Abstract
Macrophage activation syndrome (MAS), a variant of secondary hemophagocyticlymphohistiocytosis, is a potentially life-threatening complication of inflammatory and autoimmune diseases. We present a case of MAS as a rare manifestation of systemic lupus erythematosus. Although initial treatment with corticosteroid, with or without cyclosporine A, is justified in patients with MAS, evidence regarding the effectiveness of this treatment protocol remains to be clarified. Our patient was successfully treated with a combination of intravenous immunoglobulin therapy and intravenous methyl predonisolone pulse therapy, which was followed by a course of oral prednisolone and oral tacrolimus. Based on our experience, we propose tacrolimus to provide a more useful adjuvant treatment to corticosteroid therapy than cyclosporine A.
REFERENCES
1. HENTER JI., HORNE A., ARICÓ M., EGELER RM., FILIPOVICH AH., IMASHUKU S., et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocyticlymphohistiocytosis. Pediatr Blood Cancer. 2007; 48(2):124-31.
2. TAN EM., COHEN AS., FRIES JF., MASI AT., MCSHANE DJ., ROTHFIELD NF., et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982; 25(11):1271-7.
3. BODE SF., LEHMBERG K., MAUL-PAVICIC A., VRAETZ T., JANKA G., STADT UZ., et al. Recent advances in the diagnosis and treatment of hemophagocyticlymphohistiocytosis. Arthritis Res Ther. 2012; 14(3):213.
4. FUKAYA S., YASUDA S., HASHIMOTO T., OKU K., KATAOKA H., HORITA T., et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology (Oxford). 2008; 47(11):1686-91.
5. EGÜES DUBUC CA., URIARTE ECENARRO M., MENESES VILLALBA C., ALDASORO CÁCERES V., HERNANDO RUBIO I., BELZUNEGUI OTANO J. Hemophagocytic syndrome as the initial manifestation of systemic lupus erythematosus. Reumatol Clin. 2014; 10(5):321-4.
6. KUMAKURA S., MURAKAWA Y. Clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome in adults. Arthritis Rheumatol. 2014; 66(8):2297-307.
7. JORDAN MB., ALLEN CE., WEITZMAN S., FILIPOVICH AH., MCCLAIN KL. How I treat hemophagocyticlym-phohistiocytosis. Blood. 2011; 118(15):4041-52.
8. HANNAH J., CASIAN A., D’CRUZ D. Tacrolimus use in lupus nephritis: A systematic review and meta-analysis. Autoimmun Rev. 2016; 15(1):93-101.
