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

• Published in: Internal Medicine Vol. 59; no. 10; pp. 1315 - 1321
• Main Authors: Furuya, Makiko Yashiro, Watanabe, Hiroshi, Sato, Shuzo, Fujita, Yuya, Tenmoku, Jumpei, Matsuoka, Naoki, Asano, Tomoyuki, Suzuki, Eiji, Kobayashi, Hiroko, Migita, Kiyoshi
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 15.05.2020; Japan Science and Technology Agency
• Subjects: a disintegrin-like metalloproteinase with thrombospondin type 1 motif; Adrenal glands; Autopsy; Bone marrow; Case Report; Connective tissue diseases; Esophagus; Health risk assessment; Internal medicine; member 13 (ADAMTS13); Mixed connective tissue disease; mixed connective tissue disease (MCTD); Purpura; Thrombocytopenic purpura; Thrombotic thrombocytopenic purpura; thrombotic thrombocytopenic purpura (TTP); a disintegrin-like metalloproteinase with thrombospondin type 1 motif; member 13 (ADAMTS13); mixed connective tissue disease (MCTD); thrombotic thrombocytopenic purpura (TTP); autopsy
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.3939-19

We 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.