A case of anti-PL-7 antibody positive polymyositis with thrombotic microangiopathy

  • TAMURA Masao
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • KITANO Masayasu
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • AZUMA Kouta
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • TSUBOI Kazuyuki
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • ABE Takeo
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • OGITA Chie
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • YOKOYAMA Yuichi
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • FURUKAWA Tetsuya
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • YOSHIKAWA Takahiro
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • SAITO Atsushi
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • NISHIOKA Aki
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • SEKIGUCHI Masahiro
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • AZUMA Naoto
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • TSUNODA Shinichiro
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • HOSONO Yuji
    Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University
  • NAKASHIMA Ran
    Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University
  • OHMURA Koichiro
    Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University
  • MATSUI Kiyoshi
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • MIMORI Tsuneyo
    Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University
  • SANO Hajime
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine

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Other Title
  • 抗PL-7抗体陽性の多発性筋炎に発症した血栓性微小血管障害症の一例

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Abstract

<p>  A 65-year-old woman with a 17-year history of polymyositis and 8-year history of rheumatoid arthritis who was treated with a low dose of prednisolone and tacrolimus (Tac) was admitted to our hospital because of general malaise and hypertension. Blood tests showed thrombocytopenia, hemolytic anemia with fragmented erythrocytes, and hypercreatinemia. Based on these clinical features, she was diagnosed with thrombotic micro-angiopathy (TMA). Thrombocytopenia and hemolytic anemia with fragmented erythrocytes improved with the discontinuation of Tac and plasma exchange; however, hypertension and renal dysfunction persisted. TMA due to calcineurin inhibitor (CNI) nephropathy was suspected based on the histopathological findings of renal biopsy. However, the condition was atypical of a CNI nephropathy because the trough level of Tac was lower than that reported previously and renal dysfunction persisted after drug discontinuation. She had mild sclerodactylia and Raynaud's symptoms, although the diagnostic criteria for systemic sclerosis (SSc) were not satisfied. Moreover, the patient tested positive for anti PL-7 antibody. The relationship between anti PL-7 antibody and pathogenesis of SSc has been reported. In this case, it was suspected that CNI nephropathy worsened because of the potential basic factors of SSc. These findings indicate that TMA may occur in patients testing positive for anti PL-7 antibody who are treated with Tac.</p>

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