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    Systemic Lupus Erythematosus (SLE)

    Cardiovascular, Ears, Eyes, Nose, and Throat, Gastrointestinal, Infections, Neurological, Respiratory

    Last Updated Jan 19, 2022
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    By Kerry Walker, Jonathan Lim


    Chronic autoimmune disease of unknown, multifactorial etiology. It presents a challenge to emergency providers due to its diverse and sometimes life-threatening manifestations.

    Affects between 20-70 per 100,000 individuals, with 90% of the cases in women, and a higher prevalence in Asian, African American, African Caribbean, and Hispanic American individuals compared with White individuals.

    Can cause manifestations in virtually any organ system through inappropriate immune response to self.

    Diagnostic Process

    Clinical Manifestations:

    Common Presentations

    Emergent Conditions


    Psychosis, mood disorders, peripheral neuropathy, headache

    Seizure, stroke, sinus thrombosis, CNS vasculitis, transverse myelitis, spinal artery thrombosis, lupus psychosis


    Pericarditis, valvular abnormalities

    Pericardial effusion, cardiac tamponade, acute coronary syndrome, Libman-Sacks endocarditis


    Pleuritis, interstitial lung disease, pulmonary hypertension

    Pulmonary embolism, pneumonia, acute lupus pneumonitis, alveolar hemorrhage


    Intestinal pseudo-obstruction

    Acute abdominal pain, mesenteric vasculitis


    Malar rash, discoid lupus, oral/nasal ulceration

    Systemic vasculitis, digital gangrene


    Polyarthralgias, polymyalgias, avascular necrosis

    Septic arthritis


    Mild-moderate anemia/leukopenia/thrombocytopenia, antiphospholipid syndrome (APS)

    Severe thrombocytopenia/APS, acute hemolytic anemia



    Lupus nephritis

    • Potential constitutional manifestations of SLE include fever, fatigue, weight loss. Infections are also a potential emergent complication of SLE, and providers should rule out opportunistic infections if the patient is immunocompromised, and sepsis depending on the clinical context.
    • Up to 40% of SLE patients may also present with manifestations of antiphospholipid syndrome (APS), which range from sequelae of thrombosis and thromboembolism to catastrophic APS, which presents with multiple thrombotic sites and organ failure.

    Physical Examination:

    • A complete and thorough examination in those who you suspect a diagnosis of SLE to be present is warranted.


    • CBC, urinalysis, creatinine, should be routinely obtained to assess for signs of increased disease activity or systemic inflammatory process.
    • In patients with suspected SLE, ANA, anti-dsDNA, anti-Smith antibody, antiphospholipid antibodies (lupus anticoagulant, IgG/IgM anticardiolipin antibodies, IgG/IgM anti-beta2-glycoprotein 1), C3/C4, ESR/CRP, and urine protein to creatinine ratios should be obtained. These can be ordered with outpatient follow-up organized for evaluation of the results.
    • In patients with known SLE and chest pain, ACS should be ruled out and cardiac workup is warranted including ECG, stress test and troponin.
    • Diagnostic imaging is not routinely obtained but can be valuable based on the symptoms.

    Diagnostic Considerations:

    • In the emergency department (ED), care providers will often see patients with SLE presenting with an exacerbation or complications from the disease or their medications.
    • However, patients with suspected, but no formal diagnosis of SLE may present to the ED.
    • Formally, a diagnosis is made using the 1997 ACR criteria or the 2012 SLICC criteria, but diagnosis in the ED is difficult to make given the broad manifestations of the disease and the time it takes for laboratory results to return.
    • A number of other diseases are often mistaken for SLE prior to diagnosis, including undifferentiated connective tissue disease, primary Sjögren’s syndrome, primary APS, fibromyalgia with positive antinuclear antibody, idiopathic thrombocytopenic purpura, drug-induced lupus, and early rheumatoid arthritis.

    Many medications are known to cause lupus-like autoimmune response and may be mistaken for SLE (see table below):

    Drugs associated with lupus-like autoimmune responses


    High risk (>5%)

    Moderate risk (1-5%)

    Low risk (0.1-1%)

    Procainamide, hydralazine


    Penicillamine, carbamazepine, methyldopa, sulfasalazine, minocycline, chlorpromazine, propylthiouracil, isoniazid

    Recommended Treatment

    • For non-infectious consequences of SLE, introduction or modulation of immunomodulating therapy is warranted.

    Image from Rosen’s Emergency Medicine 9th Edition

    • For infections in SLE patients who are on chronic steroid therapy, a stress dose of glucocorticoids (i.e. hydrocortisone 50-100 mg IV every 8 hours) + appropriate antimicrobial coverage should be initiated.
    • Many symptoms related to SLE are related to inflammation and are amenable to NSAIDs. In patients with CKD or peptic ulcer disease, where NSAIDs are contraindicated, alternatives include acetaminophen and narcotics.
    • For drug-induced lupus, cessation of the offending agent is therapeutic, and NSAIDs/steroids can be used for symptom control.
    • For acute thrombotic events, unfractionated or low-molecular-weight heparin is indicated. In catastrophic APS, in addition to glucocorticoids and anticoagulation, IVIG, plasma exchange, and cyclophosphamide can be considered.


    Criteria For Safe Discharge Home

    • Non-life-threatening presentation.
    • Appropriate follow-up as an outpatient is available.
    • If the patient had undifferentiated chest pain with ACS ruled out in the ED, they should have expedited evaluation with a stress test in an observation unit or outpatient basis. If stress test is done in an outpatient setting, daily aspirin should be prescribed.

    Criteria For Hospital Admission

    • Presentation of SLE flare, new thrombotic events, infection, or life-threatening presentation.

    Criteria for ICU admission:

    • Progressive circulatory or respiratory derangement in spite of initial resuscitation.

    Quality Of Evidence?


    Diagnosis of SLE using the 1997 ACR and 2012 SLICC criteria have high sensitivity and specificity – HIGH


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