Respiratory failure and macrophage activation syndrome as an onset of systemic lupus erythematosus: A case report

BACKGROUND: Macrophage activation syndrome (MAS) is defined as a specific secondary hemophagocytic lymphohistiocytosis that refers particularly to those triggered by autoimmune diseases. MAS is a rare and highly lethal complication of systemic lupus erythematosus (SLE), which can be associated with, or mimic, disease flare. However, the data regarding the clinical course, management and outcome of SLE with MAS is limited, especially in adults. Lack of clinical recognition of the disease often leads to poor prognosis. CASE SUMMARY: We report a 36-year-old Chinese woman without relevant past medical history who was admitted to hospital with a 6-d history of jaundice and a high fever of 39.4°C lasting one day. Abdominal magnetic resonance imaging excluded obstructive jaundice, no infection was identified and empiric superior antibiotic treatment (meropenem) showed no clinical improvement. However, newly emerged pancytopenia and respiratory failure endangered the patient’s life. Autoimmune work-up finally led to the diagnosis of SLE, which initially presented as MAS and manifested respiratory failure, although neither bone marrow biopsy nor lymph node biopsy showed hemophagocytosis. To our knowledge, such a scenario has never been reported in detail before. The patient had a favorable reaction to combination treatment with corticosteroid and cyclosporine A and has been in clinical remission during the 1-year follow up period. CONCLUSION: Respiratory failure and MAS can be an onset of SLE. Early diagnosis and appropriate treatment are extremely important for a better prognosis.