A case of TMA developed during treatment for SLE.

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  • Kawanishi Ayana
    Department of Pediatrics, Hamamatsu University School of Medicine
  • Yasuoka Ryuhei
    Department of Pediatrics, Hamamatsu University School of Medicine
  • Koda Masaki
    Department of Pediatrics, Hamamatsu University School of Medicine
  • Katoh Yukiko
    Department of Pediatrics, Hamamatsu University School of Medicine
  • Uchida Hiroshi
    Department of Pediatrics, Hamamatsu University School of Medicine
  • Kitagata Ryoichi
    Department of Pediatrics, Hamamatsu University School of Medicine
  • Natsume Osamu
    Department of Pediatrics, Hamamatsu University School of Medicine

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  • 全身性エリテマトーデスの治療中に発症した血栓性微小血管症の女児例

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Abstract

<p>Thrombotic microangiopathy (TMA) is a serious complication of systemic lupus erythematosus (SLE) that reportedly occurs in parallel with increased SLE disease activity. We report the case of a girl who developed TMA soon after the onset and treatment of SLE, despite improvement in SLEDAI (SLE disease activity index) and serological markers. The 12-year-old girl had developed SLE with severe lupus nephritis. After the initial round of steroid pulse therapy, her platelet count increased and her anti-ds-DNA antibody titer decreased. However, her hemoglobin level and platelet count declined after the second round of steroid pulse therapy (day 12 of hospitalization), and even schistocytes were observed in a peripheral blood smear. Those suggested the development of TMA. On day 16, we commenced plasma exchange therapy and initiated treatment with intravenous cyclophosphamide and oral mycophenolate mofetil; on day 33, the schistocytosis resolved. Because TMA can develop at any time during the course of SLE treatment, clinicians should consider TMA as a possible complication in SLE patients who develop schistocytosis or experience any unusual changes in SLE disease activity.</p>

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