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Mixed connective tissue disease associated with antineutrophil cytoplasmic antibodies against proteinase-3 and systemic atherosclerosis: a case report

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Abstract

A 47-year-old woman presented with facial spasm, swollen fingers and Raynaud’s phenomenon due to cerebrovascular disorder and mixed connective tissue disease (MCTD). Although she was positive for both antineutrophil cytoplasmic antibodies against proteinase-3 (PR3-ANCA) and anti-U1 RNP antibodies, she did not meet the American College of Rheumatology classification criteria for Wegener’s granulomatosis (WG). Physical and histopathological examinations revealed severe systemic atherosclerosis without any of the traditional risk factors. Elevated levels of malondialdehyde-modified LDL and antioxidized LDL autoantibodies, which are considered to be key factors in the pathogenesis of atherosclerosis, were also detected in the serum of this patient. In this case, systemic atherosclerosis might have been linked to these autoimmune reactions.

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Abbreviations

MCTD:

Mixed connective tisue disease

SLE:

Systemic lupus erythematosus

WG:

Wegener’s granulomatosis

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Author notes

  1. Yoko Wada

    Present address: Division of Clinical Rheumatology, Niigata Prefectural Senami Hospital, 2-4-15, Senami-Onsen, Murakami City, 958-8555, Niigata , Japan

Authors and Affiliations

  1. Department of Internal Medicine, Nagaoka Central General Hospital, Japan

    Masato Kanazawa, Yoko Wada, Hian In & Kazuaki Yahata

  2. Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Japan

    Yoko Wada, Satoshi Ito, Mitsuhiro Ueno & Fumitake Gejyo

  3. Department of Neurology, Nagaoka Central General Hospital, Japan

    Tsukasa Ohno

  4. Department of Dermatology, Nagaoka Central General Hospital, Japan

    Junko Izumi

  5. Department of Otolaryngology, Nagaoka Central General Hospital, Japan

    Hisao Tanaka

  6. School of Health Sciences, Faculty of Medicine, Niigata University, Japan

    Masaaki Nakano

Authors
  1. Masato Kanazawa
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Correspondence to Yoko Wada.

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Kanazawa, M., Wada, Y., Ohno, T. et al. Mixed connective tissue disease associated with antineutrophil cytoplasmic antibodies against proteinase-3 and systemic atherosclerosis: a case report. Clin Rheumatol 23, 456–459 (2004). https://doi.org/10.1007/s10067-004-0911-x

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  • DOI: https://doi.org/10.1007/s10067-004-0911-x

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