Dacryoadenitis and Sjögren-like Symptoms Caused by Nivolumab

A 70-year-old woman, treated with nivolumab for metastatic malignant melanoma, presented with bilateral swelling of her upper eyelids. Physical examination revealed a marked enlargement of both lacrimal glands (panel A). Slit-lamp examination showed a marked reduction in tear breakup time in OU and superficial punctate keratopathy (panel B). The Schirmer test results were 1 mm bilaterally, indicating severe dry eyes. The gum test revealed decreased saliva production (8 ml per 10 minutes). Blood tests revealed normal IgG4, anti-Ro/SS-A, and La/SS-B levels. Gadolinium-enhanced MRI revealed bilateral enlargement of the lacrimal and parotid glands (panels C and D). Histopathology of a right lacrimal gland biopsy revealed extensive inflammatory cell infiltrate, composed predominantly of lymphocytes and plasma cells (panel E). Immunohistochemical staining showed that the lymphocytes were predominantly CD20-positive B cells, with sparse IgG4-positive plasma cells. Based on these findings, the patient was diagnosed with immune checkpoint inhibitor-induced dacryoadenitis and parotitis, resulting in Sjögren syndrome-like symptoms. She was treated with oral prednisolone (30 mg/d), which resulted in a reduction in the size of both lacrimal glands, but the severe dry eye symptoms persisted. Immune checkpoint inhibitors often cause sicca syndrome, characterized by dry eyes and xerostomia, accompanied by inflammatory cell infiltration, particularly CD3 T cells, in glandular tissues. However, in this case, the infiltrating cells were primarily CD20-positive B cells, and the lacrimal and parotid glands were destroyed by acute inflammation, resulting in Sjögren syndrome-like symptoms. Physicians should be aware that immune checkpoint inhibitors can induce dacryoadenitis and sialadenitis, leading to symptoms resembling Sjögren syndrome.