Lupus

Question 1

What is Systemic Lupus Erythematous?

Answer 1

Chronic inflammatory disease of unknown cause.

Question 2

What does SLE affect?

Answer 2

Skin, joints, kidneys, lungs, nervous system, serous membranes

Question 3

What is SLE in origin?

Answer 3

Autoimmune

Question 4

Clinical course of SLE characterized by what?

Answer 4

Periods of remission and chronic/acute relapses

Question 5

What factors play a role in pathogenesis?

Answer 5

Genetics and environmental factors

Question 6

Who has higher incidence?

Answer 6

Blacks and indian tribes

Question 7

Which genetic HLA haplotypes are increased in SLE population?

Answer 7

HLA DR2 and DR3

Question 8

Which Haplotypes on chromosome 2 are associated with increased incidence of rheumatoid and lupus?

Answer 8

STAT4 haplotypes

Question 9

What do STAT4 haplotypes code for?

Answer 9

Cytokines

Question 10

Deficiencies of which complements are associated with SLE and discoid lupus?

Answer 10

Complements C2 and C4

Question 11

What do twin studies show about SLE?

Answer 11

Monozygotic twins both more commonly develop SLE than dizygotic twins

Question 12

What is the estimated number of susceptibility genes needed for development of SLE disease?

Answer 12

4

Question 13

Which drugs can cause an SLE like illness?

Answer 13

Procainamide and hydralazine

Question 14

What are three other environmental factors than can start SLE?

Answer 14

1) Hairy dye
2) Smoking
3) UV light

Question 15

Women of what age are at greatest risk?

Answer 15

Childbearing years

Question 16

Estrogen effect on SLE?

Answer 16

Prolongs survival of autoimmune cells

Question 17

Effects of prolonged autoimmune cells in SLE?

Answer 17

Increases T-helper cell cytokine production

Stimulates B-cell to produce autoantibodies

Question 18

Correlation between CD40 ligand in women?

Answer 18

More X chromosomes express CD40 ligand in women with lupus than women without

Question 19

Is a low level of auto reactivity pathologic?

Answer 19

No, physiologic

Question 20

Activated T cells stimulate B cells to produce what?

Answer 20

Autoantibodies

Question 21

Total T cells are what in lupus?

Answer 21

Decreased

Question 22

T cell function is skewed toward what in lupus?

Answer 22

B-cell help

Question 23

Direction of IL-10 and 12 in SLE?

Answer 23

IL-10 increased

IL-12 decreased

Question 24

Problems with immune regulation in SLE?

Answer 24

Phagocytes defective in clearing immune complexes

T-cell CD8 suppressor cells deficient in function

Question 25

What happens to lymphocytes in patients with SLE?

Answer 25

Apoptosis

Question 26

Increased apoptosis in SLE can lead to what?

Answer 26

Leakage on intracellular antigens which triggers further autoimmune response

Question 27

Where does tissue damage in blood vessels and kidneys come from?

Answer 27

Deposition of antigen-antibody complexes

Question 28

Antibodies to phospholipids is known as what?

Answer 28

Lupus anticoagulent

Question 29

Lupus anticoagulent is associated with what?

Answer 29

``` Prolonged PTT Venous/arterial thrombosis Fetal distress Thrombocytopenia Livedo reticularis ```

Question 30

Most common systemic symptom of SLE?

Answer 30

Fatigue (Too nonspecific)

Question 31

How does arthralgia present in SLE?

Answer 31

Symmetric, mimics RA Not as erosive as RA WBC

Question 32

SLE patients have what reaction to sun?

Answer 32

Rashes or activation of systemic disease | Butterfly rash

Question 33

Erythematous maculopapular eruption is what?

Answer 33

SLE pruritic eruption that can occur after sun exposure

Question 34

What is Discoid lupus?

Answer 34

Sharply defined papule and plaques that become atrophic in the center while edges remain edematous and erythematous. Scars

Question 35

Difference between subacute cutaneous lupus and discoid lupus?

Answer 35

Looks like discoid but no scarring

Question 36

Where are oral ulcers commonly found in SLE?

Answer 36

Hard and soft palate

Question 37

How common is alopecia in SLE?

Answer 37

Greater than 50%, can be patchy

Question 38

Cardiac problems due to SLE?

Answer 38

Libman-Sacks (Valve replacement due to bacteria) Resting tachycardia, abnormal EKG Coronary artery disease due to immune complex Conduction deficits

Question 39

What is a big clue for SLE diagnosis?

Answer 39

Leukopenia

Question 40

How will erythrocyte sedimentation rate present in SLE?

Answer 40

Elevated

Question 41

Four pathologic types of Kidney damage in SLE?

Answer 41

1) Mesangial glomerulonephritis 2) Focal proliferative nephritis 3) Diffuse proliferative glomerulonephritis 4) Membranous glomerulonephritis

Question 42

How does Cerebrospinal fluid look in SLE?

Answer 42

Usually normal

Question 43

Abnormal EEG common or uncommon in SLE?

Answer 43

Common 80%

Question 44

What is Raynaud's phenomenon?

Answer 44

3 color change of fingers on exposure to cold. White-> blue -> red

Question 45

Lupus anticoagulant is associated with what?

Answer 45

Frequent miscarriages

Question 46

Bone most frequently damaged by avascular necrosis

Answer 46

Hip

Question 47

Most important lab diagnostic?

Answer 47

ANA

Question 48

Key to SLE treatment?

Answer 48

Patient education

Question 49

Mild SLE treated with?

Answer 49

Antimalarial hydroxychloroquin

Question 50

Major organ disease from SLE treated with?

Answer 50

Corticosteroids and cytotoxic agents

Question 51

Greatest cause of death in SLE patients?

Answer 51

Infection