• Stephanie Lemaitre, Gemma Mateu Esquerda, Antoni Castro Guardiola, Jordi Teruel Agustin, Nicolae Sanda, and Miguel González-Candial.
• IDIBGI-Institut d'Investigació Biomèdica de Girona Dr. Josep Trueta (IDIBGI), Parc Hospitalari Martí i Julià de Salt-Edifici.
• Medicine (Baltimore). 2018 Sep 1; 97 (39): e12197.

RationaleImmunoglobulin 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 ConcernsA 78-year old man presented with unilateral proptosis responsive to steroids, initially attributed to nonspecific orbital inflammation.DiagnosisRight 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.InterventionsBoth the orbital inflammation and abdominal mass infiltrating the mesentery were responsive to steroids and rituximab administered to treat IgG4-RD.OutcomesIn 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.LessonsThis 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.

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