Lupus (SLE) Flashcards
SLE
Chronic autoimmune disease with a diverse clinical presentation that causes the immune system to attack healthy tissues and organs throughout the body
Epidemiology
Sex: Female>male
Onset: 15-45
Ethnicity: more prevalent in Asian, African, Arab, Hispanic, American Indians
Pathophysiology
Genetics
Hormonal: estrogen can increase incidence
Environment: smoking, pollution, pesticides, UV exposure, psychological stress, EBV, medications
Drug-induced Lupus Erythematosus (DILE)
Overreaction to certain medications
Symptoms occur 3-6 months of drug initiation
Resolution occurs within weeks of drug discontinuation
Around 10% of SLE cases are drug-induced and 46 medications that can cause DILE
DILE examples
My Pretty Malar Marking Probably Has A Transient Quality
Methimazole
Propylthiouracil
Methyldopa
Minocycline
Procainamide
Hydralazine
Anti-TNF
Terbinafine
Isoniazid
Quinidine
Signs/symptoms
Fatigue
Depression
Photosensitivity
Joint pain
N/V
Fever
Weight loss
Butterfly rash
Other signs and symptoms
Discoid rash, raynauds, oral/nasal ulcers
Lupus retinopathy
Lupus nephritis
Diagnostic tools
SLICC: Must meet > 4 total features with 1 from each group OR biopsy proven lupus with + Anti-dsDNA or + ANA
EULAR: Patients score is > 10 AND at least 1 clinical criterion is fulfilled
Key labs
ANA: positive in lupus patients but not specific
Anti-dsDNA: highly specific for diagnosis, important marker in lupus nephritis
Anti-SM: highly specific
Antiphospholipid antibody: increase clotting factors
Hydroxychloroquine
Antimalarial–>inhibit overactive immune cells
Recommended for ALL lupus patients
Reduces flares and manages pain
Dose: 200-400 mg PO daily
Hydroxychloroquine side effects
Retinal toxicity
Anxiety, depression, insomnia
Hypersensitivity reactions
Hypoglycemia, hemolytic anemia (G6PD)
Monitoring parameters
Baseline: CBC, LFT, SCr, ECG
Vision exam 3 months after starting therapy, then annually
NSAIDS
Considered 1st line for mild symptoms
Ibuprofen: 400-600 mg PO Q6-8H
Naproxen: 500 mg PO BID
NSAIDS monitoring
CBC
SCr
LFTs
BP
S/sx for bleeding and fluid retention
Glucocorticoids
Anti-inflammatory and helpful during flares
Adjunctive treatment, if not responsive to NSAIDS/HCQ
Can use all three together
Oral prednisone
Mild-moderate disease: 5-30 mg/day
Severe: 1 mg/kg/day
IV methylprednisolone
500-1000 mg IV daily x 3-6 days, then PO prednisone
Topical glucocorticoids
cutaneous lupus
Low-potency: Fluocinolone valerate and Hydrocortisone butyrate (Face)
Moderate-potency: Triamcinolone acetonide and Betamethasone valerate (Trunk and extremities)
High potency: Clobetasol (Scalp sores and palms)
Glucocorticoids monitoring parameters
BP
BMP: baseline then q6 months
FLP: baseline then q6 months
Bone mineral density
Immunosuppressants
Adjunct to steroid therapy to lower the dose
Insufficient response to HCQ
Methotrexate
5-15 mg weekly
SE: Bone marrow suppression, infection
Mycophenolate mofetil (MMF)
1-1.5 g twice daily
SE: Bone marrow suppression, infection, malignancy, AIS
Cyclophosphamide
1-1.5 mg/kg once daily
SE: Bone marrow suppression, infection malignancy
Azathioprine
50 mg daily
SE: Bone marrow suppression, infection malignancy
Monitor: TPMT
Biologics
Inadequate response to antimalarial and immunosuppressants
Severe disease
Belimumab
10 mg/kg every 2 weeks x 3 doses
SE: hypersensitivity, infusion reactions
Anifrolumab
300 mg every 4 weeks
SE: hypersensitivity reactions
Rituximab
1 g on days 0 and 15 or 375 mg/m2 once weekly for 4 doses
SE: infusion reactions, Hep B reactivation
Pre-medicate 30 minutes prior (methylprednisolone)
Calcineurin Inhibitors
Tacrolimus
Pimecrolimus
Voclosporin
Cutaneous lupus treatment
First line:
Topical agents:
GC: Clobetasol, betamethasone, triamcinolone, hydrocortisone
CNI: Tacrolimus, pimecrolimus
HCQ
Systemic GC
Refractory: High dose GC, MTX, MMF
Lupus nephritis treatment
Mild-moderate (Class 1 or 2 LN): GC +/- another immunosuppressant (AZA, MMF, CNI)
Severe nephritis (Class 3 or 4 LN): MMF (preferred) or Cyclophosphamide +/- GC
Triple therapy: Belimumab + MMF or CYC +/- GC
CNI + MMF +/- GC
Medications that are safe in pregnancy
HCQ
NSAIDS
GC
Antiphospholipid antibody
An autoimmune disorder characterized by antiphospholipid antibody that can cause blood clots and miscarriages
Prophylaxis:
No prior fetal loss: ASA 81 mg
Prior fetal loss: ASA 81 mg +/- LMWH
