NeuroOp Guru: Neuroretinitis or papillitis? One disease in disguise

In this episode of NeuroOp Guru in collaboration with Ophthalmology Times, host Andrew G. Lee, MD, is joined by Drew Carey, MD, to discuss whether idiopathic recurrent neuroretinitis and idiopathic recurrent papillitis represent the same disease spectrum, drawing on a new multicenter retrospective cohort study.¹

Lee is the chair of the Blanton Eye Institute at Houston Methodist Hospital and a professor of ophthalmology, neurology, and neurosurgery at the Weill Cornell Medical College. Carey is the Neil R. Miller Rising Professor of Ophthalmology in the division of neuro-ophthalmology with the Wilmer Eye Institute at Johns Hopkins University School of Medicine in Baltimore, Maryland.

When one nerve looks like two different diseases

Carey noted that most cases of acute neuroretinitis present classically: a swollen optic nerve, macular exudates with cystoid macular edema (CME), and the telltale macular star, with roughly 60% to 70% of cases tied to Bartonella infection. But in about 30% of cases, an infectious workup comes back negative, leaving clinicians with a presumed autoimmune, relapsing condition—previously described as idiopathic recurrent neuroretinitis—without a confirmatory antibody test. Some patients instead present with recurrent optic disc edema alone, without CME, in a pattern some clinicians attribute to recurrent non-arteritic anterior ischemic optic neuropathy (NAION), particularly in younger patients without vascular risk factors.¹

Researchers at Mayo Clinic and the University of Minnesota pooled cases to characterize the clinical spectrum of these two conditions and evaluate whether they represent a single disease process.¹

The retrospective, multicenter cohort study included 30 patients diagnosed between January 2015 and October 2023, none of whom showed retrobulbar optic nerve enhancement on MRI during acute attacks. Ten patients had a mixed picture, with episodes of both idiopathic recurrent papillitis (disc edema without CME or exudates) and idiopathic recurrent neuroretinitis (disc edema with CME and exudates). Seven patients had only the neuroretinitis picture, and 13 had only the papillitis picture. Median age at first attack was similar between groups—34 years for neuroretinitis and 38 years for papillitis.¹

Carey noted that many patients present with CME on the first episode but recurrences that involve papillitis alone, a pattern he compares with relapsing-remitting conditions like sarcoidosis that can go ‘quiet’ for months or years before flaring again, requiring clinicians to weigh the risks and benefits of long-term immunosuppression. He noted the study’s limited data suggest mycophenolate mofetil may be more effective at reducing relapses than azathioprine or a TNF-alpha inhibitor, though he cautioned that the retrospective, 30-patient cohort makes it hard to gauge treatment response given how infrequently and unpredictably relapses occur.

He also noted the study did not address plasma exchange or IVIG in the acute phase, observing that because the condition is not demyelinating and no causative antibody has been identified, those modalities likely offer less benefit than they do in antibody-mediated optic neuritis, and that most patients respond well to steroids in the acute setting.

Reference

1. Hur M, Rattanathamsakul N, Jang S, et al. Clinical characteristics and chronic immunotherapy in idiopathic recurrent neuroretinitis and idiopathic recurrent papillitis: describing a potential new phenotype. J Neuroophthalmol. 2026;46(1):86-91. doi:10.1097/WNO.0000000000002371