An anti-U3RNP antibody-positive case of systemic sclerosis who developed thrombotic microangiopathy with renal crisis and various other critical organ involvements

DOI
  • Torigoe Masataka
    Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of medicine, Oita University
  • Maeshima Keisuke
    Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of medicine, Oita University
  • Kiyonaga Yasuhiro
    Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of medicine, Oita University
  • Imada Chiharu
    Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of medicine, Oita University
  • Ozaki Takashi
    Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of medicine, Oita University
  • Haranaka Miwa
    Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of medicine, Oita University
  • Ishii Koji
    Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of medicine, Oita University
  • Shibata Hirotaka
    Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of medicine, Oita University

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Other Title
  • 血栓性微小血管障害症に腎クリーゼなど多彩な臓器病変を合併した抗U3RNP抗体陽性全身性強皮症の一例

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Abstract

    We observed an anti-U3RNP antibody-positive case of systemic sclerosis (SSc) who developed thrombotic microangiopathy (TMA) with renal crisis and various other critical organ involvements. A 59-year-old woman consulted our outpatient department with cold-induced discoloration of the fingers, and the follow-up was started for Raynaudʼs phenomenon and antinuclear antibody positivity in2011. In the following year, she was diagnosed as SSc, based on progressive dermal sclerosis and fingertip pitting scars. In April 2013, she developed TMA with renal crisis,and acute heart failure at the same time, and was admitted to our hospital with an emergency. Administration of the ACE inhibitor, hemodialysis, and plasma exchange were started and high blood pressure and cytopenia due to TMA were controlled well. Methylpredonisolone pulse therapy to address progressive severe myocardial dysfunction was accompanied by a marked response,however, this resulted in diffuse alveolar hemorrhage shortly after. The respiratory damage subsided through continuation of the above treatment and strict management of cardiorespiratory condition. With the intensive therapy, each state of the disease was improved and stabilized successfully. In contrast to what our patient showed, some studies have suggested Japanese SSc patients with anti-U3RNP antibody positivity tend to have less frequent and less severe internal organ dysfunction. Multiple life-threatening complications, which our case presented with, are considered quite rare especially in a Japanese case with this antibody positivity and are thus worth reporting.

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