title: Description of patients with IgG4-related disease from a Hungarian centre.
abstract: OBJECTIVE
  Although most reported patients with immunoglobulin G4-related disease (IgG4-RD) are from the Far East, we aimed to identify patients suffering from IgG4-RD in our University Centre in Debrecen, Hungary.
  
  
  METHODS
  Serum IgG4 levels were measured at 51 of our 800 patients followed up because of Sjögren's syndrome (SS) if one or more clinical signs during the disease course raised the possibility of IgG4-RD (persisting salivary gland swelling, absence of anti-Ro/SSA and anti-La/SSB antibodies in the serum, and positive salivary gland biopsy, coexistence of autoimmune pancreatitis, autoimmune hepatitis, or primary sclerosing cholangitis, persisting lymphadenopathy). Where available, histological samples of small salivary gland biopsies were revised to detect the particular features of IgG4-RD. Pathologists and surgeons were informed about the disease and asked to refer suspicious cases.
  
  
  RESULTS
  Based on our survey, eight patients were identified with IgG4-RD. Pancreatic, salivary gland, aortic, and retroperitoneal manifestations were detected. Of the 51 patients with SS, four appeared to have IgG4-RD, but eventually one was excluded.
  
  
  CONCLUSIONS
  Although IgG4-RD is not yet well known to physicians of Western countries, it occurs in Caucasians and probably in other races as well. Moreover, our eight cases diagnosed with IgG4-RD demonstrate a relatively large European patient population collected in a single centre. European clinicians, and especially rheumatologists, should be informed and at least certain laboratories should be prepared to investigate patient samples if the suspicion of IgG4-RD is raised. The main clinical significance of an accurate diagnosis is the extreme corticosteroid sensitivity of IgG4-RD.
authors.affiliation: Department of Medicine, Division of Clinical Immunology, University of Debrecen Medical and Health Science Centre , Hungary.
authors.forename: A
authors.initials: A
authors.lastname: Szántó
authors.forename: G
authors.initials: G
authors.lastname: Nagy
authors.forename: C S
authors.initials: CS
authors.lastname: Molnár
authors.forename: Z
authors.initials: Z
authors.lastname: Griger
authors.forename: T
authors.initials: T
authors.lastname: Tarr
authors.forename: M
authors.initials: M
authors.lastname: Zeher
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology; D001660:Biliary Tract Diseases / Q000175:diagnosis* / Q000276:immunology; D005260:Female; D006801:Humans; D006814:Hungary; D007074:Immunoglobulin G / Q000097:blood*; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010182:Pancreatic Diseases / Q000175:diagnosis* / Q000276:immunology; D012187:Retroperitoneal Space / Q000473:pathology*; D012466:Salivary Gland Diseases / Q000175:diagnosis* / Q000276:immunology; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000276:immunology
chemical_list: D007074:Immunoglobulin G