IgG4-associated Multifocal Systemic Fibrosis Complicating Sclerosing Sialadenitis, Hypophysitis, and Retroperitoneal Fibrosis, but Lacking Pancreatic Involvement
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概要
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A 71-year-old man was admitted with malaise, mild fever, anorexia, body weight loss, lower back pain, thirst, and polydipsia. He showed bilateral swelling of the submandibular glands. Examinations showed panhypopituitarism and a high serum IgG4 concentration. Fluorodeoxyglucose positron emission tomography (FDG-PET) revealed uptake in the pituitary gland, bilateral submandibular gland, bilateral hilar and mediastinal lymph nodes, and a mass consistent with retroperitoneal fibrosis, but not in the pancreas. Biopsy specimens from the submandibular gland and retroperitoneal mass indicated sialadenitis and retroperitoneal fibrosis respectively, and showed severe fibrosis and inflammation with marked lymphoplasmacytic infiltration and IgG4-positive plasma cell infiltration. Hormone replacement therapy with hydrocortisone resulted in marked clinical improvement. Systemic involvement found in this patient possibly corresponded to the new concept of IgG4-associated multifocal systemic fibrosis.
- 社団法人 日本内科学会の論文
著者
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Yasuo Masanori
The First Department of Internal Medicine, Shinshu University School of Medicine
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Hanaoka Masayuki
The First Department of Internal Medicine, Shinshu University School of Medicine
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KOIZUMI Tomonobu
the First Department of Internal Medicine, Shinshu University School of Medicine
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Kawa Shigeyuki
Center For Health Safety And Environmental Management Shinshu University
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Tanabe Tsuyoshi
The First Department of Internal Medicine, Shinshu University School of Medicine
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Kubo Keishi
The First Department Of Internal Medicine Shinshu University
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Tsushima Kenji
The First Department Of Internal Medicine Shinshu University School Of Medicine
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Urushihata Kazuhisa
The First Department Of Medicine Shinshu University
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Fujimoto Keisaku
The First Department Of Internal Medicine Shinshu University
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Hamano Hideaki
The Second Department of Internal Medicine, Shinshu University School of Medicine
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Shigematsu Satoshi
the Department of Aging Medicine and Geriatrics, Shinshu University School of Medicine
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Uehara Takeshi
the Department of Clinical Laboratory, Shinshu University School of Medicine
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Urushihata Kazuhisa
The First Department of Internal Medicine, Shinshu University School of Medicine
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