Systemic Lupus Erythematosus Flashcards

1
Q

What is SLE?

A

chronic autoimmune disease with diverse clinical presentation
immune system attacks healthy tissues and organs throughout the body

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2
Q

Epidemiology

A

more ocmmon in women and in women of childbearnig age - peak incidence 15-45 yrs; more prevalent in non-whites

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3
Q

Etiology

A

exact etiology is unknown, but many pre-disposing factors

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4
Q

Pre-disposing factors

A

genetics: 1st degree relatives more likelt to develop SLE, or identical/fraternal twins
hormonal: estrogen production may modulate the incidence and severity of SLE
environment: cigaretter smoking, meds, UV light, air pollution, viruses (EBV), psychological stress, pesticides

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5
Q

Drug-induced lupus erythematosus

A

overreaction to certain meds; sx occur 3-6mo of drug initiation; resolution occurs within weeks of drug discontinuation
~10% of SLE cases are drug-induced

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6
Q

DILE examples

A

My Pretty Mala Marking Probably Has A Transient Quality
methimazole, propylthiouracil, methyldopa, minocycline, procainamide, hydralazine, anti-TNF agents (infliximab, etanercept), terbinafine, isoniazid, quinidine

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7
Q

Signs/symptoms

A

fatigue, depression, photosensitivity, joint pain, N/V, fever, weight loss, malar “butterfly” rash, discoid rash, raynaud phenomenon, lupus retinopathy, lupus nephritis

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8
Q

Raynaud phenomenon

A

exagerrated vascular response to cold temperature and emotional stress
pallor: constriction of blood flow, white; cyanosis: tissue hypoxia, blue; rubor: reperfusion, red

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9
Q

Diagnostic tools

A

SLICC
EULAR/ACR

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10
Q

SLICC

A

must meet >/=4 total features with 1 from each group OR biopsy proven lupus nephritis WITH systemic lupus (+anti-dsDNA antibodies or +ANA)

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11
Q

EULAR

A

only the highest weighted criterion score within a single domain is used
patient’s score is >/= 10 AND at least 1 clinical criterion is fulfilled

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12
Q

Key labs

A

anti-nuclear antibody (ANA), anti-double-stranded DNA (anti-dsDNA), anti-smith antibody (anti-SM), and antiphospholipid antibody
these are all negative in a healthy pt and all positive in lupus pt
ANA not specific, anti-dsDNA and anti-SM are high specificity; antiphospholipid antibody increases clotting factors

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13
Q

Pharmacologic treatments

A

hydroxychloroquine, NSAIDs, glucocorticoids, immunosuppressants, biologics

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14
Q

Hydroxychloroquine

A

MOA: antimalarial - inhibits overative immune cells
for ALL pts with SLE
reduces flares + helps manage pain

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15
Q

Hydroxychloroquine dosing

A

200-400mg PO daily
max: 400mg daily

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16
Q

Hydroxychloroquine SEs

A

retinal toxicity (bull’s eye maculopathy), neuropsychiatric effects, QT prolongation, cardiomyopathy, hypersensitivity, hypoglycemia, hemolytic anemia (if G6PD deficiency)

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17
Q

G6PD deficiency

A

X-linked disorder (present more often in males) that causes RBCs to prematurely break down
G6PD deficient, won’t convert NADP+ to NADPH, which won’t make glutathione, which won’t fight free radicals that damage blood cells

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18
Q

Hydroxychloroquine monitoring

A

CBC, LFTs, SCr, EKG
eye exam

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19
Q

NSAIDs

A

MOA: inhibit COX1 and 2 to decrease the formation of prostaglandin precursor
benefits: antipyretic, anti-inflammatory, and analgesic - particularly helpful with fevers, serositis, myalgias, and arthralgias
1st line for mild sx

20
Q

NSAIDs dosing

A

ibuprofen: 400-600 mg PO q6-8h
naproxen: 500 mg PO BID

21
Q

NSAIDs SEs/monitoring

A

bleeding, gastritis, perforation, increased BP, worsening HF, CV events, increased SCr, renal toxicity, hepatotoxicity
monitor: CBC, LFTs, SCr, BP, s/sx of fluid retention and bleeding

22
Q

Glucocorticoids

A

MOA: inhibit B and T cell responses
benefits: anti-inflammatory and helpful during flares; variable dosage forms
adjunctive treatment if not responsive to NSAIDs/antimalarial

23
Q

Glucocorticoid dosing

A

oral: mild-mod disease: 5-30mg/day prednisone; severe: 1 mg/kg/day prednisone
IV: 500-1000 mg IV daily x 3-6 days of methylprednisolone, then PO prednisone

24
Q

Glucocorticoids dosing topical - low-potency

A

fluocinolone valerate and hydrocortisone butyrate (face)

25
Q

Glucocorticoids dosing topical - moderate-potency

A

triamcinolone acetonide and betamethasone valerate (trunk and extremeties)

26
Q

Glucocorticoids dosing topical - high-potency

A

clobetasol (scalp sores and palms)

27
Q

Glucocorticoids PO/IV SEs

A

glaucoma, increased BP, increased risk of osteoporosis, GI bleed, gastritis, psychosis/sleep disturbances, weight gain, increased BG, increased risk of infection, cushing syndrome

28
Q

Glucocorticoids topical SEs

A

skin atrophy, rosacea, telangiectasis

29
Q

Glucocorticoids monitoring

A

BP, BMP, FLP, bone mineral density

30
Q

Immunosuppresants

A

MOA: suppression of immune function from attacking healthy cells
adjunct to steroid therapy to lower the dose; used if insufficient response to HCQ
methotrexate, azathioprine, cyclophosphamide, mycophenolate mofetil

31
Q

Methotrexate dosing and SEs

A

5-15mg once WEEKLY
BMS, infection, only one with NO malignancy!

32
Q

Mycophenolate mofetil dosing and SEs

A

1-1.5g BID
BMS, infection, malignancy, AIS

33
Q

Cyclophosphamid dosing and SEs

A

1-1.5 mg/kg QD
BMS, infection, malignancy

34
Q

Azathioprine dosing and SEs

A

50 mg QD
BMS, infection, malignancy
monitor: TPMT deficiency

35
Q

If TPMT deficiency do not give

A

azathioprine
it will increase immunosuppressive effects because more 6-TGNs (active metabolites)

36
Q

Biologics - last line

A

MOA: monoclonal antibodies that block B-cell mediated immunity
inadequate response to antimalarial and immunosuppressants; for severe disease
belimumab, rituximab, anifrolumab

37
Q

Biologics clinical pearls

A

no live vaccines 30 days before starting therapy OR during therapy
do not use more than 1 biologic at the same time

38
Q

Belimumab dosing and SEs

A

10 mg/kg every 2 weks x 3 doses
hypersensitivity and or infusion reactions

39
Q

Anifrolumab dosing and SEs

A

300 mg every 4wks
hypersensitivity reactions

40
Q

Rituximab dosing and SEs

A

1g on days 0 and 15 or 375mg/m2 once weekly for 4 doses
infusion reactions, Hep B reactivation
premedicate ~30 min prior

41
Q

Additional therapies

A

calcineurin inhibitors: tacrolimus, pimecrolimus, voclosporin (1st FDA approved oral med for lupus nephritis)

42
Q

Nonpharmacologic treatment

A

balance of rest and exercise, smoking cessation, limit sun exposure and use of sunscreen

43
Q

Cutaneous lupus

A

presents on skin with rash and lesions
first line: topical agents - clobetasol, betamethasone, triamcinolone, hydrocortisone; tacrolimus, primecrolimus; HCQ; systemic GC
refractory: high dose GC, MTX, mycophenolate mofetil

44
Q

Lupus nephritis

A

mild-mod nephritis: GC+/- another immunosuppressant (AZA, MMF, CNI)
severe nephritis: MMF (preferred) or cyclophosmadie +/- GC; triple therapy - belimumab + MMF or cyclophosphamide +/- GC or CNI + MMF +/- GC

45
Q

Pregnancy and SLE

A

higher materal and fetal risk: miscarriage, fetal growth retardation, maternal mortality, preeclampsia
best prognosis is when pt achieves remission for >/= 6 mo before pregnancy - pregnancy can cause flares
contraception: avoid estrogen-containing contraception, screen for antiphospholipid syndrome (could increase clot risk)

46
Q

Medications in pregnancy

A

hydroxychloroquine (drug of choice)
NSAIDs: d/c at 20 weeks or later b/c of premature closing of ductus ateriosus
glucocorticoids: choose lowest dose for shortest duration

47
Q

Antiphospholipid antibody

A

autoimmune disorder characterized by antiphospholipid syndrome that can cause blood clots and miscarriages
prophylaxis: no prior fetal loss - aspirin 81mg, recurrent fetal loss - aspirin 81mg +/- LMWH
acute thrombotic event/hx of thrombosis: LMWH
NO warfarin!