Primary juvenile Sjögren's syndrome in a 4-year-old female patient: a Palestinian case report.

INTRODUCTION AND CLINICAL SIGNIFICANCE

Primary Sjögren's syndrome (PSS) is a rare disease in the pediatric age group, with not enough data regarding these patients. In addition, to date only few papers emphasizing uveitis as a finding of PSS have been published.

CASE PRESENTATION

We describe a case of a 4-year-old Palestinian girl with bilateral parotid gland enlargement, dry eyes and mouth, eyelids swelling, arthralgia, dysphagia, and fatigue for 2 months duration. The patient was diagnosed with uveitis. Investigations showed elevated CPR and erythrocyte sedimentation rate. ANA was initially negative on the enzyme-linked immunosorbent assay, but elevated on the immunofluorescence assay 6 weeks after the initial test. She was diagnosed with PSS according to the clinical findings, ultrasonography, and salivary - gland biopsy results.

CLINICAL DISCUSSION

Uveitis is a rare manifestation of PSS and has not yet been reported in the literature as a manifestation of pediatric PSS. Because uveitis is usually asymptomatic in this age group, case-regular ophthalmologic follow-up is recommended in all patients with PSS, as a delay in diagnosis and management may cause permanent vision loss.

CONCLUSION

PSS is rare in the pediatric population, with onset between 10 and 14 years old. Salivary gland ultrasound and minor salivary gland biopsy are essential tools for the diagnosis of PSS in childhood. Treatment options for PSS in children are limited, but medications like methotrexate and prednisolone can help improve systemic symptoms and reduce parotid swelling. Pilocarpine can be added for dryness, and steroid eye drops and artificial tears for dry eyes. Further research is needed to establish management protocols specifically for the pediatric age group.