)
-expression of tissue-specific proteins in the thymus
-no lymphocytes access to some tissue (brain, eye, testis)
-supression of autoimmune response by regulator T cells
-induction of anergy in autoreactive B+T cell

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4
Q

anergy

A

-state of programmed functional inactivation following exposure to an antigen. B+ T cells are said to be anergic when they cannot respond to their specific antigen

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5
Q

senescence

A

(immunosenescence) is the graudal loss of immune function due to natural aging

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6
Q

Autoimmune effector functions

A

-similar to hypersensitivity reactions, autoimmune diseases can be classified by the effector mechanism causing the disease
-three types: Type II, Type III, and Type IV
-can also be group by organ specific or systemic

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7
Q

autoimmune hemolytic anemia: autoantigen and consequence

A

-Rh blood group antigens, I antigen
-destruction of RBC by complement and phagocytes anemia

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8
Q

Autoimmune thrombocytopenia purpura: autoantigen and consequence

A

-platelet integrin
-gpllb:llla
-abnormal bleeding

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9
Q

Goodpasture’s syndrome: autoantigen and consequence

A

-non collagenous domain of basement membrane collegen type IV
-glomerulonephritis , pulmonary hemorrhage

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10
Q

pemphigus vulgaris: autoantigen and consequence

A

-epidermal cadherin
- blistering of skin

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11
Q

acute rheumatic fever: autoantigen and consequence

A

-streptococcal cell wall antigens, antibodies cross-react with cardiac muscle
-arthritis
-myocarditis
-late scarring of heart valves

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12
Q

graves’ disease: autoantigen and consequence

A

-thyroid stimulating hormone receptor
-hyperthyrodism

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13
Q

myasthenia gravis: autoantigen and consequence

A

-acetylocholine receptor
-progressive weakness

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14
Q

type 2 diabetes (insulin resistant diabetes): autoantigen and consequence

A

-insulin receptor (antagonist)
-hyperglycemia
-ketoacidosis

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15
Q

hypoglycemia: autoantigen and consequence

A

-insuline receptor (agonist)
-hypoglycemia

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16
Q

subacute bacterial endocarditis: autoantigen and consequence

A

-type III
-bacterial antigen
-glomerulonephritis

17
Q

mixed essential cryoglobulinemia: autoantigen and consequence

A

-type III
-rheumatoid factor IgG complexes (with or without hepatitis C antigens)
-systemic vasculitis

18
Q

systemic lupus erythematosus: autoantigen and consequence

A

-type III
-DNA, histones, ribosomes, snRNP, scRNP
-glomerulonephritis
-vasculitis
-arthritis

19
Q

Type I diabetes (insulin-dependent diabetes mellitus): autoantigen and consequence

A

-type IV
-pancreatic B cell antigen
-B cell destruction

20
Q

Rheumatoid arthritis: autoantigen and consequence

A

-unknown synovial joint antigen
-joint inflammation and destruction

21
Q

multiple sclerosis: autoantigen and consequence

A

-myelin basic protein, proteolipid protein
-brain degeneration paralysis

22
Q

Lupus erythematosus

A

-is a group of similar conditions which IgG antibody is formed to nuclear antigens (DNA histones) that have been released due to cell death
- systemic lupus (most common)
-discoid lupus
-drug induced lupus
-neonatal lupus

23
Q

SLE Epidemiology

A

-SLE is 10x more common in women than men
-more prevalent in non-Europeans
-no single genetic factor or cause linked to SLE, though there is a genetic component that is heritable in families (HLA)
-environmental exposure have been suggested to play a role in onset or exacerbation (infection, drugs, hormones, stress)

24
Q

SLE symptoms

A

-difficult to diagnosis b/c symptoms mimic a variety of other clinical syndromes
-symptoms caused by immune complex deposition: fever, pain, confusion, proteinuria, loss of appetite
-90% of SLE patients suffer from arthritis
50% of SLE patients suffer from classical butterfly facial rash
-was first described as SLE on the basis of the butterfly shaped rash on the face that gives the patient a wolf-like apperance
-rash is caused by the deposition of immune complexes in the skin (type III)

25
Q

erythema- meaning
lupus- meaning

A

red, redness of the skin
-wolf

26
Q

antinuclear antibodies (ANA)

A

Ab directed against nuclear antigens
-other contributing factor to the development of ANAs is an abnormality in apoptosis
-body replaces more than 1 million cells every second
-the inefficient clearing of dying cells and the accumulation of cell remnants are considered an intrinsic defect that leads to the development of ANAs and ultimately SLE
-there is also probably a breakdown in T-cell tolerance
-nuclear antigens form immune complexes that travel throughout the body
-antibodies that target “normal” proteins within the nucleus of a cell
-immune complex deposition in: kidneys, joints, blood vessels, skin, lungs, liver, nervous system, heart

27
Q

diagnosis and testing (SLE)

A

-test for the presence of ANAs (95% of SLE patients have a positive ANA, but ANA can also be found in other autoimmune disease (sjorgen’s syndrome and rheumatoid arthritis)

28
Q

indirect immunofluorescence (IFF)

A

-classic test used to test for ANAs
-test various dilutions of patients serum against a cell substrate to observe presence and pattern of ANA staining
-cell substrate: HEp-2 cells, human laryngeal epithelioma cancer cells

29
Q

common ANA patters

A

-homogenous diffuse
-nucleolar
-speckled
-peripheral (rim)

30
Q

systemic lupus erythematosus

A

-autoreactive B cells probably exist normally in circulation but without a T-cell that also recognizes the auto antigen… the B cell will not be activated and produce antibodies
1. H1-specific helper T cell activates histone H1-specific B cells that process nucleosomes containing H1 and present H1 peptides
2. Activates B cell differentiates into plasma cells secreting anti-H1 antibody
3. H1-specific helper T cell activates DNA- specific B cells that process nucelosomes and present H1 peptides
4. Activated B cell differentiates into plasma cells secreting anti-DNA antibody

31
Q

SLE (ANA-IIF)

A

-have the hept2 cell mobilized onto the cell and incubate the patient serum to see if they have antibodies. Come in with a detection antibody (anti-human IgG) and get it to fix and incubate then wash away. Then set it up with a fluroscent scope

32
Q

Homogenous staining: antibody/antigen and disease

A

-deoxyribonucleoprotein
-histones
-dsDNA
-Systamatic lupus
-most important

33
Q

peripheral staining: antibody/antigen and disease

A

-dsDNA
-SLE
-most important

34
Q

speckled staining: antibody/antigen and disease

A

-saline extractable antigens
-anti smith Ab (Sm)
-SLE

-Anti-Ro (SS-A)
- Anti-La (SS-B)
-sjogren’s syndrome

-Anti-topoisomerase I (Scl-70)
-systemic sclerosis

anti-nuclear ribonucleoprotein (RNP)
-SLE
-Mixed connective tissue disease (MCTD)

35
Q

Nucleolar staining: antibody/antigen and disease

A

-nucleolar RNA
-SLE
-systemic scleroderma

36
Q

Three common ANA type for SLE

A

-Anti-dsDNA
-Anti-Sm
-Anti-histone

37
Q

SLE testing and ELISA

A

-ELISA’s are used to narrow the diagnosis (mostly in the case of speckled stained cells)
-the ELISA can identify what specific antibody is present in the patient serum
-antibodies to dsDNA producing a homogenous or peripheral stain are the most disgnostic for SLE
(70% present in patients with SLE)(<1% in patients without SLE)
-Anti-Sm is also seen predomiately in SLE patients but is rarer 20-30% and produced the speckled pattern

38
Q

SLE testing and flow cytometry

A

-each bead has its own fluorescent profile is coated with a different specific antigen
-mix together with patient IgG or patient sample, then come in with anti-human IgG and as long as their is patient antibody to patient antigen you will get production of complex
-you wash away and use flow cytometer to read as a flow cytometry

Unit 3: Immune Related Diseases (4 decks)

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