Nivolumab-induced Central Nervous System (CNS) Vasculitis Brought on by a Steroid Taper
Nishika Karbhari1, Brahyan Galindo Mendez1, Nathaniel Robbins1
1Neurology, Dartmouth-Hitchcock
Objective:
To illustrate a case of nivolumab-induced CNS vasculitis that developed while tapering steroids and was successfully treated expectantly without immunosuppression
Background:
Immune checkpoint inhibitors (ICIs) with anti-PD-1/PD-L1 and anti-CTLA4 activity are associated with CNS vasculitis. Generally, both intra- and extracranial vasculitides are treated with steroids
Results:
A 75 year-old male received two months of concurrent chemoradiation with weekly carboplatin/paclitaxel for esophageal adenocarcinoma. Esophagectomy was considered, but the patient opted for active surveillance. Three years later, enlarging lung nodules heralded recurrent, metastatic esophageal cancer. Treatment with FOLFOX and nivolumab was initiated. Six months later, due to worsening fatigue, he was transitioned to nivolumab monotherapy. Over the following two weeks, he developed progressive dyspnea. Work-up suggested ICI-associated pneumonitis, and he was started on 50 mg prednisone, with weekly taper. A month later, at 30 mg daily, he developed confusion and was hospitalized. A brain MRI revealed multifocal lesions, some with ring-enhancement, concerning for metastases or abscess. Cerebellar biopsy demonstrated small-vessel CNS vasculitis, but given clinical improvement without treatment the patient was managed expectantly and returned back to baseline over two weeks
Conclusions:
This case of biopsy-proven ICI-associated CNS vasculitis developed while tapering steroids, and was treated conservatively. Steroids are first-line treatment for vasculitis; the development of vasculitis while tapering steroids thus raises the possibility of a rebound effect facilitating the development of vasculitis. Cases of CNS vasculitis that started while tapering steroids have been infrequently reported in the literature, but to our knowledge this is the first description of this phenomenon with ICI-associated CNS vasculitis. Additionally, this patient underwent biopsy with concern for metastasis or abscess, but ultimately did not necessitate treatment for vasculitis. This raises the possibility that active surveillance is a reasonable management strategy in cases of self-resolving ICI-associated CNS vasculitis