Pediatric patients can develop Sjogren's syndrome. However, the features used to diagnose it may differ from criteria used in adults. The diagnosis can be challengiing, with a high index of suspicion required
Patient selection: pediatric patient with Sjogren syndrome
Clinical features in pediatric patients:
(1) sicca symptoms (dry eyes and/or dry mouth)
(2) arthralgia or arthritis
(3) recurrent or persistent parotitis
(4) reduced unstimulated whole salivary flow
Some patients have a history of another autoimmune disease, head and neck radiation or malignant lymphoma.
Less common findings:
(1) lymphadenopathy
(2) transverse myelitis
(4) neuro-myelitis-optica syndrome
(4) cutaneous vasculitis
(5) peripheral neuropathy
(6) myositis
(7) pulmonary disease
(8) interstitial nephritis
Laboratory testing may show:
(1) positive ANA
(2) positive rheumatoid factor
(3) anti-SSA/Ro and/or anti-SSB/La (both negative in 22%)
(4) hypogammaglobulinemia
(5) cytopenia
Salivary gland biopsy:
(1) half have a focus score >= 1 per 4 square mm
(2) 20% have some inflammation with focus score < 1 per square mm
Many patients had incomplete testing performed. In addition, many children do not meet the 2016 ACR/EULAR criteria.
