Pediatric Infection and Vaccine

The Korean Society of Pediatric Infectious Diseases (대한소아감염학회)
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Macrophage Activation Syndrome Triggered by Herpes Viral Infection as the Presenting Manifestation of Juvenile Systemic Lupus Erythematosus

헤르페스 바이러스 감염으로 발생한 대식 세포 활성 증후군을 첫 증상으로 한 소아 전신 홍반 루푸스

  • Noh, Ji Hye (Department of Pediatrics, Yonsei University, Wonju College of Medicine) ;
  • Jeong, Do Young (Department of Pediatrics, Yonsei University, Wonju College of Medicine) ;
  • Jeon, In Su (Department of Pediatrics, Yonsei University, Wonju College of Medicine) ;
  • Kim, Hwang Min (Department of Pediatrics, Yonsei University, Wonju College of Medicine)
  • 노지혜 (연세대학교 원주의과대학 소아과학교실) ;
  • 정도영 (연세대학교 원주의과대학 소아과학교실) ;
  • 전인수 (연세대학교 원주의과대학 소아과학교실) ;
  • 김황민 (연세대학교 원주의과대학 소아과학교실)
  • Received : 2015.08.10
  • Accepted : 2015.09.17
  • Published : 2015.12.25
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Abstract

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.

대식세포 활성 증후군(MAS, Macrophage activation syndrome)은 전신 홍반성 루푸스(SLE, systemic lupus erythematous) 환자에서 감염에 의해 나타날 수 있는 드문 합병증이다. MAS는 기저의 자가면역질환의 임상양상과 유사하게 나타나거나 혹은 감염성 합병증과 혼돈될 수 있어 감별에 주의 하여야 한다. 14세 여환이 2주간 지속되는 발열과 통증을 동반하는 얼굴의 피부 발진을 주소로 내원하였다. 피부 발진과 간비대, 범혈구 감소증, aspartate aminotransferase, lactate dehydrogenase, 혈청 ferritin이 상승하여, MAS를 의심하였다. 피부 병변과 항핵체 양성, C3와 C4의 감소, 간접 쿰스검사 양성으로 SLE를 진단하였다. 따라서 본 증례는 MAS가 HSV에 의하여 촉발된 것을, SLE의 첫 증상으로서 나타낸 증례로서, 촉발 요인 및 기저질환을 치료함으로써 중증의 합병증 없이 호전되었다.

Keywords

References

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  1. Macrophage Activation Syndrome: A Report of Two Cases and a Literature Review vol.2017, pp.2090-6897, 2017, https://doi.org/10.1155/2017/5304180