The Journal of the Japanese Respiratory Society ONLINE JOURNAL

ABSTRACT

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Article in Japanese

Case Report

A case of systemic IgG4-related disease with a middle-posterior mediastinal lesion

Sachiyo Maki1), Akira Takakura1), Yasuhiro Hiyoshi1), Masanori Yokoba2), Noriyuki Masuda1) and Masato Katagiri2)

1)Respiratory Medicine, School of Medicine, Kitasato University
2)School of Allied Health Sciences, Kitasato University

ABSTRACT

A 62-year-old Japanese man visited our hospital for the examination of a middle-posterior mediastinal lesion noted on a chest CT image. Magnetic resonance imaging (MRI) showed swelling of the hypophysis, bilateral orbital muscles and bilateral lacrimal and submandibular glands. CT subsequently showed a middle-posterior mediastinal lesion, centrilobular nodules in the lung fields, swelling of the pulmonary hilar lymph nodes and soft tissue tumor around the bilateral ureteropelvic junctions. The patient's serum IgG4 level was elevated, and biopsy specimens from the lacrimal gland showed abundant IgG4-positive plasma cells. These findings were consistent with systemic IgG4-related disease. Following steroid treatment, the lesions reduced. To the best of our knowledge, there are only 2 case reports regarding systemic IgG4-related disease accompanied with a middle-posterior mediastinal lesion.

KEYWORDS: Middle-posterior mediastinal lesion, IgG4-related disease, IgG4-positive plasma cells

RECEIVED: 2011.3.18

JJRS, 49(12): 908-912, 2011