The Journal of the Japanese Respiratory Society ONLINE JOURNAL

ABSTRACT

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Case Report

A case of IgG4-related disease with marked thickening of the bronchial wall

Masachika Hayashi1), Masaaki Okajima1), Takashi Shimizu1), Natsue Shimizu1), Kazuaki Morohashi1), Jozi Toyama1) and Kazuhisa Mori2)

1)Department of Internal Medicine, Joetsu General Hospital
2)Department of Oral and Maxillofacial Surgery, Nippon Dental University School of Life Dentistry

ABSTRACT

A 52-year-old man noticed hardening and swelling of his salivary glands in the summer of 2009. We suspected Mikulicz disease and performed several work-ups. His serum IgG4 level was elevated, and a chest computed tomography scan demonstrated marked thickening of the bronchial wall. A histopathologic examination of a bronchial mucosa biopsy specimen revealed diffuse infiltration of IgG4-positive cells in the submucosal layers, and on this basis we diagnosed IgG4-related disease. After the administration of corticosteroids, the patient's symptoms, his serum IgG4 level and bronchial wall thickening all improved. When encountering a patient with thickening of the bronchial wall, IgG4-related disease should be considered.

KEYWORDS: IgG4-related disease, Bronchial wall thickening, Mikulicz disease, Autoimmune pancreatitis, Corticosteroid

RECEIVED: 2011.4.8

JJRS, 49(12): 936-941, 2011