Abstract The purpose of this case series is to delineate the rationale for employing oral cyclophosphamide along with rituximab in three patients with nonparaneoplastic autoimmune retinopathy (npAIR) secondary to birdshot chorioretinopathy. Case 1: A 50-year-old male with a history of amblyopia in the right eye (OD) presented with floaters and reduced vision in the OD. He was diagnosed with birdshot chorioretinopathy (BSCR) in both eyes (OU). He was successfully treated with cyclosporine and mycophenolate mofetil; however, later, he developed npAIR. He started rituximab infusion and oral cyclophosphamide. After 7 months of treatment, oral cyclophosphamide was stopped due to severe side effects. Case 2: A 58-year-old male presented with an acquired color vision problem in the left eye. He was diagnosed with BSCR in OU. He responded to a clinical trial study for BSCR; however, later, he was diagnosed with npAIR. This patient failed oral cyclophosphamide therapy due to ineffectiveness and side effects. He, subsequently, failed rituximab and bortezomib therapy. Case 3: A 52-year-old female was diagnosed with BSCR. She was successfully treated with mycophenolate mofetil and infliximab for BSCR. She, subsequently, was diagnosed with npAIR, which failed oral cyclophosphamide therapy due to ineffectiveness and side effects and was successfully treated with rituximab and intravenous immunoglobulin infusions. The inclusion of oral cyclophosphamide into the treatment regimen for patients with npAIR secondary to BSCR may raise concerns regarding its safety and efficacy, suggesting the need for careful consideration and evaluation during treatment.
Morvey et al. (Thu,) studied this question.