Children with Sjögren’s tend to present differently than adults. Children rarely present with the predominant symptoms of dry mouth and dry eyes. However, around a third of children with childhood Sjögren's have symptoms of dry mouth and dry eyes. Uniquely, there is much higher rate of recurrent and persistent parotitis in childhood presentations. Parotitis is inflammation of the salivary gland by the cheek. It was found that about 60% of children had a history of parotitis, as opposed to 24% of adults with Sjögren’s.
The clinical presentation of childhood Sjögren’s widely varies from child to child. Unlike adults, the most common presentation in children is parotid enlargement. Many children have aspects of ocular and oral dryness at presentation but have difficulty perceiving and reporting these symptoms as dryness. Vaginal irritation can occur affecting around 5% of the females with childhood Sjögren’s. Joint symptoms, a characteristic rash, fatigue, fevers, and enlarged lymph nodes also occur. Neurologic, kidney, blood, lungs, and abdominal manifestations can occur but are more rare presentations.
There are no valid diagnostic criteria specific for childhood Sjögren’s. Many aspects of the adult criteria are difficult to complete and interpret in children. Therefore, diagnosing childhood Sjögren’s can be difficult and is based on multiple clinical features as well as clinical judgement of the treating physician.
As with adults, children with childhood Sjögren’s can get additional autoimmune diseases. Some of the most likely diseases seen with childhood Sjögren’s are systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA).
The goals for treatment of childhood Sjögren’s is to prevent pain, decrease inflammation, and ideally prevent long term damage.
While important differences exist in the clinical presentation of Sjögren’s in children versus adults, the treatments are generally similar. This is in part due to the lack of any studies evaluating the differences in response to treatment. In children with recurrent parotitis it is common practice to treat with hydroxychloroquine to prevent or minimize recurrences. Acute exacerbations are sometimes treated with corticosteroids (short courses of oral dosing or 1-2 doses at high doses intravenously). In refractory recurrent parotitis, there has been success with the addition of either daily low-dose corticosteroids or methotrexate. Other immunomodulatory therapies such as mycophenolate mofetil, abatacept, and rituximab are also worth considering, especially if additional extraglandular manifestations are present. While not a major presenting feature, some children develop sicca symptoms, and for these symptoms the treatment approach often does not differ from that used for adult patients. Regardless of symptoms, children with Sjögren’s should have regular dentist and ophthalmologist evaluations and treatments (such as topical fluoride treatments) to maximize oral and ocular health.
The prognosis of childhood Sjögren’s is different for each child and is dependent on their specific Sjögren’s manifestations. The course as well as a child’s response to specific treatments is different for each individual. Some children’s symptoms may improve greatly with treatment over time, with time to improvement highly variable depending on the child and the symptom(s). Therefore, it is important to work closely with a rheumatologist to determine the best treatment plan. Children also need routine monitoring for damage by oral and ocular health specialist. Additionally, children should maintain a healthy lifestyle including staying active.
Pediatric Support Group
Thanks to dedicated volunteers, Sharon and Jamie, two mothers of children with Sjögren’s, the Foundation launched a support group for children and their parents. Please contact Sharon at (301) 875-2701 for more information.