Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report

Wesam Gouda,Abdelhfeez Moshrif,Awad S Abbas,Md Asiful Islam,Faisal Alsaqabi,Tarek M Abdel-Aziz

doi:10.1186/s13256-021-03072-1

Abstract

BackgroundMacrophage activation syndrome is classified as a secondary form of hemophagocytic lymphohistiocytosis. It is a hyperinflammatory complication observed to be comorbid with a variety of autoimmune diseases, including adult-onset Still’s disease and systemic juvenile idiopathic arthritis. Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus, which, if untreated, can be fatal, though determining the optimum treatment strategy is still a challenge.Case presentationHerein, we report a case of macrophage activation syndrome in a 33-year-old Egyptian female as an unusual complication of a systemic lupus erythematosus flare in adult patients. Our patient was initially treated with a combination of intravenous methylprednisolone pulse therapy and intravenous immunoglobulin therapy, which was followed by a course of oral prednisolone and oral cyclosporine with little response. Switching from oral prednisone to intravenous dexamethasone sodium phosphate showed a more favorable clinical and biochemical response.ConclusionMacrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus. Our case demonstrates that dexamethasone sodium phosphate can be a successful alternative treatment for patients with systemic lupus erythematosus complicated by macrophage activation syndrome in whom the response to pulse methylprednisolone was inadequate to manage their illness, proving to be remarkably effective in a relatively short time frame.

Highlights

Macrophage activation syndrome is classified as a secondary form of hemophagocytic lymphohis‐ tiocytosis
Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus
Our case demonstrates that dexamethasone sodium phosphate can be a successful alternative treat‐ ment for patients with systemic lupus erythematosus complicated by macrophage activation syndrome in whom the response to pulse methylprednisolone was inadequate to manage their illness, proving to be remarkably effective in a relatively short time frame

Summary

Conclusion

MAS should be included in the differential diagnosis of SLE patients with persistent fever and pancytopenia. Physicians ought to have a high level of suspicion for diagnosing MAS in the setting of relevant clinical symptoms and signs. And proper treatment is crucial to prevent the high mortality rate. Anti-dsDNA antibody high titer, and complement levels, hyperferritinemia and hypertriglyceridemia are regarded as the most important parameters for differentiating between MAS and SLE flare. Our case demonstrates that DSP can be a successful alternative treatment for MAS patients in whom the response to pulse mPSL was inadequate to manage their illness, proving to be remarkably effective in a relatively short time frame. Larger trials are required to determine the value and exact mechanism of DSP in MAS patients

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