Advanced SearchSearch Tips
Macrophage Activation Syndrome Triggered by Herpes Viral Infection as the Presenting Manifestation of Juvenile Systemic Lupus Erythematosus
facebook(new window)  Pirnt(new window) E-mail(new window) Excel Download
  • Journal title : Pediatric Infection and Vaccine
  • Volume 22, Issue 3,  2015, pp.210-215
  • Publisher : The Korean Society of Pediatric Infectious Diseases
  • DOI : 10.14776/piv.2015.22.3.210
 Title & Authors
Macrophage Activation Syndrome Triggered by Herpes Viral Infection as the Presenting Manifestation of Juvenile Systemic Lupus Erythematosus
Noh, Ji Hye; Jeong, Do Young; Jeon, In Su; Kim, Hwang Min;
  PDF(new window)
Macrophage activation syndrome (MAS) is a rare complication in systemic lupus erythematosus (SLE) that can be triggered by infections. Due to the fact that MAS may mimic clinical features of underlying rheumatic disease, or be confused with an infectious complication, its detection can prove challenging. This is particularly true when there is an unknown/undiagnosed disease; and could turn into an even greater challenge if MAS and SLE are combined with a viral infection. A-14-year-old female came to the hospital with an ongoing fever for 2 weeks and a painful facial skin rash. Hepatomegaly, pancytopenia, increased aspartate aminotransferase, elevated serum ferritin and lactate dehydrogenase were reported. No hemophagocytic infiltration of bone marrow was reported. The patient was suspected for hemophagocytic lymphohistiocytosis. Her skin rashes were eczema herpeticum, which is usually associated with immune compromised conditions. With the history of oral ulcers and malar rash, positive ANA and low C3, C4 and the evidence of hemolytic anemia, she was diagnosed as SLE. According to the diagnostic guideline for MAS in SLE, she was diagnosed MAS as well, activated by acute HSV infection. After administering steroids and antiviral agent, the fever and skin rash disappeared, and the abnormal laboratory findings normalized. Therefore, we are reporting a rare case of MAS triggered by acute HSV infection as the first manifestation of SLE.
Macrophage activation syndrome;Herpes viral infection;Systemic lupus erythematosus;
 Cited by
Macrophage Activation Syndrome: A Report of Two Cases and a Literature Review, Case Reports in Rheumatology, 2017, 2017, 2090-6897, 1  crossref(new windwow)
Stichweh D, Arce E, Pascual V. Update on pediatric systemic lupus erythematosus. Curr Opin Rheumatol 2004;16:577-87. crossref(new window)

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;148:683-6. crossref(new window)

Shimizu M, Yokoyama T, Tokuhisa Y, Ishikawa S, Sakakibara Y, Ueno K, et al. Distinct cytokine profile in juvenile systemic lupus erythematosus-associated macrophage activation syndrome. Clin Immunol 2013;146:73-6. crossref(new window)

Parodi A, Davi S, Pringe AB, Pistorio A, Ruperto N, Magni-Manzoni S, et al. Macrophage activation syndrome in juvenile systemic lupus erythematosus: a multinational multicenter study of thirty-eight patients. Arthritis Rheum 2009; 60:3388-99. crossref(new window)

Ramos-Casals M, Cuadrado MJ, Alba P, Sanna G, Brito-Zeron P, Bertolaccini L, et al. Acute viral infections in patients with systemic lupus erythematosus: description of 23 cases and review of the literature. Medicine 2008;87:311-8. crossref(new window)

Ueda Y, Yamashita H, Takahashi Y, Kaneko H, Kano T, Mimori A. Refractory hemophagocytic syndrome in systemic lupus erythematosus successfully treated with intermittent intravenous cyclophosphamide: three case reports and literature review. Clin Rheumatol 2014;33:281-6. crossref(new window)

Petri M, Orbai AM, Alarcon GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677-86. crossref(new window)

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;47:1686-91. crossref(new window)

Egues Dubuc CA, Uriarte Ecenarro M, Meneses Villalba C, Aldasoro Caceres V, Hernando Rubio I, Belzunegui Otano J. Hemophagocytic syndrome as the initial manifestation of systemic lupus erythematosus. Reumatol Clin 2014;10:321-4. crossref(new window)

Jimenez AT, Vallejo ES, Cruz MZ, Cruz AC, Jara BS. Macrophage activation syndrome as the initial manifestation of severe juvenile onset systemic lupus erythematosus. Favorable response to cyclophosphamide. Reumatologia Clinica (English Edition) 2014;10:331-5. crossref(new window)

Vilaiyuk S, Sirachainan N, Wanitkun S, Pirojsakul K, Vaewpanich J. Recurrent macrophage activation syndrome as the primary manifestation in systemic lupus erythematosus and the benefit of serial ferritin measurements: a case-based review. Clin Rheumatol 2013;32:899-904. crossref(new window)

Stephan J, Kone-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM. Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology 2001;40:1285-92. crossref(new window)

Yeap ST, Sheen JM, Kuo HC, Hwang KP, Yang KD, Yu HR. Macrophage activation syndrome as initial presentation of systemic lupus erythematosus. Pediatr Neonatol 2008;49:39-42. crossref(new window)

Lambotte O, Khellaf M, Harmouche H, Bader-Meunier B, Manceron V, Goujard C, et al. Characteristics and long-term outcome of 15 episodes of systemic lupus erythematosusassociated hemophagocytic syndrome. Medicine (Baltimore) 2006;85:169-82. crossref(new window)

Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child 2001;85:421-6. crossref(new window)

Isome M, Suzuki J, Takahashi A, Murai H, Nozawa R, Suzuki S, et al. Epstein-Barr virus-associated hemophagocytic syndrome in a patient with lupus nephritis. Pediatr Nephrol 2005;20:226-8. crossref(new window)

Behrens EM, Beukelman T, Paessler M, Cron RQ. Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis. J Rheumatol 2007;34:1133-8.

Benarroch LK, Sterba G, Bosque M. Macrophage activation syndrome (MAS) as a debut of systemic lupus erythematous (SLE) in a child. J Allergy Clin Immunol 2006;117:S210.

Hur M, Kim YC, Lee KM, Kim KN. Macrophage activation syndrome in a child with systemic juvenile rheumatoid arthritis. J Korean Med Sci 2005;20:695-8. crossref(new window)

Shim YS, Kim HS, Kim KN. A case of macrophage activation syndrome successfully treated with combination therapy including etanercept. J Rheum Dis 2012;19:225-9. crossref(new window)

Keum SW, Kim MJ, Bae EY, Ham SB, Chung NK, Jeong DC, et al. A case of macrophage activation syndrome developed in female adolescent with systemic lupus erythematosus. J Rheum Dis 2014;21:96-100. crossref(new window)

Hwang JY, No SM, Lee J, Jang PS, Kim YH, Kim JT, et al. A case of hemophagocytic lymphohistiocytosis in a child with systemic lupus erythematosus. Korean J Pediatr 2003;46: 1029-31.

Kim ES, Kim YG, Kim WS, Jung YS, Han JH, Bae CB, et al. Three cases of secondary hemophagocytic lymphohistiocytosis associated with systemic erythematosus lupus. J Rheum Dis 2015;22:180-5. crossref(new window)