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TitleExacerbation of ocular paraneoplastic syndromes with Immune Checkpoint inhibitors (ICI)
PurposeTo describe different ocular paraneoplastic syndromes in patients treated with ICI, its relation with different types of ICI and different types of tumors and its implications for treatment.
MethodsDifferent real-world cases are described, completed with findings in literature.
ResultsPatients treated with ICI, can present with different ocular paraneoplastic syndromes, such as Carcinoma Associated Retinopathy(CAR), Melanoma Associated Retinopathy (MAR) and paraneoplastic Acute Exudative Polymorphous Vitelliform Maculopathy (pAEPVM). In our cases, the different types of ocular paraneoplastic syndromes are related to different types of primary tumors, with MAR and pAEPVM seen in melanoma and CAR in lungcancer, irrespective of the type of ICI. Visual prognosis is limited in MAR and CAR.
ConclusionParaneoplastic disorders result from an anti-tumor immune response against a shared autoantigen between the tumor and ocular tissue. ICI enhance the anti-tumor immune response, which can lead to increased cross-reaction against ocular structures and unmasking of a predisposed paraneoplastic syndrome. Different types of primary tumors are related to different crossreactive antibodies. Therefore the different types of paraneoplastic syndromes are related to different types of primary tumors and are probably unrelated to the type of ICI. ICI-related paraneoplastic syndromes often lead to an ethical dilemma. Continuation of ICI treatment can lead to irriversible visual loss in MAR and CAR. In these cases overall survival must be weighed against quality of life. In pAEPVM however, the vitelliform lesions can disappear with tumor control, which may involve continuation of ICI.
Conflict of interestNo
Authors 1
Initials of first name(s)PP