Ocular manifestations of juvenile Sjögren's disease

Area of Science:

• Pediatric Rheumatology
• Ophthalmology
• Autoimmune Diseases

Background:

• Juvenile Sjögren's disease (jSjD) is a rare autoimmune condition affecting children.
• Pediatric presentation of jSjD often differs significantly from adult Sjögren's syndrome.
• Current diagnostic criteria for Sjögren's syndrome are not optimized for pediatric populations.

Purpose of the Study:

• To review and enhance understanding of juvenile Sjögren's disease (jSjD).
• To explore diagnostic criteria, ocular manifestations, ophthalmic testing, and management strategies specific to jSjD.
• To highlight the need for pediatric-specific approaches in jSjD research and clinical practice.

Main Methods:

• Literature review focusing on diagnostic criteria, clinical features, and management of jSjD.
• Analysis of pediatric-specific symptoms and comparison with adult Sjögren's syndrome.
• Evaluation of ancillary ophthalmic tests for diagnosing keratoconjunctivitis sicca in jSjD.

Main Results:

• Children with jSjD commonly exhibit recurrent parotitis and extra-glandular symptoms preceding sicca symptoms.
• Existing adult Sjögren's syndrome classification criteria inadequately address pediatric-specific presentations.
• Underutilization of diagnostic tests like ocular staining score (OSS) and Schirmer I may lead to underdiagnosis of keratoconjunctivitis sicca in jSjD.

Conclusions:

• Early ophthalmological referral is recommended for evaluating ocular features in jSjD.
• Management of keratoconjunctivitis sicca in jSjD involves lubrication and anti-inflammatory strategies.
• Developing universally accepted jSjD classification criteria is essential for advancing research and patient identification.