Pediatrics & Neonatology
Volume 49, Issue 2 , Pages 39-42, April 2008

Macrophage Activation Syndrome as Initial Presentation of Systemic Lupus Erythematosus

Department of Pediatrics, Chang Gung Memorial Hospital, Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan

Received 27 November 2007; received in revised form 15 January 2008; accepted 25 March 2008.

Article Outline

Macrophage activation syndrome (MAS) is known to be a severe and potentially life-threatening complication of rheumatic disorder, especially systemic juvenile rheumatoid arthritis. It is very rare for MAS to be an initial presentation of systemic lupus erythematosus (SLE). Here, we report a 14-year-old girl in whom MAS developed as an initial presentation of SLE. With early diagnosis and administration of cyclosporine A, she had a fair outcome. Further testing showed positive anti-dsDNA about 8 months later.

KEY WORDS:  cyclosporine A , macrophage activation syndrome , systemic lupus erythematosus , type V hyperlipo-proteinemia

No full text is available. To read the body of this article, please view the PDF online.

 

Back to Article Outline

References 

  1. Kogawa K , Lee SM , Villanueva J , Marmer D , Sumegi J , Filipovich AH . Perforin expression in cytotoxic lymphocytes from patients with hemophagocytic lymphohistiocytosis and their family members . Blood . 2002;99:61–66
  2. Janka G . Familial and acquired hemophagocytic lymphohistiocytosis . Eur J Pediatr . 2007;166:95–109
  3. Ravelli A . Macrophage activation syndrome . Curr Opin Rheumatol . 2002;14:548–552
  4. Henter J-I , Elinder G , Ost A . Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society . Semin Oncol . 1991;18:29–33
  5. Janka GE , Schenider EM . Modern management of children with haemophagocytic lymphohistiocytosis . Br J Haematol . 2004;124:4–14
  6. Henter J-I , Tondini C , Pritchard J . Histiocytic syndromes . Crit Rev Oncol Hematol . 2004;50:157–174
  7. Kelly A , Ramanan AV . Recognition and management of macrophage activation syndrome in juvenile arthritis . Curr Opin Rheumotol . 2007;19:477–481
  8. Henter JI , Horne A , Arico M , et al.   HLH–2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis . Pediatr Blood Cancer . 2007;48:124–131
  9. Hadchouel M , Prieur AM , Griscelli C . Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: possible relationship to drugs or infection . J Pediatr . 1985;106:561–566
  10. Grom AA . Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities? . Curr Opin Rheumatol . 2003;15:587–590
  11. Avcin T , Tse SM , Schneider R , Ngan B , Silverman ED . Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood . J Pediatr . 2006;148:683–686
  12. McCann LJ , Hasson N , Pilkington CA . Macrophage activation syndrome as an early presentation of lupus . J Rheumatol . 2006;33:438–440
  13. Benarroch LMK , Sterba G , Bosque M . Macrophage activation syndrome as a debut of systemic lupus erythematosus . J Allergy Clin Immunol . 2006;117:S210
  14. Stephan JL , Kone-Paut I , Galambrun C , Mouy R , BaderMeunier B , Prieur AM . Reactive haemophagocytic syndrome in children with inflammatory disorders: a retrospective study of 24 patients . Rheumatology . 2001;40:1285–1292
  15. Hochberg MC . Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus . Arthritis Rheum . 1997;40:1725
  16. Tan EM , Cohen AS , Fries JF , et al.   The 1982 revised criteria for the classification of systemic lupus erythematosus . Arthritis Rheum . 1982;25:1271
  17. Smeenk R , Brinkman K , Van den Brink H , et al.   Antibodies to DNA in patients with systemic lupus erythematosus: their role in the diagnosis, the follow-up and the pathogenesis of the disease . Clin Rheumatol . 1990;9(1 Suppl 1):100–110
  18. Chen HH , Kuo HC , Wang L , et al.   Childhood macrophage activation syndrome differs from infection-associated hemophagocytosis syndrome in etiology and outcome in Taiwan . J Microbiol Immunol Infect . 2007;40:265–271
  19. Mouy R , Stephan JL , Pillet P , Haddad E , Hubert P , Prieur AM . Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: report of five cases . J Pediatr . 1996;129:750–754

PII: S1875-9572(08)60010-8

doi:10.1016/S1875-9572(08)60010-8

Pediatrics & Neonatology
Volume 49, Issue 2 , Pages 39-42, April 2008