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Recurrent macrophage activation syndrome as the primary manifestation in systemic lupus erythematosus and the benefit of serial ferritin measurements: a case-based review

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Abstract

Macrophage activation syndrome (MAS) is a fatal complication in rheumatic diseases. It is characterized by prolonged fever, pancytopenia, and hepatosplenomegaly, which are consequences of uncontrolled macrophage activation. MAS in children is most commonly associated with systemic juvenile idiopathic arthritis. Its association with systemic lupus erythematosus (SLE) is relatively rare, so we report a Thai boy who initially presented with MAS and eventually was diagnosed as having SLE. He also had recurrent MAS during the course of therapy. Hyperferritinemia is one of the abnormal laboratory findings in MAS and it has been used as an inflammatory marker. However, its correlation with disease activity remains unclear. Therefore, a review of literature regarding MAS-associated SLE in children and ferritin level in this disease was carried out.

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Acknowledgments

We acknowledge the help of Stephen Pinder, a native English speaker in the Section for Clinical Epidemiology, for proofreading the English. We also thank Dr. Pat Mahachoklertwattana, Dr. Suwat Benjaponpitak, and Dr. Mahippathorn Chinnapha for their kind review of the article.

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Authors and Affiliations

  1. Division of Allergy, Immunology, and Rheumatology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Rachatevi, Bangkok, 10400, Thailand

    Soamarat Vilaiyuk

  2. Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Rachatevi, Bangkok, 10400, Thailand

    Nongnuch Sirachainan

  3. Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Rachatevi, Bangkok, 10400, Thailand

    Suthep Wanitkun

  4. Division of Nephrology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Rachatevi, Bangkok, 10400, Thailand

    Kwanchai Pirojsakul

  5. Division of Pediatric Critical Care, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Rachatevi, Bangkok, 10400, Thailand

    Jarin Vaewpanich

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  1. Soamarat Vilaiyuk
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  2. Nongnuch Sirachainan
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  3. Suthep Wanitkun
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Correspondence to Soamarat Vilaiyuk.

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Vilaiyuk, S., Sirachainan, N., Wanitkun, S. et al. 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 32, 899–904 (2013). https://doi.org/10.1007/s10067-013-2227-1

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  • DOI: https://doi.org/10.1007/s10067-013-2227-1

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