Systemic Lupus Erythematosus (SLE)

Question 1

What is SLE?

Answer 1

Autoimmune condition with numerous autoantibodies that causes diffuse inflammation throughout the body and blood

Question 2

What are some autoantibodies associated with SLE?

Answer 2

Antinuclear Ab (ANA)
Anti-double-stranded DNA (anti-dsDNA)

Question 3

What type of cancer risk is increased in SLE?

Answer 3

Lymphoma

Question 4

What is diagnosis of SLE based on?

Answer 4

Presence of at least 4 of 11 known manifestations

Question 5

What are the 11 known manifestations of SLE?

Answer 5

Malar Rash
Discoid Rash
Photosensitivity
Oral Ulcers
Arthritis
Serositis
Renal Disorder
Neurologic Disorder
Hemolytic Anemia
ANA (+)
Immunologic disorder

Question 6

What is serositis?

Answer 6

Inflammation of the pleura and pericardium creating chest pain with possible pericardial and pleural effusion

Question 7

What are the Immunologic Disorder in SLE?

Answer 7

Anti-dsDNA
Anti-Sm
False (+) test for syphilis
(+) LE Cell Proliferation

Question 8

What antibody is most specific for SLE?

Answer 8

Anti-dsDNA (60%) and anti-Sm (30%)

Question 9

What lab value correlates with disease activity in SLE?

Answer 9

Decreased C’ level:drops during exacerbations

Question 10

What disease is most often (+) for Anti-SSA (anti-Ro) and anti-SSB (anti-La)?

Answer 10

Sjogren Syndrome

Question 11

What must you check if a mother is anti-SSA (anti-ro) +?

Answer 11

Fetal EKG due to risk of heart block

Question 12

How do you treat an acute lupus flare?

Answer 12

High-dose bolus of steroids

Question 13

How do you treat mildly chronic lupus?

Answer 13

Hydroxychloroquine if limited to skin and joints

Question 14

How do you treat Lupus Nephritis?

Answer 14

Steroids +/- mycophenolate or cyclophosphamide

Belimumab controls progression

Question 15

What is the most common cause of death in Lupus in younger patients?

Answer 15

Infection

Question 16

What is Antiphospholipid Syndrome (APS)?

Answer 16

Idiopathic disorder with IgG or IgM Ab against (-) charged phospholipids

Question 17

What is the most common cause of death in lupus in older patients?

Answer 17

Accelerated Atheroscleorosis leading to MI

Question 18

What are commonly target phospholipids in APS?

Answer 18

Lupus Anticoagulant
Anticardiolipin Ab
Beta-2 Glycoprotein

Question 19

How does APS present?

Answer 19

Thromboses of arteries and veins with recurrent spontaneous abortion

Question 20

What lab values are commonly seen in APS?

Answer 20

elevated aPTT, normal PT, normal INR

False (+) VDRL or FPR with normal FTA

Question 21

What is the best initial diagnostic test for APS?

Answer 21

Mixing study: the aPTT will remain elevated (prolonged)

Question 22

What is the most specific test for Lupus anticoagulant?

Answer 22

Russel Viper Venom Test (RVVT): prolonged with APS and does not correct on mixing

Question 23

The use of what drug interferes with the RVVT?

Answer 23

Heparin

Question 24

How do you treat APS?

Answer 24

Heparin then warfarin for DVT/PE lifelong

no treatment if asymptomatic

Question 25

When should you investigate for Anticardiolipin Ab as a cause of spontaneous abortion?

Answer 25

2 or more first-trimester events or a single 2nd trimester event