title: Clinical features of 10 patients with IgG4-related retroperitoneal fibrosis.
abstract: OBJECTIVE
  To elucidate the clinical characteristics of IgG4-related retroperitoneal fibrosis (RF).
  
  
  METHODS
  IgG4-related RF was diagnosed when all of the following three criteria were fulfilled: retroperitoneal soft tissue masses surrounding the aorta and/or adjacent tissues, elevation of the serum IgG4 levels, and abundant infiltration of IgG4-positive plasma cells in at least one organ or site. Ten patients were diagnosed as having IgG4-related RF.
  
  
  RESULTS
  The mean age at diagnosis was 70.1 years, and the male-to-female ratio was 1:0.6. Only two patients had initial symptoms predominantly related to RF (back pain and edema of the lower extremities), while the remaining eight patients reported initial symptoms due to associated diseases. On laboratory examination, a severe inflammatory reaction was observed in one patient. Elevation of the levels of serum IgG and IgE, eosinophilia and positivity of antinuclear antibodies were detected in seven, five, two and seven patients, respectively. The retroperitoneal masses were detected primarily in the left renal hilus in four patients, in the periaortic region in five patients and in both regions in one patient. Hydronephrosis was present in five patients. The histological diagnosis was confirmed in the retroperitoneal masses (resection, n=1 biopsy, n=2) and extraretroperitoneal lesions (n=7). Twenty-four other IgG4-related diseases were found to be associated with IgG4-related RF in nine patients (autoimmune pancreatitis (n=2), sialadenitis (n=4), dacryoadenitis (n=5), lymphadenopathy (n=9), pulmonary pseudotumor (n=1) and pituitary pseudotumor (n=1)). Seven patients underwent steroid therapy, all of whom responded well and showed no instances relapse.
  
  
  CONCLUSIONS
  IgG4-related RF has several clinical characteristic features. Our diagnostic criteria may be helpful in obtaining a correct diagnosis.
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Japan.
authors.forename: Kazuro
authors.initials: K
authors.lastname: Chiba
authors.forename: Terumi
authors.initials: T
authors.lastname: Kamisawa
authors.forename: Taku
authors.initials: T
authors.lastname: Tabata
authors.forename: Seiichi
authors.initials: S
authors.lastname: Hara
authors.forename: Sawako
authors.initials: S
authors.lastname: Kuruma
authors.forename: Takashi
authors.initials: T
authors.lastname: Fujiwara
authors.forename: Go
authors.initials: G
authors.lastname: Kuwata
authors.forename: Hideto
authors.initials: H
authors.lastname: Egashira
authors.forename: Koichi
authors.initials: K
authors.lastname: Koizumi
authors.forename: Satomi
authors.initials: S
authors.lastname: Koizumi
authors.forename: Junko
authors.initials: J
authors.lastname: Fujiwara
authors.forename: Takeo
authors.initials: T
authors.lastname: Arakawa
authors.forename: Kumiko
authors.initials: K
authors.lastname: Momma
authors.forename: Keigo
authors.initials: K
authors.lastname: Setoguchi
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Shinohara
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D018836:Inflammation Mediators / Q000097:blood; D008297:Male; D008875:Middle Aged; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology*; D012189:Retrospective Studies
chemical_list: D007074:Immunoglobulin G; D018836:Inflammation Mediators