IgG4-associated multifocal systemic fibrosis complicating sclerosing sialadenitis, hypophysitis, and retroperitoneal fibrosis, but lacking pancreatic involvement.
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.
Tanabe
Tsuyoshi
T
The First Department of Internal Medicine, Shinshu University School of Medicine.
Tsushima
Kenji
K
Yasuo
Masanori
M
Urushihata
Kazuhisa
K
Hanaoka
Masayuki
M
Koizumi
Tomonobu
T
Fujimoto
Keisaku
K
Kubo
Keishi
K
Uehara
Takeshi
T
Shigematsu
Satoshi
S
Hamano
Hideaki
H
Kawa
Shigeyuki
S
D000368:Aged; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007018:Hypopituitarism / Q000097:blood* / Q000150:complications / Q000188:drug therapy; D007074:Immunoglobulin G / Q000009:adverse effects / Q000097:blood*; D008297:Male; D010179:Pancreas / Q000473:pathology; D012185:Retroperitoneal Fibrosis / Q000097:blood* / Q000150:complications / Q000188:drug therapy; D012793:Sialadenitis / Q000097:blood* / Q000150:complications / Q000188:drug therapy
D007074:Immunoglobulin G; D006854:Hydrocortisone