• Internal medicine · Jan 2012

    Review Case Reports

    Hypertrophic pachymeningitis and tracheobronchial stenosis in IgG4-related disease: case presentation and literature review.

    • Hiroyuki Yamashita, Yuko Takahashi, Hiroyuki Ishiura, Toshikazu Kano, Hiroshi Kaneko, and Akio Mimori.
    • Division of Rheumatic Diseases, National Center for Global Health and Medicine, Japan. hiroyuki_yjp2005@yahoo.co.jp
    • Intern. Med. 2012 Jan 1;51(8):935-41.

    AbstractImmunoglobulin G4 (IgG4)-related disease is a distinctive mass-forming disorder with frequent systemic involvement, most commonly in the pancreas, salivary glands and lacrimal glands. A few cases of dural involvement and one case of central airway stenosis have also been described. We report here a rare case of IgG4-related disease with intracranial hypertrophic pachymeningitis and irregular tracheobronchial stenosis. We review four previously reported cases of IgG4-related pachymeningitis. We currently lack international standards for the diagnosis of extrapancreatic IgG4-related disease. Based on the findings of the present case and those reported previously, we discuss the distinctive features of IgG4-related pachymeningitis.

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