title: IgG4-related Disease Masquerading as Recurrent Scleritis and Chronic Conjunctivitis.
abstract: OBJECTIVE
  To report atypical ophthalmologic manifestations and complications of IgG4-related disease (IgG4-RD).
  
  
  METHODS
  Patients with isolated ophthalmologic involvement of IgG4-RD other than lacrimal or orbital infiltration seen between 2009 and 2011 in a single tertiary center were retrospectively reviewed and their clinical and histological features, treatment, and prognosis were studied.
  
  
  METHODS
  Two patients (mean age 56.5 years) were included. One patient presented with recurrent anterior and posterior scleritis, and one patient had chronic conjunctival infiltration. Histopathology demonstrated lymphoplasmacytic proliferation with overexpression of IgG4(+) plasma cells. Both patients initially responded to a high dose of oral corticosteroids (1 mg/kg/d). However, one patient required the adjunction of methotrexate and one patient developed an intra-epithelial conjunctival carcinoma on the site of the initial lesion.
  
  
  CONCLUSIONS
  Patients with atypical presentation of IgG4-RD, such as chronic conjunctival infiltration or scleritis, can suffer from considerable diagnostic delay leading to fibrosis or malignancy development. We report the first case of conjunctival carcinoma in a patient with IgG4-RD.
authors.affiliation: Ophthalmology Department, Pitie-Salpetriere Hospital , Paris , France .
authors.forename: Elise
authors.initials: E
authors.lastname: Philippakis
authors.forename: Nathalie
authors.initials: N
authors.lastname: Cassoux
authors.forename: Frédéric
authors.initials: F
authors.lastname: Charlotte
authors.forename: Phuc
authors.initials: P
authors.lastname: LeHoang
authors.forename: Bahram
authors.initials: B
authors.lastname: Bodaghi
authors.forename: Coralie
authors.initials: C
authors.lastname: Bloch-Queyrat
authors.forename: Valérie
authors.initials: V
authors.lastname: Touitou
mesh_terms: D000888:Antibodies, Anti-Idiotypic / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology; D002908:Chronic Disease; D003231:Conjunctivitis / Q000175:diagnosis*; D057210:Delayed Diagnosis; D003937:Diagnosis, Differential; D005128:Eye Diseases / Q000175:diagnosis* / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology* / Q000473:pathology; D012189:Retrospective Studies; D015423:Scleritis / Q000175:diagnosis*
chemical_list: D000888:Antibodies, Anti-Idiotypic; D007074:Immunoglobulin G; C049937:anti-IgA
keywords: Conjunctivitis; IgG4-related disease; in situ carcinoma; scleritis