title: A case of retroperitoneal and mediastinal fibrosis exhibiting elevated levels of IgG4 in the absence of sclerosing pancreatitis (autoimmune pancreatitis).
abstract: There is now increasing evidence that IgG4 is closely involved in idiopathic sclerosing lesions, such as sclerosing pancreatitis and sclerosing sialadenitis. In this report, we describe a case of IgG4-related retroperitoneal and mediastinal fibroses. A 52-year-old man presented with dull back pain and was found to have a continuously surrounding paraaortic mass. A biopsy specimen taken from the retroperitoneum showed a diffuse lymphoplasmacytic infiltration intermixed with fibrosis. Many IgG4-positive plasma cells were demonstrated on immunostaining. His serum IgG4 concentration was 392 mg/dL (reference range, <70). We treated this patient with a corticosteroid, which markedly diminished the paraaortic mass along with lowering of his serum IgG4 concentration. The possible involvement of IgG4 was suggested in the pathogeneses of retroperitoneal and mediastinal fibroses in this patient. IgG4 might be useful in the clinical management of retroperitoneal or mediastinal fibrosis to differentiate them from malignant tumors and predict steroid sensitivity.
authors.affiliation: Division of Pathology, Kanazawa University Hospital, Japan. yzen@med.kanazawa-u.ac.jp
authors.forename: Yoh
authors.initials: Y
authors.lastname: Zen
authors.forename: Aiko
authors.initials: A
authors.lastname: Sawazaki
authors.forename: Shiro
authors.initials: S
authors.lastname: Miyayama
authors.forename: Kazuo
authors.initials: K
authors.lastname: Notsumata
authors.forename: Nobuyoshi
authors.initials: N
authors.lastname: Tanaka
authors.forename: Yasuni
authors.initials: Y
authors.lastname: Nakanuma
mesh_terms: D003937:Diagnosis, Differential; D005355:Fibrosis; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis*; D008297:Male; D008477:Mediastinal Diseases / Q000175:diagnosis* / Q000473:pathology; D008875:Middle Aged; D010195:Pancreatitis / Q000175:diagnosis; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis* / Q000473:pathology
chemical_list: D007074:Immunoglobulin G