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Pathoma > 2.4 Autoimmune Disorders > Flashcards

2.4 Autoimmune Disorders Flashcards

1
Q

Was is the underlying cause of autoimmune disorders?

A

Loss of self-tolerance

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2
Q

Positive Selection

A

Double positive cells are checked for binding of MHC and Ag - cells that progress become single positive cells

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3
Q

Negative Selection

A

Single positive cells are checked for binding to self-Ag - those that do are killed so that they are not allowed to attack self tissues

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4
Q

What happens with AIRE mutation?

A

Autoimmune Polyendocrine Syndrome - loss of the presentation of some self Ag on the dendritic cells and the medullary cells in the thymus leading to autoimmune lymphocytes

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5
Q

What is the triad of autoimmune polyendocrine syndrome?

A
  • Hypoparathyroidism
  • Adrenal Failure
  • Candida Infections
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6
Q

How is central tolerance in B cells generated?

A

Negative Selection of the immature Ig

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7
Q

What are the mechanisms that occur with failure of negative selection for B cells?

A
  • Receptor Editing

- Apoptosis

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8
Q

Peripheral Tolerance

A

T cell that binds self-Ag with the T-cell receptor on the MHC II without the secondary CD28-B7 signal can lead to:

  • Anergy
  • Apoptosis
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9
Q

Autoimmune Lymphoproliferative Syndrome (ALPS)

A

Fas apoptosis pathway mutation - allows for the survival of self reactive lymphocytes in the periphery - IgG generated against cells in the blood

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10
Q

What are some signs of ALPS?

A
  • Cytopenia
  • Lymphadenopathy
  • Hepatosplenomegaly
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11
Q

What does FOXP3 mutation cause?

A

IPEX Syndrome

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12
Q

What are the markers of T regulatory cells?

A
  • CD25

- FOXP3

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13
Q

What are some of the triggers for the self-reactive lymphocytes?

A
  • Bystander Activation

- Molecular Mimicry

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14
Q

Bystander Activation

A

Infection or inflammation leads to the activation of the self-reactive lymphocyte as a side effect of the triggering condition - but it is not direct

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15
Q

Systemic Lupus Erythematous

A

Chronic and systemic with flares and remissions

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16
Q

What populations are SLE more common in?

A

African Americans and Hispanics - more seen in females

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17
Q

What is the pathogenesis of SLE?

A

Type III reaction due to Ag-Ab complex deposition in multiple tissues as Ab are directed against the hosts nuclear material leading to the activation of complement

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18
Q

How is SLE developed?

A
  • UV light causes cell apoptosis releasing DNA
  • Self-reactive B cells are activated
  • DCs amplify the signal leading to the priming of B cells to the next exposure event
19
Q

What is early complement deficiency associated with (C1q, C4, C2)?

A

Lupus

20
Q

What are some clinical findings of lupus?

A
  • Fever
  • Weight Loss
  • Fatigue
  • LAD
  • Raynaud’s Sign
  • Butterfly Rash on Face
  • Discoid Rash
  • Nasopharyngeal Ulcers
  • Arthritis
  • Sensitivity to Sunlight
  • Renal Damae
  • Libman-Sacks Endocarditis
21
Q

What hypersensitivity reaction is responsible for the anemia, thrombocytopenia and leukopenia seen in lupus?

A

Type II - direct Ab

22
Q

What is a lab sign for lupus?

A

ANA Ab

  • anti-dsDNA or anti-Sm are the specific Ab
  • antiphospholipid Ab also indicative of SLE
23
Q

What are the 3 antiphospholipid Ab?

A
  • Anticardiolipin
  • Lupus Anticoagulant
  • Anti-B2 Glycoprotein I
24
Q

What can anticardiolipin cause a false positive for?

A

Syphilis - VDRL and RPR

25
Q

What labs does lupus anticoagulant interfere with?

A

It will cause an elevated PTT

26
Q

What is the coagulation state caused by lupus anticoagulant - APA Syndrome?

A

Hypercoagulable which is PARADOXICAL

27
Q

Antihistone Ab

A

Characteristic of drug induced lupus

28
Q

What drugs can induce lupus?

A
  • Hydralazine
  • Procainamide
  • Isoniazid
29
Q

What are some treatments for SLE?

A
  • Avoid sun
  • Glucocorticoids
  • Immunosuppressants
30
Q

Sjogren Syndrome

A

Autoimmune destruction of the lacrimal and salivary glands - Type IV lymphocyte mediated damage with fibrosis

31
Q

What are the clinical findings o f Sjogren Syndrome?

A

Dry eyes and mouth

32
Q

Who is a common patient of Sjogren syndrome?

A

Older woman

33
Q

What is a common associated disorder with Sjogren syndrome?

A

Rheumatoid Arthritis - rheumatoid factor will often be present

34
Q

What are the autoimmune Ab in Sjogren syndrome?

A

Anti-ribonucleoprotein - two of this class are the anti-SSA and anti-SSB

35
Q

What can anti-SSA cause?

A

Neonatal lupus and congenital heart block

36
Q

What cancer does Sjogren’s syndrome increase risk of?

A

B-cell Lymphoma

37
Q

Scleroderma

A

Hardening of tissue due to fibroblast activation and deposition of collagen

38
Q

What is the limited type of scleroderma?

A

CREST Syndrome

39
Q

What are the features of CREST syndrome?

A 
Calcinosis/Anti-Centromere Ab
Raynaud's Sign
Esophageal Dysmotility
Sclerodactyly
Telangiectasis
40
Q

What is the diffuse type of scleroderma?

A

Skin with visceral involvement often int eh GI tract and lungs (most common cause of death). Can also affect the kidneys (second most common cause of death)

41
Q

What are the Ab found in diffuse scleroderma?

A

DNA Topoisomerase I Ab

42
Q

Mixed Connective Tissue Disease

A

Mixed features of SLE, sclerosis and polymyositis

43
Q

What Ab will be seen in mixed connective tissue disease?

A

ANA with serum Ab against U1 ribonucleoprotein

Pathoma (119 decks)

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