Childhood-Onset Non-Infectious Uveitis in the “Biologic Era”. Results From Spanish Multicenter Multidisciplinary Real-World Clinical Settings

ABSTRACT

Objective

To characterize and describe clinical experience with childhood-onset non-infectious uveitis.

Study Design

A multicenter retrospective multidisciplinary national web-based registry of 507 patients from 21 hospitals was analyzed. Cases were grouped as immune disease-associated (IMDu), idiopathic (IDIu) or ophthalmologically distinct. Characteristics of juvenile idiopathic arthritis-associated (non-HLA-B27-related) uveitis (JIAu), IDIu, and pars planitis (PP) were compared.

Results

IMDu (62.3%) and JIAu (51.9%) predominated in young females; and IDIu (22.7%) and PP (13.6%) in older children, without sex imbalance. Ocular complications occurred in 45.3% of cases (posterior synechiae [28%], cataracts [16%], band keratopathy [14%], ocular hypertension [11%] and cystoid macular edema [10%]) and were associated with synthetic (86%) and biologic (65%) disease-modifying antirheumatic drug (DMARD) use. Subgroups were significantly associated (p < 0.05) with different characteristics. JIAu was typically anterior (98%), insidious (75%), in ANA-positive (69%), young females (82%) with fewer complications (31%), better visual outcomes, and later use of uveitis-effective biologics. In contrast, IDIu was characteristically anterior (87%) or panuveitic (12.1%), with acute onset (60%) and more complications at onset (59%: synechiae [31%] and cataracts [9.6%]) and less DMARD use, while PP is intermediate, and was mostly bilateral (72.5%), persistent (86.5%) and chronic (86.8%), with more complications (70%; mainly posterior segment and cataracts at last visit), impaired visual acuity at onset, and greater systemic (81.2%), subtenon (29.1%) and intravitreal (10.1%) steroid use.

Conclusion

Prognosis of childhood uveitis has improved in the “biologic era,” particularly in JIAu. Early referral and DMARD therapy may reduce steroid use and improve outcomes, especially in PP and IDIu.

KEYWORDS:

• Autoimmune diseases
• biological therapy
• disease-modifying antirheumatic drugs
• juvenile idiopathic arthritis
• uveitis

Abbreviations

IMDu: immune disease-associated uveitis; IDIo: ophthalmologically distinct idiopathic uveitis; IDIu: unclassified idiopathic uveitis; PP: pars planitis; JIA: juvenile idiopathic arthritis; JIAu: juvenile idiopathic arthritis-associated uveitis; DMARD: disease-modifying antirheumatic drug; sDMARD: synthetic DMARD; bDMARD: biologic DMARD; ANA: anti-nuclear antibodies; HLA: human leukocyte antigen; OCT: ocular coherence tomography; IQR: interquartile range; SD: standard deviation. VA: visual acuity. BCVA: best corrected visual acuity.

Acknowledgments

We are grateful for funding from the Spanish Society of Pediatric Rheumatology (SERPE), help with database analysis from the Sección de Apoyo Estadístico (SAE), Área Científica y de Investigación (ACTI), at Murcia University (www.um.es/web/acti) and support from the Spanish Society of Rheumatology (SER) for technical proofreading of the manuscript (FERBT2023).

Disclosure statement

On behalf of all authors, I declare that we have disclosed all competing interests related to this work. If any exist, they have been included in the manuscript’s “declaration of interests” section.

Competing interests

PMdC: Payment for lectures: Pfizer; Support for attending meetings and travel: Pfizer. MCP: Support for attending meetings and travel: Abbvie, Pfizer, Sobi, Novartis. DC: Payment for lectures: Pfizer, GSK; Support for attending meetings and travel: Pfizer. JCN: Consulting fees: Janssen; Payment for lectures: Galapagos, Biogen, Abbvie, UCB pharma, Lilly, MSD; Support for attending meetings and travel: MSD, Galapagos, Janssen, Gedeon. AS: Payment for lectures: Galapagos, Abbvie, Celgene. FLL: Payment for lectures: Santen Pharmaceutics; Support for attending meetings and travel: Santen, Abbvie; Advisory board: Santen Pharmaceutics. EC: Consulting fees: Active Biotech. Payment for lectures: GSK, Alimera.Support for attending meetings and travel: Novartis. Advisory board: Eyevensis. Leadership in boards(10): EVER board (chair IM section), GMC ARVO chair, Spanish Society of Ocular Inflammation (SEIOC) board member.

Ethical approval

This study was approved by the Hospital Clínico Universitario Virgen de la Arrixaca Ethics Committee. Promoter code: SER-ADA-2019-01. Internal code: January 10, 2019-HUVA. The retrospective nature of the study and anonymization prior to patient registry made informed consent unnecessary. Subsequently, all researchers proceeded to obtain ethics committee approval from their centers in accordance with local regulations.

Additional information

Funding

Spanish Society of Pediatric Rheumatology (SERPE).