C40-31 Neuro-ocular Sarcoidosis - A Diagnostic and Therapeutic Dilemma

Abstract Central nervous system sarcoidosis often requires providers to be creative while navigating treatment challenges. We present a case of progression to neuro-ocular sarcoidosis with challenges in therapeutic treatment. A 65-year-old male with a known past medical history of sarcoidosis developed a persistent, migrating headache, left-sided facial weakness and numbness, blurry vision, diplopia, and left eye pain. He presented to the emergency department and underwent a stroke evaluation. Initial labs showed elevated liver function enzymes with normal bilirubin and elevated ESR and CRP with normal thyroid function tests. His brain MRI revealed T2 hyperintensity lesions of the left ocular muscles with extension into the optic nerve sheath and involvement of the left and right scalp. Spinal fluid analysis was normal. Given the concern for progression of underlying pulmonary sarcoidosis to neuro-ocular sarcoidosis, the patient was given pulse dose steroids for three days with improvement of symptoms. A CT chest, abdomen, and pelvis revealed cirrhosis, consistent with the elevated liver function enzymes on admission. Treatment plans for outpatient infliximab with a steroid taper bridge were made. The patient was started on dapsone prophylaxis for Pneumocystis. He had a delay in initiating infliximab. At clinic follow up he was found to have a methemoglobin level of 6.7% and low pulse oximetry, requiring dapsone cessation. This patient’s initial delay in infliximab creates a significant challenge in transitioning to a steroid-sparing agent further complicated by methemoglobinemia on dapsone. While methotrexate has excellent activity in neuro-ocular sarcoidosis, this patient has significant concern for liver disease, limiting its use. Other DMARD agents, such as mycophenolate, have increased neurosarcoidosis relapse risk. Additionally, this patient is at significant risk for opportunistic infections given the challenges in prescribing prophylactic antimicrobials. Pentamadine and atovaquone present alternate possibilities although less efficacious. This case highlights multiple challenges associated with treating neuro-ocular sarcoidosis. Other than infliximab and methotrexate, few nonsteroidal agents capable of controlling neurosarcoidosis exist. Also, pertinent factors make antimicrobial prophylaxis for steroid treatment in this patient difficult. Sarcoidosis, particularly its more rare and severe subtypes like neurosarcoidosis, requires a unique balance between therapeutic efficacy, treatment side effects, and patient-specific limitations. While research is continuing to advance in the areas of therapy, further research is desperately needed to find additional effective agents for controlling neuro-ocular sarcoidosis. This abstract is funded by: None