Bilateral Orbital IgG4-Related Disease with Systemic and Corneal Involvement Showing an Excellent Response to Steroid and Rituximab Therapy: Report of a Case with 11 Years Follow-Up

Orbit. 2015;34(5):299-301. doi: 10.3109/01676830.2015.1057290. Epub 2015 Aug 18.

Abstract

IgG4-related disease is a newly recognized fibro-inflammatory condition. The purpose of this report is to present a patient with 11 years of follow-up, who revealed characteristic features of IgG4-related disease with systemic, orbital and corneal involvement and showed a favorable response to steroids and rituximab treatment.

Keywords: Bilateral IgG4-related orbital disease; peripheral ulcerative keratitis; sclerosing autoimmune pancreatitis; steroid and rituximab treatment.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / drug therapy
  • Corneal Ulcer / diagnosis*
  • Corneal Ulcer / drug therapy
  • Drug Combinations
  • Follow-Up Studies
  • Glucocorticoids / therapeutic use*
  • Humans
  • Immunoglobulin G / blood*
  • Immunologic Factors / therapeutic use
  • Male
  • Orbital Diseases / diagnosis*
  • Orbital Diseases / drug therapy
  • Pancreatitis / diagnosis*
  • Pancreatitis / drug therapy
  • Paraproteinemias / diagnosis*
  • Paraproteinemias / drug therapy
  • Plasma Cells / pathology
  • Rituximab / therapeutic use*
  • Tomography, X-Ray Computed

Substances

  • Drug Combinations
  • Glucocorticoids
  • Immunoglobulin G
  • Immunologic Factors
  • Rituximab