Pathoma: Autoimmune Disorders

Question 1

Positive selection is the process in which ________.

Answer 1

T cells are selected based on their ability to bind MHC

Question 2

Negative selection is the process in which _________.

Answer 2

T cells that bind self antigen are selected out

Question 3

Double positive cells are found in the ______ of the thymus, while single positive cells are found in _______.

Answer 3

cortex; medulla

Question 4

What two cells express self antigens in the thymus?

Answer 4

Dendritic cells and medullary epithelial cells

Question 5

The triad of autoimmune polyglandular syndrome is __________.

Answer 5

adrenal insufficiency, hypoparathyroidism, and candida infections

Question 6

What is peripheral tolerance?

Answer 6

If a lymphocyte binds antigen in the body without a co-stimulatory signal multiple times (such as CD4 binding to a B cell), it will undergo apoptosis.

Question 7

Autoimmune lympho-proliferative syndrome (ALPS) is a disorder of _________.

Answer 7

the apoptotic pathway needed for peripheral tolerance

Question 8

Other than secreting immunosuppressive cytokines, how else do T regs suppress the immune response?

Answer 8

They also express CTLA-4, which binds to B7 on antigen-presenting cells; B7 also binds to CD28 on helper T cells, so interfering with that pathway prevents more activation of CD4 T cells.

Question 9

What three receptors do Tregs have?

Answer 9

CD4, CD25, and FoxP3

Question 10

Describe IPEX?

Answer 10

IPEX is an X-linked disorder of the FoxP3 receptor that results in autoimmunity, polyendocrinopathy and enteropathy.

Question 11

Typically lupus patients have the disease __________.

Answer 11

for the rest of their lives

Question 12

The ________ complement components are necessary for getting rid of immune complexes.

Answer 12

early

Question 13

Raynaud’s phenomenon results from _________.

Answer 13

arterio-vaso spasms that limit blood supply to extremities

Question 14

Both type ____ and ____ immune sensitivities are found in lupus.

Answer 14

II (anemia); III (glomerulonephritis)

Question 15

What is unique about Libman-Sacks endocarditis?

Answer 15

In LSE, vegetations grow on both sides of the valve, as opposed to the usual one.

Question 16

Anticardiolipin antibodies can lead to false positive _______ tests.

Answer 16

syphilis

Question 17

Antihistone antibodies are characteristic of __________.

Answer 17

drug-induced lupus (commonly isoniazid, hydralazine, and procainamide)

Question 18

The two most common causes of death in SLE are __________.

Answer 18

renal failure and infection

Question 19

Sjögren’s syndrome is characterized by ________.

Answer 19

type IV autoimmune destruction of the lacrimal and salivary glands

Question 20

Serum tests in Sjögren’s often show _______.

Answer 20

rheumatoid factor and anti-ribonucleoprotein antibodies

Question 21

Neonatal lupus (often from Sjögren’s syndrome in pregnancy) can result in _________.

Answer 21

congenital heart block

Question 22

Biopsy of the salivary gland will show ________ in those with Sjögren’s syndrome.

Answer 22

lymphocyte infiltration

Question 23

Systemic sclerosis results from ____________.

Answer 23

an autoimmune reaction to mesenchymal cells, leading to fibrosis (collagen deposition) of connective tissue

Question 24

Those with systemic sclerosis usually have ________ antibodies, while those with diffuse scleroderma have _______ antibodies.

Answer 24

anti-centromere; anti-topoisomerase