• Internal medicine · Jan 2020

    Case Reports

    An Autopsy Case of Mixed Connective Tissue Disease Complicated by Thrombotic Thrombocytopenic Purpura.

    • Makiko Yashiro Furuya, Hiroshi Watanabe, Shuzo Sato, Yuya Fujita, Jumpei Tenmoku, Naoki Matsuoka, Tomoyuki Asano, Eiji Suzuki, Hiroko Kobayashi, and Kiyoshi Migita.
    • Department of Rheumatology, Fukushima Medical University School of Medicine, Japan.
    • Intern. Med. 2020 Jan 1; 59 (10): 1315-1321.

    AbstractWe herein report a patient with mixed connective tissue disease (MCTD) who had been stable for years but suddenly developed thrombotic thrombocytopenic purpura (TTP). The patient showed a clinical pentad of signs of TTP, low activity of ADAMTS13, and positivity of anti-ADAMTS13 antibodies. She did not respond to plasma exchange or steroid therapy and died five days after admission. An autopsy revealed microthrombi in the brain, heart, kidney, adrenal glands, esophageal submucosa, and bone marrow as well as diffuse alveolar hemorrhaging. Physicians should bear in mind that TTP can occur in MCTD patients regardless of disease activity.

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