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Systemic Lupus Erythematosus Flashcards

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1
Q

SLE can affect all organ systems while DLE primarily affects…

A

Skin (discoid rashes)

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2
Q

Which groups are more at risk for SLE?

A
  • Women during childbearing years
  • Black and Hispanic people
  • Those with a first degree relative with SLE
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3
Q

What environmental factors can trigger SLE?

A
  • Sunlight (UV)*****
  • Stress
  • Smoking
  • Medications (over 100)
  • Viruses or virus-like elements
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4
Q

What hormones can trigger SLE?

A

Estrogen and prolactin

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5
Q

T/F: Breastfeeding decreases risk of SLE

A

TRUE

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6
Q

What does the presentation of SLE look like?

A

Fever, arthralgias, in a woman of childbearing age

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7
Q

What is the ACR diagnostic criteria acronym?

A

4/11:
SOAP
BRAIN
MD

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8
Q

What does the SOAP part of the ACR diagnostic criteria stand for?

A
  • Serositis (pain/rub/effusion)
  • Oral ulcers (typically painless)
  • Arthritis* (red, swollen, tender)
  • Photosensitivity (skin rash from sun)
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9
Q

What does the BRAIN part of the ACR diagnostic criteria stand for?

A
  • Blood disorders (anemia/leuko/lymph/thrombocytopenia)
  • Renal involvement (persistent proteinuria or cellular casts)
  • Antinuclear Ab*
  • Immunologic disorder (anti-DNA Ab, anticardiolipin, lupus anticoagulant)
  • Neurologic disorder (seizures, psychosis)
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10
Q

What does the MD part of the ACR diagnostic criteria stand for?

A
  • Malar rash
  • Discoid rash
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11
Q

Which labs are indicative of aPL (+)?

A

Anticardiolipin, lupus anticoagulant (hypercoagulable)

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12
Q

What is Secondary APS?

A

Thrombotic event in aPL (+) patients (happens 50-70% of the time)

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13
Q

Why is APS dangerous?

A

Associated with DVT, stroke, neurologic manifestations, pregnancy complications

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14
Q

What diagnostic criteria is required for LN?

A
  • Persistent proteinuria and/or cellular casts
  • Renal biopsy and histology to confirm
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15
Q

What are symptoms of LN?

A
  • Foamy urine
  • Peripheral edema
  • Concomitant HTN
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16
Q

What steroid should be used for the face initially?

A

Hydrocortisone 1% for the shortest amount of time possible

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17
Q

What should all SLE and DLE patients be on unless they have a true contraindication?

A

Hydroxychloroquine
400mg QD-BID for suppression
200-400 mg QD for maintenance

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18
Q

How long does it take HCQ to work?

A

2-4 months (adequate trial is 6 months)

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19
Q

What can be used to treat symptoms while other medications are taking time to kick in?

A

NSAIDs

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20
Q

T/F: HCQ should be discontinued during pregnancy

A

FALSE: it should be continued

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21
Q

What are the major ADEs to look out for for HCQ?

A
  • Flu-like symptoms
  • Ocular-toxicity*** (eye exam at baseline, 5 years, then annually)
  • Allergic skin eruptions
  • Hair and skin changes
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22
Q

When should steroids be used?

A

(ALWAYS ADJUNCT)
- Moderate-severe initial presentation
- Organ-threatening or life-threatening SLE
- Inadequate response to HCQ or NSAIDs
- Poor quality of life without

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23
Q

How are steroids used for rapid relief of symptoms?

A

Oral dose (prednisone 20-60 mg/day) or IV pulse followed by oral taper
(limit exposure to prevent long term effects)

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24
Q

What patients can use belimumab as adjunct therapy?

A
  • Non-active-CNS, antibody-positive SLE
  • Musculoskeletal or cutaneous disease unresponsive to HCQ/NSAID/steroid
  • LN stage 3, 4, 5
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25
How long does belimumab take to work?
2-4 months
26
What are ADEs of belimumab?
- Depression/suicidality/CNS side effects - PML - Serious infection with live vaccines and other biologics - N/V, allergic reaction, infusion reaction
27
Should we use belimumab in pregnancy/breastfeeding?
NO
28
What is the MOA of belimumab?
B lymphocyte stimulator antagonist
29
What is the MOA of anifrolumab?
Interferon antagonist
30
When should we avoid anifrolumab?
Active LN or CNS disease
31
What benefits does anifrolumab have?
Symptomatic relief and stabilization of organ disease (ADJUNCT ONLY)
32
When should we use immunosuppresants?
- Failed HCQ/NSAID/steroid for symptoms - Organ-threatening SLE (mainly LN) Often used with steroids
33
What immunosuppressants can we use for SLE?
Azathioprine, cyclophosphamide, cyclosporine, methotrexate, mycophenolate
34
When is methotrexate indicated?
- Primary presentation of arthritis - Concomitant RA
35
When is azathioprine indicated?
Second-line after steroids for moderate disease course
36
What is the safest immunosuppressant to use during pregnancy?
Azathioprine
37
When is mycophenolate indicated?
- Proliferative (class 3 and 4) LN - Second line for membranous (class 5) LN (Better efficacy than cyclophosphamide in these situations)
38
When should we avoid MMF?
- Neuropsychiatric disease - Pregnancy
39
Why is cyclophosphamide no longer the gold standard immunosuppressant for LN?
INCREDIBLY TOXIC hematologic, cardiac, neurologic, permanent infertility
40
When can we use cyclosporine?
Membranous LN (class 5) slightly less toxic than cyclophosphamide? PROBABLY NEVER
41
When can we use rituximab?
Last line, off-label for severe renal, hematologic, or neuropsychiatric SLE refractory to other agents
42
When can we use calcineurin inhibitors (tacrolimus)?
Proliferative (class 5) LN +/- MMF
43
What is voclosporin?
Oral calcineurin inhibitor used as adjunct with other immunosuppressants for active LN (NOT cyclophosphamide) Probably not used...
44
What are ADEs of voclosporin?
- BBW for infections and malignancies - Avoid in eGFR < 45 mL/min/1.73m^2 - CYP3A4 interactions
45
What are non-pharm treatments for SLE?
- Avoid triggers (sun, smoke, stress) - Treat/prevent infections (vaccines)
46
What should be used first-line for skin-disease in SLE?
- Topical steroids - Hydroxychloroquine (consider moving to systemic steroids)
47
What should we use for refractory/severe SLE after maximizing steroids?
Probably MMF or AZA
48
What can we use after failing immunosuppressants?
Belimumab, anifrolumab
49
A patient presents with new LN. What should we do?
Induction therapy: MMF or CYC PLUS steroid Once we hit remission, taper steroid and switch immunosuppressant(s) as needed
50
Patient with class 3 or 4 LN failed steroids + MMF... what should we do?
Consider adding on tacrolimus or using rituximab
51
What should we use ACEi/ARBs in LN?
- Persistent proteinuria (>0.5 g/24h) - HTN (>130/80 mmHg) All class 5 LN cases
52
When is a steroid + MMF required in class 5 LN?
Urine protein >3g in 24 (can still be used <3g)
53
When should we use statin therapy in LN?
LDL >100 mg/dL
54
Which SLE drugs can be used in pregnancy?
- HCQ - APAP (instead of NSAIDs for pain) - Topical steroids - AZA (continue if already on it)
55
How should LN be treated in pregnancy?
HCQ/AZA - prednisone for clinically active LN
56
What can be considered for highly active LN in pregnancy?
Preterm delivery (>28 weeks)
57
A patient is aPL (+) but has not had a thrombotic event. What should we use?
Aspirin 81 mg QD
58
A patient is pregnant and is aPL (+) but has not had a thrombotic event. What should we use?
Aspirin 81 mg QD +/- LMWH (enoxaparin)
59
A patient is pregnant and is aPL (+) and HAS had a thrombotic event (APS). What should we use?
Aspirin 81 mg QD + LMWH (enoxaparin)
60
A patient is aPL (+) and had a venous thrombosis. What should we use?
Warfarin target INR 2-3
61
A patient is aPL (+) and had a arterial thrombosis. What should we use?
Warfarin target INR 3-4
62
When should we follow up for s/s at office visits?
Every 3-6 months - UA, BMP, CBC, lipids, serological disease markers

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