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

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

Author information

1
IDIBGI-Institut d'Investigació Biomèdica de Girona Dr. Josep Trueta (IDIBGI), Parc Hospitalari Martí i Julià de Salt-Edifici.
2
Ophthalmology Department, Oculoplastics.
3
Pathology Department.
4
Internal Medicine Department, Hospital Universitari de Girona Dr. Josep Trueta, Avinguda de França.
5
Radiology Department, Centre d'Atenció Primària (CAP) Güell, Carrer de Joan Pons, Girona, Spain.
6
Neuroophthalmology, Hopitaux Universitaires de Geneve (HUG), Rue Gabrielle-Perret-Gentil, Geneva, Switzerland.

Abstract

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.

PMID:
30278491
PMCID:
PMC6181517
DOI:
10.1097/MD.0000000000012197
[Indexed for MEDLINE]
Free PMC Article

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