Rheumatology: Autoimmunity/ Systemic Lupus Erythematosus

Question 1

What are two examples of organ specific autoimmunity?

Answer 1

1. Goodpastures

2. Myasthenia gravis

Question 2

What is an example of systemic autoimmunity?

Answer 2

Systemic lupus erythmatosus

Question 3

What type of immunopathology is characterized by systemic lupus erythmatosus?

Answer 3

Type II AND Type III

Question 4

Name the criteria for the diagnosis of systemic lupus erythmatosus..

Answer 4

1. Malar rash
2. Discoid rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis
6. Serositis
7. Renal involvement
8. CNS involvement (seizures or psychosis)
9. Hematologic disorders
10. Immunologic
11. Antinuclear antibodies

Question 5

How many of the diagnostic criteria does a patient need to meet in order to be diagnosed with SLE?

Answer 5

4 out of 11

Question 6

Does SLE effect more men or women?

Answer 6

Women

Question 7

What ethnicity does SLE effect more often?

Answer 7

African Americans and Hispanics

Question 8

What are the genetic predisposing factors associated with SLE?

Answer 8

HLA-DR3 and C4A null alleles

Question 9

What is upregulated in SLE?

Answer 9

INF-alpha and INF-beta it is called “INF Signature”

Question 10

When is the onset of women with SLE?

Answer 10

Childbearing years

Question 11

What are two environmental factors that affect SLE?

Answer 11

1. Hormones
   - -Estrogen: accelerating disease
   - -Androgens: protective
2. Sun Exposure
   - -UV light > Keratinocytes > Cytokines > Increased antibody release

Question 12

What are three specific antibody-mediated manifestations in SLE?

Answer 12

1. Hemolytic anemia
2. Anti-Phospholipid Antibodies
3. CNS Manifestations

Question 13

Describe the process of hemolytic anemia seen in patients with systemic lupus erythmatosus.

Answer 13

Autoantibodies to red blood > Recruitment of complement > Destruction in the reiculoendothelial system

Question 14

What does anti-phospholipid antibodies cause increased or decreased clotting?

Answer 14

Kind of both.
-Block prothrombin activation (clotting) elevated PTT

-But then increased clotting leading to thrombosis

Question 15

Name some of the CNS manifestations seen in type II SLE.

What percentage of patients with SLE have CNS manifestations?

Answer 15

Anti-neuronal antibodies- depression, cognitive dysfuction, psychosis, seizures

Stroke or chorea due to vascular ischemia from vasculitis and thrombosis

66% of patients with SLE

Question 16

Describe the manifestation of type III SLE.

Answer 16

Lupus glomerulonephritis.
Nearly all patients have some form of kidney involvement
Immune complexes deposit in kidney and recruit complement
IgG - Double stranded DNA complex