title: [IgG4-related orbital inflammation].
abstract: BACKGROUND
  Serum immunoglobulin G4 (IgG4)-related systemic disease is a newly found entity and should be considered as a further differential diagnosis to the big pool of unspecific orbital inflammation. We describe clinical appearance and treatment options.
  
  
  METHODS
  We retrospectively analyzed all patients with the diagnosis IgG4-related orbital inflammation who were examined in our outpatient clinic. We compared symptoms, clinical and histological findings and therapy.
  
  
  RESULTS
  In the last 17 months five patients were diagnosed with IgG4-related orbital disease. Biopsies were performed of most affected areas as seen in the computerised tomography scan. Histology revealed marked infiltration with IgG4-positive plasma cells. Serum IgG4-levels were partly significantly elevated. Based on these findings diagnosis of an IgG4-related disease was agreed and treatment with oral prednisone installed. In one patient a therapy with Rituximab was successfully started.
  
  
  CONCLUSIONS
  Extended unspecific orbital inflammation, especially when bilateral and associated with systemic disease should be considered to be IgG4-related. Histological proof of IgG4-positive plasma cells and elevated IgG4 serum levels do confirm this diagnosis. Strict follow-up and interdisciplinary work-up is recommended.
authors: University Hospital Zurich, Division of Ophthalmology, Zurich, Switzerland. caterina.schweier@usz.ch
authors: C
authors: C
authors: Schweier
authors: H
authors: H
authors: Kordic
authors: K
authors: K
authors: Chaloupka
mesh_terms: D000293:Adolescent; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D003937:Diagnosis, Differential; D009877:Endophthalmitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D008875:Middle Aged
chemical_list: D000893:Anti-Inflammatory Agents; D007074:Immunoglobulin G