Systemic Lupus Erythematosus (Mabry) (Exam 2)

chronic autoimmune disease that affects multiple organ systems

-female to male ratio up to 13:1

-predominantly occurs in people 15-44 years

-more common in black women

SLE

what are environmental risks for SLE? (6)

UV light

infections (ex: Epstein-Barr)

smoking

mercury

stress

silica

what are dermatologic clinical presentations of SLE? (3)

butterfly rash

photosensitivity

alopecia

what are musculoskeletal clinical presentations of SLE? (2)

arthralgia

myalgia

what are CNS clinical presentations of SLE? (4)

neuropsychiatric disease

seizure

psychosis

cognitive impairment

what are hematologic clinical presentations of SLE? (3)

hemolytic anemia

leukopenia

thrombocytopenia

what are other clinical presentations of SLE? (4)

oral or nasal ulcers

nephritis

fatigue

anti-phospholipid syndrome

what labs are used to diagnose SLE? (6)

(+) antinuclear antibodies (ANA)

(+) anti-single strand DNA (anti-ssDNA)

(+) anti-double strand DNA (anti-dsDNA)

(+) anti-Smith (anti-Sm)

(+) antiphospholipid antibodies

low complement (C3, C4, CH50)

diagnosis can be ___________ & may be ____________

difficult

delayed

what drugs can cause drug-induced lupus? (10)

My Pretty Malar Marking Probably Has A Transient Quality

methimazole

propylthiouracil

methyldopa

minocycline

procainamide

hydralazine

anti-TNF agents

terbinafine

isoniazid

quinidine

similar clinical and laboratory features to SLE

usually resolves within weeks after discontinuation of drug

drug-induced lupus

what are the two other types of lupus?

cutaneous lupus

neonatal lupus

what are treatment goals for lupus? (3)

maintain lowest degree of activity

prevent organ damage and minimize drug side effects

optimize health related quality of life

what are non-pharm education for lupus? (2)

avoid UV light & sun

-sunscreen

-protective clothing

smoking cessation

what are other care considerations for lupus? (4)

vaccinations

effective contraception and planned pregnancy

monitor for infections

routinely assess for CV disease, osteoporosis, renal disease, etc.

all lupus patients should be treated with what?

an antimalarial unless not tolerated or contraindicated

what antimalarial is used for lupus?

hydroxychloroquine

what should be considered based on type and severity of organ involvement?

glucocorticoids

consider adding what in patients not responding followed by what?

consider adding immunomodulating, immunosuppressive agents in patients not responding followed by biologics

recommended for all patients unless contraindicated

-increases survival, reduces flares, prevents organ damage

slow onset of action at 8-12 weeks; 6 months for full effect

hydroxychloroquine

what is dose for hydroxychloroquine? target dose?

200-400mg given once daily, or divided into two daily doses

target dose of 5 mg/kg/day (real body weight)

what are adverse effects of hydroxychloroquine? (4)

rash

diarrhea

abdominal cramps

adverse ocular effects

what drug has increased risk of retinal toxicity with a cumulative dose?

hydroxychloroquine

is hydroxychloroquine safe for pregnancy?

yes

limit to short courses for acute flares, patients starting or changing chronic medications, or for moderate to severe disease

glucocorticoids

what is the maintenance dose for prednisone?

<= 5 mg/day

examples of immunosuppressants (4)

methotrexate (MTX)

azathioprine (AZA)

mycophenolate mofetil (MMF)

cyclophosphamide (CYC)

used for all patients with an inadequate response to hydroxychloroquine (alone or in combination with corticosteroids), to reduce long-term corticosteroids, or in patients with severe disease

immunosuppressants

published evidence says MTX or AZA is stronger?

MTX

potent immunosuppressant with efficacy in renal and non-renal lupus

MMF

can be considered in organ-threatening disease (especially renal, cardiopulmonary, or neuropsychiatric)

CYC

do not give these with other biologic DMARDs or live vaccines

biologics

consider after hydroxychloroquine, corticosteroids, and immunosuppressants for patients unable to taper corticosteroids and/or who have frequent flares

-given IV every 4 weeks or weekly SQ

Belimumab (Benlysta)

where are severe ADEs for Belimumab (Benlysta)? (5)

hypersensitivity

malignancy

increased infection risk

psychiatric events

progressive multifocal leukoencephalopathy (PML)

what are common ADEs for Belimumab (Benlysta)? (5)

nausea

diarrhea

fever

depression

insomnia

consider after hydroxychloroquine, corticosteroids, and immunosuppressants for patients unable to taper corticosteroids and/or who have frequent flares

moderate to severe disease

given IV every 4 weeks

Anifrolumab (Saphnelo)

what are severe ADEs for Anifrolumab (Saphnelo)? (3)

hypersensitivity

malignancy

increased infection risk

non-TNF biologic, off label indication

considered only in patients with severe refractory disease or in those with contraindications or intolerance to immunosuppressants

Rituximab (Rituxan)