Disease of the Year for 2023: Pediatric Uveitis

As 2023 is drawing to a close, the editors of Ocular Immunology and Inflammation (OII) are proud to present to our readers the special edition of our journal dealing with pediatric uveitis as the final issue of the year. To our knowledge, this is the first time that the disease of the year edition has been published in hard copy before the end of the nominated year and this bears testament to the tireless work of everyone involved with this journal. From the editors to the editorial board, the review board, reviewing editors and reviewers, the invited authors and the production team, everyone has played their part in bringing about this novel feat, and we thank you for that.

Readers will notice that we have received contributions from many different parts of the world giving a truly international flavor to this issue. In this issue, there are articles describing different themes within pediatric uveitis which we would like to highlight.

The first theme looks at epidemiology and patterns of disease from different corners of the world. In one of our invited reviews, Gentile and colleagues provided a comprehensive review of what has been published on the epidemiology of pediatric uveitis to date.¹ In addition, we have included more recent studies from a few different countries to broaden our knowledge of this topic. From Turkey, we have the second report of the national registry on pediatric uveitis outlining the demographic and clinical profiles of 442 children with uveitis in that country where 75.3% had non-infectious uveitis.² In another manuscript, we have data from 388 children with uveitis in Egypt which showed that intermediate uveitis was the most common anatomical form.³ Kardes and colleagues, again from Turkey, described the risk factors for ocular compilations and visual loss in 51 children with Juvenile Idiopathic Arthritis-associated uveitis (JIAU).⁴ From Taiwan, Wu and colleagues described the epidemiology, treatment, and outcomes of JIAU in 44 children with a mean age of 11.1 years.⁵ Khazaei and colleagues described the epidemiology and clinical course of pediatric uveitis in 97 patients at a tertiary referral center in northeastern Iran. Uveitis was found to be predominantly anterior (43%) and non-infectious (82.5%).⁶ Lastly, Fukuda and colleagues submitted their data from 80 patients (140 eyes) who presented with pediatric uveitis to a tertiary referral center in Western Japan. Most patients were female and tubulointerstitial nephritis (11.3%) and JIAU (10%) were the most frequent causes identified. Only 7.6% of patients had infectious uveitis.⁷

The next theme considers different non-infectious and infectious disease entities within the scope of pediatric uveitis. Markomichelakis and colleagues looked at the long-term outcomes and complications of different types of non-infectious uveitis in 296 children from first presentation to 10-year follow-up. Most children (53.4%) in this Greek population presented with anterior uveitis and the leading diagnosis was idiopathic uveitis.⁸ Maccora and colleagues reported demographic, clinical features and outcomes of 126 children with idiopathic non-infectious uveitis in a multicenter international cohort. This study showed a slight male preponderance, with most cases being bilateral (84.1%) and anterior (54.0%).⁹ In an invited review, van Meerwijk and colleagues have outlined the screening strategies for and clinical management of JIAU.¹⁰ They also elaborated on current molecular markers for the disease and focused on glaucoma as a common cause of vision loss in JIAU. In another invited review, Khochtali and colleagues provided a contemporary overview of pediatric pars planitis with a stepladder approach to the management of this condition.¹¹ Janetos and colleagues were invited to submit a review dealing with tubulointerstitial nephritis and uveitis (TINU) in children. They have provided us with an update on the epidemiology, diagnosis, prognosis, and treatment of this disease which was originally described in 1975.¹² Another invited review reminds our readers that children may also be affected by sarcoidosis, even though it typically occurs in adults. The two distinct clinical presentations, associated with younger and older children, are thought to have different causes and are described in detail.¹³ Monogenic autoinflammatory syndromes (MAISs) result from pathogenic genetic variants in the innate immune system. The diagnosis and management of MAISs can be challenging, and we have therefore included a review of the clinical features of MAISs along with different treatment options.¹⁴ Lastly, we also invited a review of the different infections that may present with posterior uveitis in children.¹⁵ This review provides a detailed description of conditions such as cat-scratch disease, tuberculosis, toxocariasis, toxoplasmosis, and viral diseases.

Treatment of different forms of non-infectious uveitis also emerged as a theme in this issue. Sonmez and colleagues described their experience using adalimumab (ADA) to achieve prompt, sustained suppression of non-infectious uveitis in a pediatric population where traditional management options were unsuccessful. They included 16 patients (31 eyes) and demonstrated that visual acuity, intraocular inflammation, vitreous haze, and central macular thickness all improved after initiation of ADA treatment.¹⁶ Staying with ADA, Hiyama and colleagues shared their data on the safety and efficacy of adalimumab to treat non-infectious uveitis in 48 patients.¹⁷ They found that combined ADA and methotrexate (MTX) treatment was required in 71.4% of sympathetic ophthalmia and Vogt-Koyanagi-Harada (VKH) disease cases but in only 18.0% of patients with Behçet’s disease was additional MTX required. Gueudry and colleagues reported 11/27 children treated with ADA for non-infectious chronic anterior uveitis who required escalation to weekly dosing to gain control of the inflammation.¹⁸ The use of subcutaneous tocilizumab in 13 patients with refractory JIAU was described by Marino and colleagues.¹⁹

Glaucoma is a well-known sequela of non-infectious uveitis in general and JIAU specifically. A group from the Netherlands conducted a retrospective case–control study to identify factors associated with the need for glaucoma surgery in patients with pediatric uveitis.²⁰ They showed that not only was JIAU associated with the need for glaucoma surgery but that the presence of both anterior segment complications and an IOP >21 mmHg during first remission were associated with greater risk of needing glaucoma surgery. Gunay and colleagues submitted two case reports where gonioscopy-assisted transluminal trabeculotomy (GATT) was successfully used to control IOP in uveitic glaucoma secondary to JIAU.²¹ Glandorf and colleagues compared the IOP lowering effects of trabeculectomy versus Ahmed valve implantation to treat glaucoma secondary to JIAU.²² They demonstrated that there was no significant difference between the groups despite slightly better success rates being achieved in the primary Ahmed valve group.

Our last theme revolves around interesting case reports and case series. Two case reports related to VHK disease. The first described a 4-year-old boy who presented with severe VKH disease requiring cataract extraction and ultimately Ahmed glaucoma valve implantation.²³ The second described concomitant VKH disease and autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome in a 2-year-old child.²⁴ Spontaneous regression of a choroidal neovascular membrane in a 6-year-old girl with rubella retinopathy²⁵ was described along with diagnostic challenges in PD-1 inhibitor-induced panuveitis which occurred in a 13-year-old girl with a soft tissue sarcoma of the chest.²⁶ An interesting case about the use of intravitreal clindamycin as an adjuvant treatment in congenital Toxoplasma retinochoroiditis in a neonate²⁷ and a multinational case series outlining ocular inflammatory events in patients younger than 18 years who were diagnosed with ocular inflammation less than 20 days after receiving COVID-19 vaccines²⁸ are some of the other highlights presented in this issue.

We trust that the diversity of topics submitted from various parts of the world will translate into this special edition containing something of interest to every one of our readers. We look forward to a new year with new challenges and opportunities to further increase our understanding of this complex subject that fascinates us so much.

Seasons’ greetings.