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Published on: October 13, 2023
Pediatric Sjögren's Syndrome: Focus on Ocular Involvement and Diagnostic Challenges
Emanuela Del Giudice1, Maria Carmela Saturno2, Maria Grazia Fiorino2
1Pediatrics and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Santa Maria Goretti Hospital, Sapienza University of Rome, Polo Pontino, 04100 Latina, Italy.
Insights
Early diagnosis of pediatric Sjögren
Area of Science:
- Pediatric Rheumatology
- Ophthalmology
- Autoimmune Diseases
Background:
- Pediatric Sjögren's syndrome is a rare autoimmune disorder with diverse symptoms and few specific diagnostic criteria for children.
- Ocular manifestations are common early signs but often missed in pediatric patients.
- This study focuses on early immunological and clinical markers in pediatric Sjögren's syndrome.
Purpose of the Study:
- To evaluate pediatric Sjögren's syndrome by integrating systemic and ocular findings.
- To identify early immunological and clinical markers for diagnosis.
- To emphasize the role of ophthalmological assessments in early detection.
Main Methods:
- Retrospective analysis of six pediatric patients with Sjögren's syndrome.
- Ophthalmological assessments: tear break-up time (TBUT), Schirmer's test, slit-lamp examination.
- Evaluation of systemic features, serological markers (ANA, anti-SSA/Ro), and minor salivary gland biopsy.
Main Results:
- Consistent tear film instability (mean TBUT 7.4-7.7s) and inhomogeneous tear films observed in all patients.
- Blepharitis and Meibomian gland dysfunction noted in 66.7% of cases.
- Systemic symptoms (arthralgia, fatigue) and seropositivity (ANA, anti-SSA/Ro) were frequent; minor salivary gland biopsy confirmed lymphoepithelial sialadenitis.
Conclusions:
- Combining ophthalmological parameters (TBUT, slit-lamp) with laboratory and clinical markers aids early diagnosis of pediatric Sjögren's syndrome.
- Early diagnosis is crucial for timely intervention and preventing long-term complications.
- Integrated diagnostic approaches enhance accuracy in identifying this rare pediatric condition.
Abstract:
Background and Objectives: Pediatric Sjögren's syndrome is a rare autoimmune disease with a heterogeneous clinical expression and limited pediatric-specific diagnostic criteria. Ocular involvement often represents an early manifestation, yet it may go unrecognized in children due to poor symptom reporting and the underuse of objective diagnostic tools. This retrospective study evaluated six pediatric patients with Sjögren's syndrome, integrating systemic and ocular findings with a focus on early immunological and clinical markers. Materials and Methods: All patients underwent ophthalmological assessments, including tear break-up time, Schirmer's test, and slit-lamp examination. Results: Tear break-up time values consistently indicated tear film instability (mean RE 7.4 ± 2.5 s; LE 7.7 ± 2.3 s), while Schirmer's test showed greater variability. Slit-lamp examination revealed inhomogeneous tear films in all patients and blepharitis in 66.7%, consistent with Meibomian gland dysfunction. Systemic features included arthralgia, Raynaud's phenomenon, fatigue, and frequent seropositivity for ANA and anti-SSA/Ro antibodies. Minor salivary gland biopsy confirmed lymphoepithelial sialadenitis in all cases. Conclusions: These findings highlight the importance of combining laboratory and clinical markers with ophthalmological parameters to support an early diagnosis of Sjögren's syndrome in pediatric patients. Integrating TBUT and slit-lamp evaluation with serological and histopathological data may enhance diagnostic accuracy and guide timely, targeted intervention to prevent long-term complications.
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