447. TNF-α Inhibitors in the Treatment of Neurobehçet Syndrome: A Case Series and Literature Review

Background/ Objectives: Behçet’s syndrome is a systemic inflammatory endothelialopathy which can result in a variable vessel vasculitis.  It is characterized by recurrent mucocutaneous ulcers, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease and/or arthritis. Neurobehçet syndrome (NBS) occurs in 5% of cases. NBS is associated with parenchymal or vascular involvement, with parenchymal imaging changes accounting for 75-80% of cases. There is a paucity of evidence supporting treatment of NBS. Our objective was to review the course of three patients with parenchymal NBS who were treated with TNF-α inhibitors (TNFi).

Methods: Three cases of NBS treated with TNFi are presented and a review of the literature was performed.

Results: Case 1. A 28-year-old Chinese male with a prior left midbrain and pons ischemic stroke (February 2020) and right basal ganglia intracerebral hemorrhage (January 2021) presented to the outpatient rheumatology clinic with new visual symptoms in June 2021. Ophthalmologic evaluation revealed retinal vasculitis with periphlebitis. Cerebrospinal fluid (CSF) revealed increased cells but negative for typical and atypical infection.  Further history revealed intermittent oral ulcers since childhood. HLA-B*51 was positive. He was diagnosed with NBS and started on pulse methylprednisolone, azathioprine, and adalimumab. At 4 months, his disease remains in remission, with improvement of ocular inflammation. Repeat CSF analysis demonstrated normalization of cell count and protein. Case 2. A 45-year-old Caucasian female with suspected Behçet’s syndrome presented to hospital in September 2021 with dyspnea, anemia and diplopia. A few weeks prior, she started azathioprine, which was thought to be the cause of anemia and was held. She described a history of intermittent oral and vaginal ulcers.  Physical exam confirmed oral ulcers, ulcerating cutaneous lesions, and cranial nerve 6^th palsy. CT PE revealed multiple bilateral pulmonary embolisms. MRI brain revealed cranial nerve VI enhancement and bilateral pontine enhancing lesions. CSF analysis demonstrated elevated protein with normal cell count. HLA-B*51 was negative. She was diagnosed with NBS and started on pulse methylprednisolone and infliximab 5 mg/kg IV. Treatment response is too premature to assess at time of abstract submission. Case 3. An 18-year-old South Asian male presented with fever, pharyngitis, and severe headaches in August 2019. Prior history included oral ulcers starting at age 6, followed by onset of erythema nodosum, painful genital ulcers, bilateral uveitis, retinal hemorrhages and epididymitis at age 15. In hospital, CSF analysis revealed elevated WBCs and protein, with negative cultures. MR revealed hyperintensity in the right hippocampal region. HLA-B*51 was positive. He was diagnosed with NBS and started on pulse steroids. Azathioprine was added as an outpatient. With tapering steroids, he experienced recurrent erythema nodosum and epididymitis. Adalimumab was added in December 2019, and azathioprine stopped in December 2020. He remains in remission on adalimumab 40 mg sc every 2 weeks and prednisone 5 mg daily.Literature Review: A literature search for treatment of NBS was performed.  Full manuscripts describing the use of TNFi for treatment of 2 or more NBS patients were included.  Abstracts and case reports were excluded.  Eight papers, describing a total of 70 NBS patients were identified.  Infliximab was most commonly utilized (61 patients, 87%), with adalimumab described in 9 patients (13%.) TNFi were effective, with 92.8% of patients experiencing complete or partial remission.  See Table 1 for details.

Conclusions: NBS is a rare manifestation of an uncommon disease. Parenchymal NBS may be treated with glucocorticoids and azathioprine, however TNFi appear effective in those with severe or refractory disease, and ongoing studies may provide additional insight into their utility.

Disclosures: None