A case of IgG4-related conjunctival tumor with severe systemic allergy treated with antibodies against cytokine receptors

PURPOSE: To present a case of IgG4-related conjunctival tumor in which anti-IL-5 receptor alpha-chain and anti-IL-4 receptor alpha-chain antibodies were administered for eosinophil sinusitis and severe bronchial asthma, but conjunctivitis could not be controlled. OBSERVATIONS: A 53-year-old male patient started to receive anti-IL-5 receptor alpha chain antibody to treat eosinophilic sinusitis and eosinophilic severe bronchial asthma. Several months later, proliferative changes of the right palpebral conjunctiva appeared and were treated with tacrolimus and betamethasone eye drops. However, the findings gradually worsened and the right upper palpebral conjunctiva remained exposed. Exposed tissue was resected and histopathological examinations revealed the presence of IgG4 positive cells (10>/400HPF). Anti-IL-5 receptor alpha chain antibody treatment was stopped and changed to prednisolone. The findings improved and anti-IL-4 receptor alpha chain antibody was added to control eosinophilic sinusitis and eosinophilic severe bronchial asthma. The lesions worsened when the prednisolone was reduced under treatment with anti-IL-4 receptor alpha-chain antibody. CONCLUSIONS AND IMPORTANCE: In this case, the proliferative changes could not be suppressed by treatment targeting IL-5R receptor alpha-chain and IL-4R receptor alpha-chain, suggesting that the patient had VKC-like severe allergic conjunctivitis as a manifestation of IgG4-related disease. Additionally, it should be noted that if the severe conjunctivitis cannot be suppressed by treatment with immunosuppressive eye drops or various systemic biological agents, the conjunctivitis may be a manifestation of IgG4 related disease.