From the Guidelines
Sjögren's syndrome in pediatric patients should be suspected if intrinsic tear-production deficiency is detected, especially in nonelderly females with rapid onset and/or marked severity, and diagnosis and treatment of underlying systemic immune disorders may decrease morbidity and be lifesaving 1.
Diagnosis and Classification
The diagnosis of Sjögren's syndrome is based on defined, objective criteria, including anti-SSA/Ro antibody positivity, focal lymphocytic sialadenitis, and abnormal ocular staining score, among others 1. The latest classification criteria are based on the weighted sum of five items, with individuals having a total score of ≥4 meeting the criteria for primary Sjögren's syndrome 1.
Treatment and Management
Treatment options for patients with dry eye secondary to Sjögren's syndrome include topical lubricants, topical anti-inflammatory therapy, and tear-conserving strategies, which seem effective, although there is a paucity of rigorous clinical trials to support therapy recommendations 1. Oral secretagogues, such as pilocarpine and cevimeline, have been shown to be effective for sicca features, mostly oral, while topical anti-inflammatories, such as cyclosporine, have been effective for moderate or severe dry eye 1.
Key Considerations
Patients with Sjögren's syndrome are at increased risk for potentially life-threatening vasculitic or lymphoproliferative disorders, and studies have shown that patients with decreased C4 levels at the time of diagnosis had a higher risk of developing lymphoma 1. Therefore, comanagement with a rheumatologist is crucial due to the many possible comorbid systemic conditions 1. Some key points to consider in the management of pediatric patients with Sjögren's syndrome include:
- Evaluation of patients should include symptoms of both discomfort and visual disturbance as well as determination of the relative contribution of aqueous production deficiency and evaporative loss of tear volume 1.
- Objective parameters of tear film stability, tear osmolarity, degree of lid margin disease, and ocular surface damage should be used to stage severity of dry eye syndrome to assist in selecting appropriate treatment options 1.
- Patient education about the nature of the problem, aggravating factors, and goals of treatment is critical to successful management 1.
From the Research
Sjögren's Syndrome in Pediatrics
- Sjögren's syndrome (SS) is generally considered rare among children, but there are a considerable number of pediatric patients with the condition 2.
- Pediatric patients with SS report fewer sicca symptoms, which can lead to under-diagnosis and inadequate medical management 2.
- Clinical features of SS in pediatric patients may differ from those in adults, with parotitis and sicca symptoms being more common 3.
- Diagnostic tests and criteria used for adults may not be effective for pediatric patients, highlighting the need for pediatric-specific criteria 3.
Diagnosis and Treatment
- A newly developed guidance for the diagnosis, treatment, and management of pediatric SS has been proposed, including epidemiology, clinical features, and diagnostic examination methodology 2.
- Traditional Chinese Medicine (TCM) has been explored as a potential complementary therapy for SS, with some studies suggesting its effectiveness in relieving symptoms 4.
- Punctal plugs and artificial tears have been compared as treatments for primary SS with dry eye, with punctal plugs showing greater improvement in tear film stability and break-up time 5.
- Autologous serum eye drops combined with punctal plugs have been found to be an effective and safe treatment for SS-related dry eye, with significant improvements in tear break-up time and staining scores 6.
Unique Aspects of Pediatric Sjögren Disease
- Pediatric SS requires a distinct approach to diagnosis and treatment due to its unique clinical features and differences from adult SS 3.
- Further research is needed to define the natural history of the disease, identify risk and prognostic factors, and evaluate the impact of therapeutics and other interventions on disease course in young patients 3.