systemic lupus erythematosus

Question 1

What % of patients with SLE have skin findings? Which cutaneous lupus is most commonly seen?

Answer 1

• 80%, ACLE is most common

Question 2

African americans have a ____ fold increased incidence of SLE and presents (earlier/later) with (higher or lower mortality)

Answer 2

4 fold increased incidence, presents earlier and has higher mortality

Question 3

Pathogenesis of SLE:

Answer 3

• environmental factors + genetics:
• patients have type I gene signature in peripheral blood leukocytes
• phagocytic defect of monocytes and macrophages to clear apoptotic cells which causes autoreactive B cells to undergo class switching specific for DNA nuclear antigens and release of inflammatory cytokines

Question 4

What are the susceptibility loci conferring the highest risk for SLE?

Answer 4

• genes encoding early complement components (C1,2,3)
• TREX1
• ITGAM

Question 5

What are the environmental triggers for SLE?

Answer 5

• sunlight

- cigarettes

- infections

• Vitamin D deficiency
• estrogen

Question 6

What are the SLE diagnostic criteria?

Answer 6

• Need four items (at least one clinical and one immunological) Or biopsy proven nephrities compatible w/ SLE in presence of positive ANA or anti-ds-DNA antibodies

Question 7

SLE patients with _____ are at increased risk of complications.

Answer 7

lupus nephritis

Question 8

What are possible fetal compliocations with pregnant SLE patient?

Answer 8

• preterm birth
• preeclampsia
• fetal loss

Question 9

Anticardiolipin antibodies in pregnant patient with SLE associated with_______

Answer 9

- increased risk of fetal loss

Question 10

Treatment for pregnant patient with SLE?

Answer 10

• continue Hydroxychloroquine and low dose steroids
• can consider azathioprine
• anticoagulation for APLS

Question 11

What complement abnormalities do you see in SLE?

Answer 11

• decreased total complement levels

Question 12

Autoantibodies against C1q in SLE patient is a/w_____

Answer 12

• severe SLE nephritis

Question 13

What routine labs are significant in SLE?

Answer 13

• increased inflam markers (ESR CRP)
• hemolytic anemai (coombs positive)
• leukopenia or lymphopenia
• thrombocytopenia
• proteinuria
• hematuria

Question 14

what percent of SLE have a positive ANA?

Answer 14

• 99%, very sensitive (good screening)

Question 15

What are the highly specific antibodies for SLE?

Answer 15

• Anti-ds DNA and Anti-smith

Question 16

Which antibodies are a/w neuropsychiatric SLE?

Answer 16

• Anti-RNP

Question 17

Drug induced lupus antibody?

Answer 17

Anti-histone

Question 18

What are the three antiphospholipid antibodies?

Answer 18

1. anti-Beta 2 glycoprotein antibody

2. anti-cardiolipin

3. lupus anticoagulant

Question 19

Treatment for mild SLE (no life threatenting visceral organ involvement)?

Answer 19

• hydroxychloroquine and NSAIDS

Question 20

Treatment for moderate-severe SLE lacking renal involvment?

Answer 20

• prednisone + steroid sparing agent ( azathioprine, MTX, MMF)

Question 21

Treatment for severe active SLE w/ renal involvement?

Answer 21

high dose prednisone + pulsed IV cyclophosphamide or MMF

Question 22

Treatment options for moderate-severe recalcitrant SLE?

Answer 22

• Rituximab

- Belimumab

Question 23

Prognosis of SLE with childhood onset?

Answer 23

• higher risk of lupus nephritis and mortality

Question 24

most common causes of death in SLE in first 5 years? After 5 years?

Answer 24

• in first 5 years= inflammatory lesions of SLE and infection
• after that, arterial (MI), venous thromboses