title: IgG4-related disease: a rheumatologist's perspective.
abstract: OBJECTIVE
  Given that the clinical features of several IgG4-related diseases (IgG4-RD) can mimic those of autoimmune disorders, the aim of this study was to find possible distinguishing characteristics that would help us identify such cases from the pool of patients in a rheumatology clinic.
  
  
  METHODS
  From our clinic's medical records, we identified patients who fulfilled the recently published diagnostic criteria for IgG4-RD. We recorded their presenting features, co-morbid conditions, laboratory, radiologic and histologic findings as well as their treatment and outcome.
  
  
  RESULTS
  We identified 11 cases of IgG4-RD: 4 cases of IgG4-related autoimmune pancreatitis (AIP), 5 cases of IgG4-related retroperitoneal fibrosis (RPF)/ periaortitis, 2 cases of IgG4-related sialadenitis and one of IgG4-related interstitial nephritis. 5 out of the 11 patients had been diagnosed with an autoimmune disease, namely rheumatoid arthritis (RA), Sjogren's syndrome (SS) and antiphospholipid syndrome (APS). 3 out of 11 patients were subsequently diagnosed with neoplastic disorders. All patients with IgG4-related AIP had raised CRP levels at presentation. Presenting features of RPF/periaortitis patients were constitutional symptoms, abnormal renal function, hypertension and back pain. Patients with IgG4-related sialadenitis had clinical features mimicking SS. The majority of patients had a favourable response to steroids.
  
  
  CONCLUSIONS
  We present common IgG4-RD presentations in the setting of a rheumatology clinic. Increased awareness may avoid delay in diagnosis.
authors: Euroclinic Hospital, Athens, Greece. fotinisoliotis@doctors.org.uk.
authors: Fotini
authors: F
authors: Soliotis
authors: Clio P
authors: CP
authors: Mavragani
authors: Sotiris C
authors: SC
authors: Plastiras
authors: Dimitra
authors: D
authors: Rontogianni
authors: Fotini N
authors: FN
authors: Skopouli
authors: Haralampos M
authors: HM
authors: Moutsopoulos
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D015415:Biomarkers / Q000097:blood; D003937:Diagnosis, Differential; D042241:Early Diagnosis; D005260:Female; D006115:Greece; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012216:Rheumatic Diseases / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D007074:Immunoglobulin G; D013256:Steroids