Introduction Immune checkpoint inhibitors (ICIs) have improved prognosis in advanced malignancies; however, they may be associated with extensive ocular immune-related adverse events (irAEs) that are sight threatening. Our study aimed to identify the presentation, characteristics, management, and clinical outcomes of ocular irAEs. Methods: In this retrospective, observational case series, we reviewed the medical records of 1280 patients at a large US tertiary cancer center between 2010 and 2020. Results: We identified 130 patients who presented with ocular irAEs (10%) with 69 males (53%) and 61 females (47%). The mean time to toxicity was 6.1 months. Adverse events include corneal toxicity (31%), neuro-ophthalmic (14%), uveitis and scleritis (13%), retinopathy (13%), periocular disorders (11%), and others. IrAEs occurred most frequently with nivolumab (26%). Most ocular irAEs were treated with topical therapy. Advanced cases required systemic corticosteroids and even cessation of ICIs. Conclusion: Our cohort is a large case series highlighting the increased potential of ocular toxicity associated with ICIs. Prompt recognition and management of ocular irAEs can minimize their effect.

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Evans syndrome is a rare autoimmune disorder where patients develop autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), and less commonly immune neutropenia. Patients typically present with fatigue, pallor, jaundice, petechiae, or epistaxis. A 27-year-old man with a history of atopic dermatitis for which he recently began treatment with dupilumab presented to the emergency department with a headache and blurry vision. Multiple Roth spots were seen on fundoscopic examination. Laboratory studies were consistent with warm AIHA, confirmed by a positive direct antiglobulin test (DAT), and severe thrombocytopenia. He was diagnosed with Evans syndrome. He was treated with corticosteroids, rituximab, and intravenous immunoglobulin (IVIG). His recovery was prolonged with the slow improvement of anemia and thrombocytopenia. This is an atypical presentation of Evans syndrome with isolated symptoms of new-onset blurry vision and headache along with the finding of Roth spots. Another interesting feature in the case is the recent use of dupilumab. Dupilumab is a monoclonal antibody that inhibits the T-helper cells type 2 (Th2) signaling pathway by blocking interleukin (IL)-4 and IL-13 binding. This alteration in the immune response could have a role in the development of Evans syndrome.

Purpose: This work reports a case of Evans syndrome first presenting with ophthalmic signs. Methods: A 27-year-old previously healthy man presented with headaches and bilateral blurred vision for 2 weeks. The visual acuity was 20/30-1 and 20/60-2 in the right eye and left eye, respectively. A fundus examination revealed Roth spots, diffuse multilayer retinal hemorrhages in the macula and periphery, and tortuous vessels in both eyes. Optical coherence tomography showed a disrupted foveal contour from intraretinal fluid and hemorrhage in both eyes. Fluorescein angiography demonstrated dilated and tortuous vessels with scattered blocking defects from hemorrhages. Results: A workup revealed warm hemolytic anemia with severe thrombocytopenia consistent with the diagnosis of Evans syndrome. Conclusions: Evans syndrome is a rare blood dyscrasia that can first present as subacute vision loss and should be added to the differential of diffuse bilateral retinal hemorrhages spanning a multitude of retinal layers.