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Familial HLA-B*52 Vasculitis: Maternal, Atypical Cogan's Syndrome with Takayasu Arteritis-mimicking Aortitis and Filial Takayasu Arteritis

  • Morinaka Satoshi
    Division of Rheumatology, Ibaraki Prefectural Central Hospital, Japan
  • Takano Yohei
    Division of Rheumatology, Ibaraki Prefectural Central Hospital, Japan
  • Tsuboi Hiroto
    Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
  • Goto Daisuke
    Division of Rheumatology, Ibaraki Prefectural Central Hospital, Japan Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan Division of Rheumatology, Ibaraki Clinical Education and Training Center, Tsukuba University Hospital, Japan
  • Sumida Takayuki
    Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan

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Abstract

<p>Cogan's syndrome (CS), a rare vasculitis characterized by non-syphilitic, interstitial keratitis and Ménière-like attacks, is classified into "typical" and "atypical" forms, while Takayasu arteritis (TAK) is a rare large-vessel vasculitis associated with human leukocyte antigen (HLA)-B*52. Very few cases meet both the CS and TAK classification criteria. We herein report a 53-year-old woman diagnosed with atypical CS and aortitis similar to TAK. Her 25-year-old daughter manifested TAK without symptoms of CS, and both are HLA-B*52 positive. Our case highlights the difficulties of distinguishing aortitis with atypical CS from aortitis with TAK. </p>

Journal

  • Internal Medicine

    Internal Medicine 59 (15), 1899-1904, 2020-08-01

    The Japanese Society of Internal Medicine

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