Exam 4 - Systemic Lupus Erythematosus

Question 1

What are potential risk factors for the development of SLE? (7)

Answer 1

ultraviolet light, stress, smoking, medications, viruses, hormones, genetics

Question 2

Explain the pre-clinical phase of SLE?

Answer 2

autoimmune proliferation as overactive B/T-cell activation and impaired tolerance and immune complex clearance (Type III hypersensitivity)

Question 3

How does SLE most commonly present? (3)

Answer 3

fever, arthralgias, and rash in a woman of childbearing age

Question 4

What are the ACR diagnostic criteria for SLE? (11)

Answer 4

serositis, oral ulcers, arthritis, photosensitivity, blood disorders, renal involve/impairment, antinuclear Ab, immunology, neurologic disorder, malar rash, discoid rash

Question 5

What are the serologic tests for SLE? (3)

Answer 5

antinuclear, anti-dsDNA, and anti-Sm (Smith proteins) Abs

Question 6

What constitutes antiphospholipid syndrome in SLE?

Answer 6

antiphospholipid antibody (aPL) positive and a thrombotic event

Question 7

What constitutes lupus nephritis? (2)

Answer 7

persistent proteinuria and/or cellular casts and renal biopsy and histology

Question 8

How does lupus NEPHRITIS commonly present (3)

Answer 8

foamy urine, peripheral edema, concomitant hypertension

Question 9

What are nDMARDs for SLE? (4)

Answer 9

topical corticosteroids, calcineurin inhibitors, acetaminophen, NSAIDs

Question 10

What is the DMARD for SLE?

Answer 10

hydroxychloroquine

Question 11

How long does hydroxychloroquine take to work?

Answer 11

2-4 months

Question 12

What are glucocorticoids reserved for in adjunctive treatment of SLE? (4)

Answer 12

moderate-severe initial presentation, organ/life-threatening SLE, inadequate response to hydroxychloroquine or NSAIDs, poor QoL without

Question 13

What is the MOA of belimumab (Benlysta)?

Answer 13

B-lymphocyte stimulator antagonist

Question 14

What is belimumab (Benlysta) reserved for in adjunctive treatment of SLE? (3)

Answer 14

non-active-CNS Ab+ SLE, unresponsive to hydroxychloroquine/NSAIDs/steroid, lupus nephritis III-V

Question 15

How long does belimumab (Benlysta) take to work?

Answer 15

2-4 months

Question 16

What are AEs of belimumab (Benlysta)? (5)

Answer 16

GI, hypersensitivity reactions, infusion reactions, suicidality, PML

Question 17

What is the MOA of anifrolumab (Saphnelo)?

Answer 17

interferon antagonist

Question 18

What are contraindications for anifrolumab (Saphnelo)? (2)

Answer 18

active lupus nephritis, CNS disease

Question 19

When is methotrexate indicated in SLE? (2)

Answer 19

concomitant RA or primary presentation of arthritis

Question 20

When is azathioprine indicated in SLE?

Answer 20

second-line (after steroids) for more moderate disease

Question 21

When is mycophenolate mofetil indicated in SLE? (2)

Answer 21

proliferative (II-IV) lupus nephritis, second-line for membranous (V) lupus nephritis

Question 22

Why is cyclophosphamide use in SLE controversial?

Answer 22

positive long-term evidence but incredibly toxic

Question 23

When is cyclosporine indicated in SLE?

Answer 23

membranous (V) lupus nephritis

Question 24

When are calcineurin inhibitors indicated in SLE?

Answer 24

membranous (V) lupus nephritis

Question 25

What is an off-label last-line treatment for SLE?

Answer 25

rituximab

Question 26

What are clinical pearls for voclosporin (Lupkynis)? (3)

Answer 26

boxed warning for infections and malignancies, eGFR <45 cutoff, 3A4 interactions

Question 27

What are first-line treatments for skin disease in SLE? (3)

Answer 27

topical steroids, calcineurin inhibitors, hydroxychloroquine

Question 28

What are treatments for refractory skin disease SLE? (7)

Answer 28

systemic steroids, methotrexate, mycophenolate, belimumab, anifrolumab, retinoids, dapsone

Question 29

What is initial treatment for lupus nephritis (III-IV)?

Answer 29

mycophenolate mofetil or cyclosporine plus steroid

Question 30

What is remission therapy for lupus nephritis?

Answer 30

tapered steroid

Question 31

What can be added to steroid + mycophenolate mofetil combos in lupus nephritis (III-IV) treatment?

Answer 31

tacrolimus

Question 32

When should lupus nephritis (III-V) treatment be reassessed?

Answer 32

3-12 months

Question 33

If lupus nephritis (III-IV) is responsive to treatment? If not?

Answer 33

mycophenolate mofetil or azathioprine with tapered steroid; alternative induction therapy or add tacrolimus or rituximab

Question 34

What is initial treatment for lupus nephritis (V) with a UPr <3?

Answer 34

RAAS blockade (ACEi/ARB)

Question 35

What are initial treatments for lupus nephritis (V) with a UPr >3? (2)

Answer 35

RAAS blockade (ACEi/ARB), mycophenolate mofetil plus steroid

Question 36

If lupus nephritis (V) is responsive to treatment? If not?

Answer 36

continue same treatment with tapered steroid; calcineurin inhibitor (monotherapy or add-on to mycophenolate mofetil) or cyclosporine or rituximab

Question 37

What are the indications for ACE-i/ARBs in SLE? (2)

Answer 37

persistent proteinuria (>0.5 g/24hr) and/or HTN

Question 38

What is the indication for statin therapy in SLE?

Answer 38

LDL >100 mg/dL

Question 39

Explain pregnancy with regards to SLE medications? (4)

Answer 39

hydroxychloroquine = good, NSAIDs = unsafe in 3rd trimester (use APAP), topical steroids must be low-potency and non-fluorinated, azathioprine is the only safe immunosuppresant

Question 40

What is the treatment for aPL+ and not pregnant? Pregnant?

Answer 40

low-dose aspirin; low-dose aspirin +/- low molecular weight heparin (enoxaparin)

Question 41

What is the treatment for APS and pregnant?

Answer 41

low-dose aspirin +/- low molecular weight heparin (enoxaparin)

Question 42

What is the treatment for APS with an arterial manifestation?

Answer 42

warfarin (INR 3-4)

Question 43

What is the treatment for APS with a venous manifestation?

Answer 43

warfarin (INR 2-3)

Question 44

What is treatment for lupus nephritis in pregnant patients?

Answer 44

hydroxychloroquine with azathioprine