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

Authors:
Hiroyuki Yamashita
Hiroyuki Yamashita
Noguchi Thyroid Clinic and Hospital Foundation
Japan
Yuko Takahashi
Yuko Takahashi
National Center for Global Health and Medicine
Shinjuku | Japan
Hiroyuki Ishiura
Hiroyuki Ishiura
Graduate School of Medicine
Toshikazu Kano
Toshikazu Kano
Nihon University School of Medicine
Japan
Hiroshi Kaneko
Hiroshi Kaneko
Nagoya University Graduate School of Medicine
Japan
Akio Mimori
Akio Mimori
Research Institute
Japan

Intern Med 2012 15;51(8):935-41. Epub 2012 Apr 15.

Division of Rheumatic Diseases, National Center for Global Health and Medicine, Japan.

Immunoglobulin 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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January 2013
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