IgG4-associated Multifocal Systemic Fibrosis Complicating Sclerosing Sialadenitis, Hypophysitis, and Retroperitoneal Fibrosis, but Lacking Pancreatic Involvement

Tsuyoshi Tanabe; Kenji Tsushima; Masanori Yasuo; Kazuhisa Urushihata; Masayuki Hanaoka; Tomonobu Koizumi; Keisaku Fujimoto; Keishi Kubo; Takeshi Uehara; Satoshi Shigematsu; Hideaki Hamano; Shigeyuki Kawa

doi:10.2169/internalmedicine.45.1759

CASE REPORTS

IgG4-associated Multifocal Systemic Fibrosis Complicating Sclerosing Sialadenitis, Hypophysitis, and Retroperitoneal Fibrosis, but Lacking Pancreatic Involvement

Tsuyoshi Tanabe, Kenji Tsushima, Masanori Yasuo, Kazuhisa Urushihata, Masayuki Hanaoka, Tomonobu Koizumi, Keisaku Fujimoto, Keishi Kubo, Takeshi Uehara, Satoshi Shigematsu, Hideaki Hamano, Shigeyuki Kawa

Author information

Tsuyoshi Tanabe
The First Department of Internal Medicine, Shinshu University School of Medicine
Kenji Tsushima
The First Department of Internal Medicine, Shinshu University School of Medicine
Masanori Yasuo
The First Department of Internal Medicine, Shinshu University School of Medicine
Kazuhisa Urushihata
The First Department of Internal Medicine, Shinshu University School of Medicine
Masayuki Hanaoka
The First Department of Internal Medicine, Shinshu University School of Medicine
Tomonobu Koizumi
The First Department of Internal Medicine, Shinshu University School of Medicine
Keisaku Fujimoto
The First Department of Internal Medicine, Shinshu University School of Medicine
Keishi Kubo
The First Department of Internal Medicine, Shinshu University School of Medicine
Takeshi Uehara
the Department of Clinical Laboratory, Shinshu University School of Medicine
Satoshi Shigematsu
the Department of Aging Medicine and Geriatrics, Shinshu University School of Medicine
Hideaki Hamano
the Second Department of Internal Medicine, Shinshu University School of Medicine
Shigeyuki Kawa
Center for Health, Safety and Environmental Management, Shinshu University

Keywords: panhypopituitarism, FDG-PET, IgG4-associated multifocal systemic fibrosis, IgG4-related autoimmune dissease, multifocal fibrosclerosis, autoimmune pancreatitis

JOURNAL OPEN ACCESS

2006 Volume 45 Issue 21 Pages 1243-1247

DOI https://doi.org/10.2169/internalmedicine.45.1759

Details

Abstract

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.

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