Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
CASE REPORTS
Hypertrophic Pachymeningitis and Tracheobronchial Stenosis in IgG4-related Disease: Case Presentation and Literature Review
Hiroyuki YamashitaYuko TakahashiHiroyuki IshiuraToshikazu KanoHiroshi KanekoAkio Mimori
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JOURNALS OPEN ACCESS

Volume 51 (2012) Issue 8 Pages 935-941

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Abstract

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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© 2012 by The Japanese Society of Internal Medicine
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