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Clinical Immunology > Tolerance, Autoimmunity and AI disorders > Flashcards

Tolerance, Autoimmunity and AI disorders Flashcards

1
Q

true or false: self-recognition/tolerance is a critical process

A

true (duh)

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2
Q

Failure to recognize self antigens results in what type of disease?

A

autoimmune diseases

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3
Q

Immune responses require what two mechanisms to happen?

A

APC presentation of foreign Ag and signal from MHC molecule on host’s cells

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4
Q

Define Tolerance

A

Lack of immune response to self antigens

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5
Q

A major mechanism of self-tolerance (autoreactivty) is the elimination of what cells?

A

Elimination of self-reactive immature lymphocytes

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6
Q

Failures of tolerance can result in what abnormalities?

A
  • autoimmune reaction to self-Ag
  • hypersensitivity
  • autoinflammatory disorders
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7
Q

B cells undergo two selection processes during maturation. Briefly describe the main focuses of these stages.

A

Positive selection: signals for survival
Negative selection: T cells in thymus that lack self-MHC are eliminated (must go through this selection before achieving immunocompetence)
Peripheral tolerance: process of negative selection in periph lymph tissues and prim lymph tissues

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8
Q

True or false: T cells dont show a marked difference in tolerance at different stages of maturation

A

true

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9
Q

What are the three pathways recognized for T cell tolerance?

A

Clonal abortion
functional deletion
t cell suppression

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10
Q

What organ is responsible for deleting autoreactive t cells that have potential to cause autoimmune diseases?

A

Thymus

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11
Q

What are the four pathways of B cell tolerance?

A

clonal abortion
clonal exhaustion
functional deletion
Ab-forming cell blockade

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12
Q

True or false: potential for autoimmunity is constantly present in immunocompetent individuals

A

true

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13
Q

What are some focuses of genetic factors in autoimmunity?

A
  • Focuses on common allelic variants (not mutations)
  • familial aggregates common
  • tendency for more than one autoimmune disease to occur in same individual
  • more frequent in women
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14
Q

What are some factors that influence the development of autoimmunity?

A

Pt age (incidence increases with age (peak at 60-70yrs))
Exogenous factors (UV, drugs, virus, chronic infectious disease)

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15
Q

What are the three major phases of Autoimmune disease?

A

Initiation (genetic predisposition/environ triggers)
Propagation (cytokines,epitope spreading)
Resolution (cell intrinsic/extrinsic)

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16
Q

What is included in Antibodies to Antinuclear AB? (ANAs)

A

DNA, histones and nonhistone protein ab

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17
Q

What is the hallmark of autoimmune diseases?

A

tissue injury caused by T lymph or ab reactivity to self

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18
Q

What is the role of the innate system in tissue injury?

A

neuts and macros are recruited to sites of ischemic injury
Macrophages release proinflam mediators and initiate programs to help in clearance of dead tissue/repair (may be detrimental if chronic)

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19
Q

What cells contribute substantially to multiple autoimmune disorders?

A

B cells

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20
Q

What are some characteristic of Organ-specific autoimmune diseases?

A
  • Produced by T cells or Ab restricted to one organ
  • lesions produced by tissue damage and autoantibodies limited to one organ
  • Examples: Type I diabetes, MS, thyroid
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21
Q

What are characteristics of Organ-nonspecific autoimmunity?

A
  • systemic autoimm disease caused by - circulation of Ab or imm complexes and affects multiple organs
  • lesions and autoab not confined to one organ
  • Examples: SLE, RA
22
Q

What is the characterization of midspectrum disorders?

A

localized lesions in a single organ and by organ-nonspecific autoantibodies

23
Q

Cardiovascular disorders: what are some key factors about vasculitis?

A
  • primary immune disorder of blood vessels
  • circulation of immune complexes
  • inflam lesions of blood vessels produce injury or necrosis of blood vessel wall
  • Ab to endothelial cells may also contribute to vasculopathy
24
Q

Cardiovascular disorders: what are some key factors about carditis?

A
  • Primary imm disorder of the heart
  • reaction of cardio myocytes to imm injury
    caused by a variety of conditions (Rheumatic fever, lyme, cardiac transplant rejection)
25
Q

An autoimmune disease characterized by the infiltration of the heart by macrophages and lymphocytes is called what?

A

Idiopathic myocarditis

26
Q

Collagen vascular disorders: Progressive systemic sclerosis (scleroderma) key facts?

A
  • collagen vascular disease of unknown cause
  • Chronic multisystem causes thickening of skin
  • initial symptoms in 3rd decade of life, slow progression
  • 40-90% have ANAs
27
Q

Collagen vascular disorders: what as some key factors about Eosinophilia-myalgia syndrome?

A
  • caused by exposure to L-tryptophan
  • pt develop severe myalgia
  • more than 50% develop scleroderma like manifestations
28
Q

Endocrine gland disorders: Thyroid disease key factors?

A

spectrum is very broad
Two major forms:
Chronic autoimmune thryroiditis and Graves disease (Shout out)

29
Q

What are the basic factors of Lymphoid (Hashimoto) chronic thyroditis?

A
  • exact cause unknown, more common in women (30-50)
  • mode of inheritence unknown (possible genetic)
  • hypothyroidism - late sequela of thyroiditis
    can have coexistence of Graves and Hashimotos
30
Q

What is the first Ab discovered against the thyroid in Hashimotos?

A

Thyroglobulin

31
Q

What is thyroid microsome?

A

Ab against thyroid microsomes, antithyroid microsomal antibodies and TPO abs (Low titers correlate with thyroid involvement)

32
Q

What Ag is directed against colloid proteins and is present in 50% of pt with subacute thyroditis?

A

Second colloid Antigen (CA2 ag)

33
Q

What Ab are found in pt with goiter or hypothyroidism?

A

T4 and T3 (thyronine and triiodothyronine)

34
Q

True or false: In hashimotos there is an increased TSH, decreased T4 and T3 and anti-TPO ab present

A

true

35
Q

What is the diagnostic eval of Hashimotos?

A

fine needle aspirate of thyroid, infiltration of lymphs

36
Q

True or false: Graves disease has high TSH and low T4 levels

A

False: Graves has low TSH and elevated free T4 levels

37
Q

What receptor differentiates between graves and toxic nodular goiter?

A

TSH receptor Ab (TRAb)

38
Q

What causes insulin-dependent diabetes mellitus?

A

imm destruction of B cells in pancreatic islets, requires exogenous insulin (CD4+ T cells responsible for imm response)

39
Q

Pt with T1D have these types of antiboidies…

A

Insulin autoantibodies (IAAs)
Glutamic acid decarboxylase (GAD)
Islet cell antigen 2 (IA-2)

40
Q

Fast facts about Latent autoimmune diabetes in Adults

A

found in pt >35 yrs old
frequently misdiagnosed as T2D
progress more rapidly to insulin dependence than T2D pt

41
Q

Dysfunction of these organs can cause which diseases?
Adrenal gland
Pituitary gland
Parathyroid gland
Polyglandular syndromes

A

adrenal glands - addisons disease
pituitary - sheenhan syndrom
parathyroid - idiopathic hypoparathyroidism
polyglandular - mulitple organs involved

42
Q

Women with unexplained infertility often have what kind of Ab?

A

smooth muscle ab, ANA and antiphospholipid Ab, Antisperm

43
Q

Chronic inflam disease of unknown cause that affects lacrimal, salivary and other excretory glands is called what?

A

Sjogren Synrome
More common in women
( funfact: phonetically this is pronounced S-ee-yorg-ren from my limited swedish language knowledge)

44
Q

The secondary form of Sjorgen syndrome is associated with what other disorders?

A

RA and connective tissue disorders

45
Q

Atropic Gastritis is the chronic inflammation of what? what is its characteristics?

A

inflammation of gastric muscosa
AutoAb to gastric parietal cells, proton pump ATPase, and intrinsic factor
Always accompanies pernicious anemia

46
Q

What disorder is classified as a megaloblastic anemia and is caused by a deficiency of vitamin B12?

A

pernicious anemia

47
Q

What kind of staining pattern is this?

A

ANAs most frequent stain pattern is homogenous

48
Q

What are the 4 groups of Autoimmune hemolytic anemia?

A

warm- reactive (most common)
cold reactive (<20%)
paroxysmal cold hemoglobinuria (rare)
drug-induced hemolysis

49
Q

What are 4 neuromuscular disorders?

A

ALS
Guillain-barre syndrom (inflam plyneuropathies)
Myasthenia gravis
Ms (most common demyelnating disease of CNS)

50
Q

What are three common forms of Multiple sclerosis?

A

Primary progressive
secondary
progressive relapse (most common)

51
Q

What are two skeletal muscle disorders?

A

polymyositis and dermatomyositis

52
Q

what are 4 skin disorders?

A

discoid lupus
bullous pemphigoid
pemhigus group
dematitis gerpetiformis

Clinical Immunology (14 decks)

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