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USMLE Pathology > Autoimmune disorders > Flashcards

Autoimmune disorders Flashcards

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USMLE® Step 1 flashcards
1
Q

What is the prevalence of autoimmune disorders in the USA?

A

~ 1%

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2
Q

Self-reactive t-cells undergo apoptosis in a process known as what?

A

Negative selection

- Part of T-cell education

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3
Q

When will T-cells become anergic - through which process?

A

When it does not receive a second signal

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4
Q

What type(s) of hypersensitivity reaction is SLE?

A
  • Type II (cytotoxic)

- Type III (antigen-antibody complex)

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5
Q

What are the classic features of SLE?

A
  • Fever
  • Weight loss
  • Malar ‘butterfly’ rash, especially on exposure to sunlight
  • Arthritis
  • Pleuritis, pericarditis, endocarditis
  • CNS psychosis
  • Renal damage
  • Anemia, thrombocytopenia or leukopenia
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6
Q

What renal damage is the most common injury in SLE?

A

Diffuse proliferative glomerulonephritis (nephritic syndrome)

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7
Q

What is unique about Libman-Sacks endocarditis?

A

Vegetations on both sides of heart valve

- Not infectious - inflammatory reaction to deposition of antiG-antiB complexes

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8
Q

How can those with SLE become susceptible to infection?

A

Antibodies destroy WBCs

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9
Q

What are the 2 main antibodies which cause SLE?

A
  • ANA is sensitive

- Anti-dsDNA is specific

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10
Q

What is the characteristic antiB in drug induced lupus?

A

Antihistone antibody

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11
Q

What drugs can cause drug-induced lupus?

A
  • Hydralazine
  • Procainamide
  • Isoniazid
  • Methyldopa
  • Minocycline
  • Phenytoin
  • Sulfa drugs
  • Etanercept
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12
Q

How would SLE with anti-histone antiBs usually be treated?

A

Removal of drug

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13
Q

What is anti-phospholipid syndrome due to (what antiBs)?

A

Autoantibodies against proteins bound to phospholipids

  • Anticardiolipin
  • Lupus anticoagulant
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14
Q

What percentgae of those with SLE will develop antiphospholipid syndromw?

A

~ 30%

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15
Q

What antiB can give a false-positive syphilis test in SLE?

A

Anticardiolipin

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16
Q

What 2 la studies can not be interperted correctly (give false +ves) in antiphospholipid syndrome?

A
  • False positive syphilis test

- Falsely-elevated PTT lab studies

17
Q

What types of thrombus cab those with anti-phospholipid get?

A
  • DVT
  • Budd-chiari
  • Placental thrombosis - pregnancy loss
  • Stroke
18
Q

What is budd-chiari syndrome most commonly seen with?

A

Polycythemia vera

19
Q

What is Sjogren syndrome a autoimmune destruction of?

A

Lacrimal and salivary glands

20
Q

What type of HS reaction is Sjorgen syndrome due to?

A

Type IV - lymphocyte mediated damage with fibrosis

21
Q

What is the classic presentation of Sjorgen syndrome?

A
  • Dry eyes
  • Dry mouth
  • Recurrent dental carries in older woman
22
Q

What antibodies are associated with Sjorgen syndrome?

What are they called and what do they target?

A

ANA and anti-ribonucleoprotein antibodies

- Anti-SS-A and anti-SS-B

23
Q

What is Sjorgen syndrome most commonly associated with?

A

RA

24
Q

What cancer are those with Sjorgen syndrome at incrased risk of?

A

B-cell lymphoma

25
Q

How will be cell lmmphoma in Sjorgen synrome present?

A

Unilateral enlargement of parotid late in disease course

- Be wary as those with Sjorgen have bilateral permanent enlargement of the parotids

26
Q

What is scleroderma?

A

Autoimmune tissue damage with activation of fibroblasts and deposition of collagen (fibrosis) on tissue

27
Q

What organs/structures does diffuse scleroderma affect most commonly?

A
  • Skin
  • Esophagus - dysphagia due to disordered motility
  • Any organ there is early visceral involvement
28
Q

What antibody characterizes diffuse scleroderma?

A
  • ANA

- Anti-DNA topoisomerase I (Scl-70) antibody

29
Q

What are the characteristics of localised scleroderma?

A

Local skin involvement and late visceral involvement

  • Calcinosis / anti-Centromere antiBs
  • Raynaud phenomenon
  • Esophageal dysmotility
  • Sclerodactyly (hands fingers)
  • Telangiectasias of skin (dialted blood vessels on surface of skin)

CREST

30
Q

Mixed connective tissue diseasee has characteristics of what other diseases?

A
  • SLE
  • Systemic sclerosis
  • Polymyositis - proximal muscle disease
31
Q

What antibodies characteristic mixed connective tissue disease?

A

Serum antiBs against

- U1 ribonucleoprotein

USMLE Pathology (15 decks)

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