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Systemic Lupus Erythematosus Flashcards

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
Q

How long does belimumab take to work?

A

2-4 months

26
Q

What are ADEs of belimumab?

A
  • Depression/suicidality/CNS side effects
  • PML
  • Serious infection with live vaccines and other biologics
  • N/V, allergic reaction, infusion reaction
27
Q

Should we use belimumab in pregnancy/breastfeeding?

28
Q

What is the MOA of belimumab?

A

B lymphocyte stimulator antagonist

29
Q

What is the MOA of anifrolumab?

A

Interferon antagonist

30
Q

When should we avoid anifrolumab?

A

Active LN or CNS disease

31
Q

What benefits does anifrolumab have?

A

Symptomatic relief and stabilization of organ disease (ADJUNCT ONLY)

32
Q

When should we use immunosuppresants?

A
  • Failed HCQ/NSAID/steroid for symptoms
  • Organ-threatening SLE (mainly LN)
    Often used with steroids
33
Q

What immunosuppressants can we use for SLE?

A

Azathioprine, cyclophosphamide, cyclosporine, methotrexate, mycophenolate

34
Q

When is methotrexate indicated?

A
  • Primary presentation of arthritis
  • Concomitant RA
35
Q

When is azathioprine indicated?

A

Second-line after steroids for moderate disease course

36
Q

What is the safest immunosuppressant to use during pregnancy?

A

Azathioprine

37
Q

When is mycophenolate indicated?

A
  • Proliferative (class 3 and 4) LN
  • Second line for membranous (class 5) LN
    (Better efficacy than cyclophosphamide in these situations)
38
Q

When should we avoid MMF?

A
  • Neuropsychiatric disease
  • Pregnancy
39
Q

Why is cyclophosphamide no longer the gold standard immunosuppressant for LN?

A

INCREDIBLY TOXIC
hematologic, cardiac, neurologic, permanent infertility

40
Q

When can we use cyclosporine?

A

Membranous LN (class 5)
slightly less toxic than cyclophosphamide?

PROBABLY NEVER

41
Q

When can we use rituximab?

A

Last line, off-label for severe renal, hematologic, or neuropsychiatric SLE refractory to other agents

42
Q

When can we use calcineurin inhibitors (tacrolimus)?

A

Proliferative (class 5) LN +/- MMF

43
Q

What is voclosporin?

A

Oral calcineurin inhibitor used as adjunct with other immunosuppressants for active LN (NOT cyclophosphamide)

Probably not used…

44
Q

What are ADEs of voclosporin?

A
  • BBW for infections and malignancies
  • Avoid in eGFR < 45 mL/min/1.73m^2
  • CYP3A4 interactions
45
Q

What are non-pharm treatments for SLE?

A
  • Avoid triggers (sun, smoke, stress)
  • Treat/prevent infections (vaccines)
46
Q

What should be used first-line for skin-disease in SLE?

A
  • Topical steroids
  • Hydroxychloroquine
    (consider moving to systemic steroids)
47
Q

What should we use for refractory/severe SLE after maximizing steroids?

A

Probably MMF or AZA

48
Q

What can we use after failing immunosuppressants?

A

Belimumab, anifrolumab

49
Q

A patient presents with new LN. What should we do?

A

Induction therapy: MMF or CYC PLUS steroid

Once we hit remission, taper steroid and switch immunosuppressant(s) as needed

50
Q

Patient with class 3 or 4 LN failed steroids + MMF… what should we do?

A

Consider adding on tacrolimus or using rituximab

51
Q

What should we use ACEi/ARBs in LN?

A
  • Persistent proteinuria (>0.5 g/24h)
  • HTN (>130/80 mmHg)
    All class 5 LN cases
52
Q

When is a steroid + MMF required in class 5 LN?

A

Urine protein >3g in 24

(can still be used <3g)

53
Q

When should we use statin therapy in LN?

A

LDL >100 mg/dL

54
Q

Which SLE drugs can be used in pregnancy?

A
  • HCQ
  • APAP (instead of NSAIDs for pain)
  • Topical steroids
  • AZA (continue if already on it)
55
Q

How should LN be treated in pregnancy?

A

HCQ/AZA
- prednisone for clinically active LN

56
Q

What can be considered for highly active LN in pregnancy?

A

Preterm delivery (>28 weeks)

57
Q

A patient is aPL (+) but has not had a thrombotic event. What should we use?

A

Aspirin 81 mg QD

58
Q

A patient is pregnant and is aPL (+) but has not had a thrombotic event. What should we use?

A

Aspirin 81 mg QD +/- LMWH (enoxaparin)

59
Q

A patient is pregnant and is aPL (+) and HAS had a thrombotic event (APS). What should we use?

A

Aspirin 81 mg QD + LMWH (enoxaparin)

60
Q

A patient is aPL (+) and had a venous thrombosis. What should we use?

A

Warfarin target INR 2-3

61
Q

A patient is aPL (+) and had a arterial thrombosis. What should we use?

A

Warfarin target INR 3-4

62
Q

When should we follow up for s/s at office visits?

A

Every 3-6 months
- UA, BMP, CBC, lipids, serological disease markers

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