title: Fulminant bilateral papilloedema during low-dose steroid taper in a child with systemic idiopathic arthritis treated with tocilizumab.
abstract: Systemic juvenile idiopathic arthritis (SJIA) is one of the most severe forms of arthritis that affects children younger than 16 years of age at onset. SJIA often requires corticosteroids to control the inflammation. However, long-term corticosteroid use may have adverse effects, including intracranial hypertension (IH). Biologic therapies have been used as corticosteroid sparing agents. We report the first case of a child with steroid-dependent SJIA treated with tocilizumab, an IL-6 receptor monoclonal antibody, who developed fulminant IH, bilateral papilloedema and vision loss when oral prednisone was weaned from 2 to 1 mg per day. Despite repeated lumbar punctures and high dose acetazolamide, he required urgent unilateral optic nerve sheath fenestration (ONSF). This endoscopic surgical intervention released the pressure exerted by the cerebrospinal fluid on the optic nerve and stopped the progression of vision loss. Nine weeks after the diagnosis of bilateral papilloedema, his vision was completely restored in one eye and partially recovered in the contralateral one. Long-term treatment with corticosteroids even at very low dose and tocilizumab may predispose to severe IH, papilloedema and vision loss. The role that tocilizumab might have played in this case in unclear. Early recognition and prompt treatment of papilloedema is crucial in avoiding permanent vision loss. Fulminant papilloedema in an immunocompromised child carries additional significant challenges. Early ONSF is a safe and effective intervention in refractory papilloedema. Children with severe papilledema secondary to IH should be managed by a multidisciplinary team in tertiary centres.
authors.affiliation: University of Western Ontario, London; and Department of Ophthalmology, London Health Sciences Center, London, ON, Canada.
authors.forename: Lulu
authors.initials: L
authors.lastname: Burstzyn
authors.affiliation: University of Western Ontario, London; Division of Paediatric Neurology; and Department of Paediatrics, London Health Sciences Center, London, ON, Canada.
authors.forename: Simon
authors.initials: S
authors.lastname: Levin
authors.affiliation: University of Western Ontario, London; and Department of Otolaryngology, London Health Sciences Center, London, ON, Canada.
authors.forename: Brian
authors.initials: B
authors.lastname: Rotenberg
authors.affiliation: University of Western Ontario, London; and Department of Paediatrics; London Health Sciences Center, London, ON, Canada.
authors.forename: Tamara
authors.initials: T
authors.lastname: Van Hooren
authors.affiliation: University of Western Ontario, London; and Department of Diagnostic Radiology and Nuclear Medicine, London Health Sciences Center, London, ON, Canada.
authors.forename: Andrew
authors.initials: A
authors.lastname: Leung
authors.affiliation: University of Western Ontario, London; Department of Paediatrics; and Paediatric Rheumatology, London Health Sciences Center, London, ON, Canada.
authors.forename: Roberta
authors.initials: R
authors.lastname: Berard
authors.affiliation: University of Western Ontario, London; Department of Paediatrics; and Paediatric Rheumatology, London Health Sciences Center, London, ON, Canada. dardelea@uwo.ca.
authors.forename: Daniela S
authors.initials: DS
authors.lastname: Ardelean
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D001171:Arthritis, Juvenile / Q000188:drug therapy*; D002648:Child; D004359:Drug Therapy, Combination; D006801:Humans; D008297:Male; D010211:Papilledema / Q000139:chemically induced*; D011241:Prednisone / Q000009:adverse effects* / Q000627:therapeutic use; D013375:Substance Withdrawal Syndrome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C502936:tocilizumab; D011241:Prednisone