Anti-PM/Scl Antibody-positive Systemic Sclerosis Complicated by Multiple Organ Involvement

  • Shimizu Tatsuya
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Saito Chie
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Watanabe Megumi
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Ishii Ryota
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Kawamura Tetsuya
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Nagai Kei
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Fujita Akiko
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Kaneko Shuzo
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Kai Hirayasu
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Morito Naoki
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Usui Joichi
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan
  • Yokosawa Masahiro
    Department of Rheumatology, Faculty of Medicine, University of Tsukuba, Japan
  • Kondo Yuya
    Department of Rheumatology, Faculty of Medicine, University of Tsukuba, Japan
  • Inoue Sae
    Department of Dermatology, Faculty of Medicine, University of Tsukuba, Japan
  • Okiyama Naoko
    Department of Dermatology, Faculty of Medicine, University of Tsukuba, Japan
  • Yamagata Kunihiro
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan

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Abstract

<p>A 40-year-old Japanese woman developed malignant-phase hypertension complicated by thrombotic microangiopathy, progressing to end-stage renal disease. Five years later, she was diagnosed with pulmonary arterial hypertension and interstitial pneumonia. Despite a lack of overt skin sclerosis, nucleolar staining in our indirect immunofluorescence analysis and nailfold capillaroscopy facilitated the diagnosis of anti-PM/Scl antibody-positive systemic sclerosis. We observed the persistent presence of anti-PM/Scl antibodies throughout the clinical course, suggesting that her kidney disease was scleroderma renal crisis. Anti-PM/Scl antibodies can be associated with multiple organ diseases. Careful attention to a patient's antinuclear antibody pattern and dermatological findings may help clarify the etiology of undiagnosed diseases. </p>

Journal

  • Internal Medicine

    Internal Medicine 60 (7), 1101-1107, 2021-04-01

    The Japanese Society of Internal Medicine

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