Autoimmunity Flashcards Preview

Pathology > Autoimmunity > Flashcards

Flashcards in Autoimmunity Deck (21):
1

Autoimmunity

Immune reaction against self-antigen

2

Central Tolerance

Central tolerance - clonal deletion of self-reactive T and B lymphocytes

thymus - T cells
bone marrow - B cells

3

Peripheral Tolerance

Peripheral tolerance - removal of self-reactive T cells that escape intrathymic negative selection

4

AIRE

• transcription factor
• promotes the expression of self proteins by thymic
epithelial cells
• thymic expression of these proteins permits the clonal elimination of tissue-specific T cells

5

Autoimmune Poly-Endocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED)

• organ-specific autoimmune disease caused by a defect in a single gene
• all persons who lack a functional autoimmune regulator gene (AIRE) gene develop the disease and one functional gene prevents the disease

6

What are symptoms of APECED?

pernicious anemia
autoimmune hepatitis
alopecia
vitiligo

7

What is the role of infection in autoimmunity?

Molecular mimicry can lead to the generation of auto-Ab

8

What is the main population affected by Systemic Lupus Erythematosus?

Women - African American women have high frequency

9

What is the genetic component of SLE?

- HLA-DQ locus linked to production of anti-double-stranded DNA, anti-Sm, and antiphospholipid antibodies
- Inherited deficiencies of complement components, such as C2, C4, or C1q

10

What are some triggers for SLE?

• Ultraviolet B light
• Sex hormones
• Dietary factors
• Infectious agents
• Smoking

11

What sex hormones are related to SLE?

Abnormal estrogen metabolism in both male and female patients with SLE as well as low levels of testosterone

12

What are some drugs that can trigger SLE?

– Procainamide
– Hydralazine
– D-penicillamine
– Isoniazid
– Minocycline
– Phenytoin
– Ethosuximide

13

What are the 3 mechanisms that can underlie SLE?

Hyperactive T cells
Hyperactive B cells
Inadequate regulatory mechanisms

14

What antigen is specific for SLE diagnosis?

Smith (Sm) antigen

15

What are some of the common identifiers for SLE?

Patients with depressed complement levels, dsDNA and two criteria most likely have SLE.

16

SLE Treatment

• Nonsteroidal anti-inflammatories - helpful with fever arthralgia or arthritis but systems should be assessed immunologically and followed for renal and hepatic dysfunction
• Antimalarials - useful for skin and arthritis symptoms
• Corticosteroids - used in high doses 1-2mg/kg for life-
threatening SLE

17

What is scleroderma (systemic sclerosis)?

It is a multisystem disorder characterized by inflammatory, vascular, and fibrotic changes of skin and various internal organ systems

18

Diffuse cutaneous scleroderma

– rapid development of symmetric skin thickening
– high risk of visceral disease early in the course

19

CREST Syndrome

Calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasias

20

What correlates with the severity of systemic sclerosis?

Thickness of the skin - hence the level of fibrosis

21

Scleroderma Treatment

- Corticosteroids for inflammation
- CCBs for Raynaud's phenomenon (nifedipine)

Decks in Pathology Class (203):