Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with protean manifestations. In pediatric patients, complications can evolve rapidly and carry significant morbidity and mortality. Emergency medicine providers should maintain a high index of suspicion for these entities in children and adolescents with known or suspected SLE. Below is a focused overview of key lupus-related emergencies, with attention to ED-relevant presentations, diagnostics, and treatment strategies.
Renal Disease (Lupus Nephritis)
Signs & Symptoms: Children may present with new-onset hypertension, edema, or decreased urine output. ED referrals are often prompted by incidental findings of proteinuria or hematuria, or clinical concerns for hypertensive encephalopathy or PRES.
Laboratory Findings:
- Hypoalbuminemia
- Metabolic acidosis
- Anti-dsDNA antibody positive
Treatment:
- Induction: Corticosteroids + cyclophosphamide or mycophenolate mofetil
- Maintenance: Azathioprine or mycophenolate
- All patients: Hydroxychloroquine unless contraindicated
Pearls:
- Urinalysis with microscopy remains the cornerstone of initial evaluation.
- Spot urine protein:creatinine ratio provides rapid assessment of proteinuria.
- Kidney biopsy is essential for staging and guiding treatment in persistent proteinuria or renal insufficiency.
Hemolytic Anemia and Thrombocytopenia
Signs & Symptoms: Common presentations include fatigue, pallor, petechiae, mucosal bleeding, or abnormal labs found during fever workup. Cross-matching may be difficult due to autoantibodies.
Laboratory Findings:
- Elevated LDH
- Low haptoglobin
- Positive Coombs test
- Cytopenias
Treatment:
- First-line: High-dose corticosteroids
- Adjunct: IVIg for thrombocytopenia
- Refractory: Rituximab
Pearls:
- Evans syndrome or isolated ITP may precede SLE diagnosis.
- Avoid transfusion unless clinically necessary.
Macrophage Activation Syndrome (MAS)
Signs & Symptoms: MAS may mimic sepsis or malignancy. Presenting features include persistent fever, hepatosplenomegaly, and altered mental status. Rapid clinical deterioration is common.
Laboratory Findings:
- Hyperferritinemia
- Cytopenias
- Elevated liver enzymes and triglycerides
- Decreased ESR with rising CRP
Treatment:
- High-dose corticosteroids
- Escalation: Cyclosporine or anakinra for refractory cases
Pearls:
- Early recognition is critical—delays increase mortality.
- Always evaluate for infectious triggers.
Pulmonary Embolism and Venous Thromboembolism
Signs & Symptoms: Acute dyspnea, pleuritic chest pain, or hypoxia. DVT symptoms or syncope may precede PE diagnosis. Risk is elevated in the context of nephrotic syndrome or APL antibodies.
Laboratory Findings and Risk Factors:
- Hypocomplementemia
- Anti-dsDNA and APL antibodies
- Nephrotic-range proteinuria
Treatment:
- Anticoagulation: LMWH, transitioning to oral agents
- Lifelong anticoagulation may be indicated with persistent APL antibodies
Pearls:
- Consider CAPS in cases with thrombosis and multi-organ dysfunction.
Neuropsychiatric Involvement
Signs & Symptoms: Children may present with seizures, psychosis, mood lability, persistent headache, or focal deficits. Differential includes infection, medication toxicity, and primary psychiatric illness.
Laboratory Findings and Risk Factors:
- Positive anti-neuronal and anti-ribosomal antibodies
Treatment:
- Severe disease: High-dose corticosteroids + cyclophosphamide
- Refractory: Rituximab
- Symptomatic management: Anticonvulsants, antipsychotics as indicated
Pearls:
- MRI, EEG, and CSF analysis are key diagnostic tools.
- Symptoms may precede formal SLE diagnosis.
Pulmonary Hemorrhage
Signs & Symptoms: Sudden onset hemoptysis and hypoxia with bilateral infiltrates. May be misdiagnosed as pneumonia or pulmonary edema.
Laboratory Findings:
- Drop in hemoglobin
- Thrombocytopenia
Treatment:
- High-dose corticosteroids
- Cyclophosphamide in severe/recurrent disease
- Supportive care, including respiratory support
Pearls:
- Strongly associated with lupus nephritis or APL syndrome.
- Requires prompt recognition and immunosuppression.
Cardiac Involvement – Pericarditis and Myocarditis
Signs & Symptoms: Chest pain, dyspnea, palpitations, or fatigue. Infants and toddlers may present with nonspecific signs such as irritability or feeding difficulty.
Laboratory and Diagnostic Findings:
- EKG: Diffuse ST elevations (pericarditis); ST depressions, T wave inversions, conduction delay (myocarditis)
- Elevated BNP and troponin
- Echo: Effusion, wall motion abnormalities
Treatment:
- NSAIDs for mild pericarditis
- Corticosteroids for myocarditis or tamponade physiology
- IVIG or immunosuppressants in refractory myocarditis
Pearls:
- Cardiac MRI aids in myocarditis diagnosis.
- Myocarditis can be rapidly progressive and fatal.
Final Thoughts
Recognition of SLE-related emergencies in the ED requires familiarity with atypical presentations and thoughtful use of diagnostics. Many complications mimic other pathologies such as sepsis, malignancy, or psychiatric disease. A multidisciplinary approach involving rheumatology, nephrology, critical care, and hematology is often essential for optimal care. Education, psychosocial support, and long-term follow-up remain critical in improving outcomes for children with cSLE.
References
Brunner HI, Huggins J, Klein-Gitelman MS. Pediatric SLE–towards a Comprehensive Management Plan. Nature Reviews. Rheumatology. 2011;7(4):225-33. doi:10.1038/nrrheum.2011.15. PMID: 21386795.
Gültekingil Keser A, Ertuğrul İ, Tekşam Ö, et al. Unusual Presentations of Childhood Systemic Lupus Erythematosus to the Emergency Department. Pediatric Emergency Care. 2017;33(10):e100-e102. doi:10.1097/PEC.0000000000000829. PMID: 27977505.
Campbell EJ, Clarke AE, Ramsey-Goldman R. Systemic Lupus Erythematosus: A Case-Based Presentation of Renal, Neurologic, and Hematologic Emergencies. Expert Review of Clinical Immunology. 2018;14(10):803-816. doi:10.1080/1744666X.2018.1518132. PMID: 30173578.
Share your thoughts