What is the management of a patient with Systemic Lupus Erythematosus (SLE) and Systemic Sclerosis (SSc) experiencing a lupus flare in the emergency department?

From the Guidelines

Evaluation and management of a patient with scleroderma-lupus overlap syndrome experiencing a lupus flare in the emergency department should prioritize a systematic approach focusing on organ involvement assessment and prompt management, with initiation of hydroxychloroquine and minimization of glucocorticoids to less than 7.5 mg/day, as recommended by the 2019 EULAR update 1. When evaluating a patient with scleroderma-lupus overlap syndrome in the emergency department, it is crucial to begin with a thorough history and physical examination, paying particular attention to vital signs, skin manifestations, joint involvement, cardiopulmonary status, and neurological function. Key steps in the evaluation process include:

• Ordering laboratory tests such as complete blood count, comprehensive metabolic panel, urinalysis, ESR/CRP, complement levels (C3, C4), anti-dsDNA antibodies, and creatine kinase
• Guiding additional testing by presenting symptoms, which may include chest imaging, ECG, or specific organ function tests Management of the lupus flare depends on the severity and organ systems involved, with considerations including:
• Initiation of prednisone 20-40mg daily for mild to moderate flares, with higher doses (methylprednisolone 500-1000mg IV daily for 3 days) for severe flares involving major organs
• Addition of hydroxychloroquine 200-400mg daily, if not already prescribed, as it is recommended in all patients with lupus, at a dose not exceeding 5 mg/kg real body weight 1
• Consideration of immunosuppressants like mycophenolate mofetil, cyclophosphamide, or rituximab for refractory cases or severe organ involvement, while aiming to minimize glucocorticoids to less than 7.5 mg/day and, when possible, withdraw them 1 Supportive care measures should also be implemented, including:
• Pain management with NSAIDs if renal function permits
• Antiemetics for gastrointestinal symptoms
• Oxygen therapy if needed The decision to admit or discharge the patient should be based on the severity of symptoms, with those having severe manifestations requiring admission and close monitoring, while those with milder symptoms can be discharged with close follow-up within 1-2 weeks.

From the FDA Drug Label

The FDA drug label does not answer the question.

From the Research

Evaluation of Scleroderma Lupus in the Emergency Department

• The evaluation of a patient with scleroderma lupus who is having a lupus flare-up in the emergency department should involve a thorough assessment of their symptoms, medical history, and current treatment regimen 2.
• Laboratory tests, such as complete blood count, blood chemistry, and urinalysis, should be performed to evaluate the severity of the flare-up and potential organ involvement 3, 4.
• Imaging studies, such as chest X-ray and renal ultrasound, may be necessary to assess for pulmonary and renal involvement 5, 6.

Management of Lupus Flare-Up

• The management of a lupus flare-up in the emergency department should involve a multidisciplinary approach, including rheumatology, nephrology, and other specialties as needed 2.
• Treatment options for lupus flare-up may include:
  • High-dose corticosteroids to control inflammation and prevent organ damage 3, 4.
  • Immunosuppressive agents, such as mycophenolate mofetil, to reduce disease activity and prevent relapses 5, 6.
  • Biologic agents, such as belimumab, to target specific immune pathways and reduce disease activity 2.
• The choice of treatment should be individualized based on the patient's medical history, disease severity, and potential comorbidities 3, 5, 4, 6, 2.

Considerations for Scleroderma Lupus

• Patients with scleroderma lupus may require special consideration due to the potential for pulmonary and renal involvement 5, 6.
• The use of immunosuppressive agents, such as mycophenolate mofetil, may be beneficial in reducing disease activity and preventing relapses in patients with scleroderma lupus 5, 6.
• Close monitoring of the patient's condition and adjustment of the treatment regimen as needed is crucial to prevent complications and improve outcomes 3, 4, 2.