Written by Clay Smith
Spoon Feed
Systemic lupus erythematosus is an autoimmune disorder with several manifestations that are tricky to diagnose and several disease manifestations that may lead to emergency care.
Why does this matter?
SLE is common. You will see these patients in the ED. Here are the common dangerous presentations and the medications and side effects you need to know.
Lupus – late 16th century: from Latin, literally ‘wolf’
Clinical Manifestations
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The most common are in the table. The name lupus comes from the malar rash, which apparently made the face appear “wolf-like” to some tortured soul.
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Any combination of 4 in the table is adequate for diagnosis.
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African-American women are disproportionately affected.
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Common and concerning ED presentations include: severe hematologic manifestations, shortness of breath, PE, chest pain, neurologic or neuropsychiatric manifestations, rapid decline in renal function, or fever.
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Notable severe manifestations of SLE: lupus nephritis, alveolar hemorrhage, or CNS vasculitis all need treatment with high-dose steroids and immunosuppressive medications.
Pregnancy
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Flares may occur during pregnancy.
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DVT/PE risk is greater.
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Preeclampsia and HELLP are more common.
Treatment
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Glucocorticoids, antimalarial agents, non-steroidal anti-inflammatory drugs, immunosuppressive agents, and B cell–targeting biologics are the mainstays.
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Hydroxychloroquine is the most commonly used drug.
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Common medications are in the table. I needed to remind myself of the adverse effects of most of these drugs and found this column helpful. I need to point out three things.
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Beware azathioprine (6-MP) and allopurinol together. This can cause profound myelosuppression. Xanthine oxidase helps break down 6-MP and is inhibited by allopurinol.
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Allopurinol can also increase cyclosporine levels.
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Know the tacrolimus-hyperkalemia association. Imagine a board question about drugs that increase potassium that lists spironolactone, lisinopril, tacrolimus, and mycophenylate. It’s not mycophenylate (…another point for you!).
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Fever may indicate opportunistic infection and should usually receive broad-spectrum antibiotic coverage.
Disposition
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Most SLE management is outpatient and is out-of-sight, out-of-mind for us in the ED.
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Hospitalization is usually needed in patients with severe thrombocytopenia, rapidly progressive kidney disease, pneumonitis or pulmonary hemorrhage, severe cardiac or neurological disease manifestations, or fever.
Source
Systemic Lupus Erythematosus. Ann Intern Med. 2020 Jun 2;172(11):ITC81-ITC96. doi: 10.7326/AITC202006020.
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