Colon cancer and IgG4-related disease with orbital inflammation and bilateral optic perineuritis: A case report

Medicine (Baltimore). 2018 Sep;97(39):e12197. doi: 10.1097/MD.0000000000012197.


Rationale: Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory condition of unknown cause. Cancer might be related to the development of certain IgG4-RD but to date, little literature documents it.

Patient concerns: A 78-year old man presented with unilateral proptosis responsive to steroids, initially attributed to nonspecific orbital inflammation.

Diagnosis: Right hemicolectomy was performed because of a suspicious lesion which turned out to be tubulovillous adenoma on histological analysis. Eight months after the surgery, a mass infiltrating the mesentery was found and biopsy revealed IgG4-RD.

Interventions: Both the orbital inflammation and abdominal mass infiltrating the mesentery were responsive to steroids and rituximab administered to treat IgG4-RD.

Outcomes: In the course of IgG4-RD, the patient developed bilateral optic perineuritis, causing bilateral visual loss. Colon cancer with synchronous multiple liver metastases was found 1 year after rituximab treatment.

Lessons: This case raises the possibility of IgG4-RD being a paraneoplastic syndrome in some patients. Cancer screening should probably be performed in some elderly patients diagnosed with IgG4-RD.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / drug therapy
  • Colectomy / methods
  • Colonic Neoplasms / complications*
  • Colonic Neoplasms / surgery
  • Diagnosis, Differential
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoglobulin G / immunology
  • Immunologic Factors / therapeutic use
  • Magnetic Resonance Imaging
  • Male
  • Optic Neuritis / complications*
  • Optic Neuritis / drug therapy
  • Orbital Diseases / complications*
  • Orbital Diseases / drug therapy
  • Rituximab / therapeutic use
  • Tomography, X-Ray Computed


  • Glucocorticoids
  • Immunoglobulin G
  • Immunologic Factors
  • Rituximab