Ch 4: Autoimmune High Yield

Question 1

How do autoimmune disorders arise? Principles of immunology

Answer 1

Loss of self-tolerance
1) self-reactive lymphocytes do not undergo apoptosis in thymus or bone marrow (no negative selection)
OR
2) self-reactive lymphocytes become anergic

Question 2

How do lymphocytes become anergic?

Answer 2

recognition of antigen in peripheral lympoid tissues w/o 2nd signal

Question 3

What is a common etiology of autoimmune disorders?

Answer 3

environmental trigger in genetically susceptible individuals

Question 4

Which two types of hypersensitivity reactions damage multiple tissues in the host for SLE?

Answer 4

Type II (cytotoxic) = direct damage to tissue
Type III (antigen-Ab complex)

Question 5

What are the clinical features of SLE?

Answer 5

fever and weight lose
malar “butterfly” rash, esp w/sunlight exposure
arthritis
pleuritis and pericarditis (serosal surfaces)
CNS psychosis
renal damage
endocarditis/myocarditis/or pericarditis
anemia
thrombocytopenia
leukopenia

Question 6

What is the most common injury to kidneys in SLE?

Answer 6

diffuse proliferative glomerulonephritis

Question 7

What is Libman-Sacks endocarditis?

Answer 7

SLE classic finding

small sterile deposits/vegetations on both sides of mitral valve, not infectious

Question 8

What 2 autoantibodies characterize SLE?

Answer 8

1) antinuclear antibody (ANA)…Sm antigen (spliceosome)

2) anti-dsDNA antibody

Question 9

Which autoantibody is sensitive but not specific for SLE?

Answer 9

Antinuclear antibody

Question 10

Which autoantibody is highly specific for SLE?

Answer 10

Anti-dsDNA antibody

Question 11

Which autoantibody is charcteristic of drug-induced SLE?

Answer 11

Antihistone Ab

Question 12

What 3 drugs can cause drug induced SLE?

Answer 12

Hydralazine, procainamide, isoniazid

Question 13

What is Antiphospholipid antibody syndrome?

Answer 13

Associated with SLE
Autoantibodies to proteins bound to phospholipids
Anticardiolipin & Lupus anticoagulant
Result: arterial/venous thrombosis, DVT, Budd-Chiari (hepatic vein thrombosis), placental thrombosis, stroke

Requires lifelong anticoagulation

Question 14

What could lead to a false + syphilius test and falsely elevated pTT labs, respectively?

Answer 14

anticardiolipin and lupus anticoagulant autoantibodies

Question 15

What is Sjogren Syndrome?

Answer 15

Autoimmune destruction of lacrimal and salivary glands

Question 16

What are the clinical features of Sjogren Syndrome?

Answer 16

keratoconjunctivitis (dry eyes)
xerostomia (dry mouth)
may be associated with RA

Question 17

Which hypersensitivity rxn is associated with Sjogren Syndrome?

Answer 17

Type IV Hypersensitivity

lymphocyte-mediated damage with fibrosis

Question 18

Which autoantibodies are associated with Sjogrens Syndrome?

Answer 18

ANA (antinuclear antibody)

anti-ribonucleoprotein antibodies ( anti-SS-A/ro and anti-SS-B/la)

Question 19

What other disease are ppl with Sjogrens Syndrome at risk to develop?

Answer 19

B-cell lymphoma

presents as unilateral enlargement of parotid gland

Question 20

What is scleroderma?

Answer 20

Activation of fibroblasts and deposition of collagen (fibrosis)

Question 21

What is localized scleroderma? Which autoantibody is it associated with?

Answer 21

involves only skin, anti-DNA topoisomerase II antibody

Question 22

What is systemic scleroderma?

Answer 22

involves skin and other visceral organs

Question 23

What is limited type systemic scleroderma?

Answer 23

limited areas of skin (hands, face, neck) with late visceral involvement
CREST syndrome

Question 24

What is CREST syndrome? Each letter of CREST?

Answer 24

Limited type systemic scleroderma, late visceral involvement
C = Calcinosis/anti-Centromere antibodies
R = Raynaud Phenomenon
E = Esophageal dysmotility&raquo_space; dysphagia
S = Sclerodactyly
T = Telangiectasias of skin (small vessels dilated)

Question 25

What is diffuse type systemic scleroderma?

Answer 25

skin and any visceral organ (GI, lungs, kidneys)

Question 26

What is mixed connective tissue disease (MCTD)?

Answer 26

Autoimmune-mediated damage with mixed features of SLE, systemic scleroderma, and polymyositis

Question 27

What are the autoantibodies in MCTD?

Answer 27

anti-U1 ribonucleoprotein