Sjögren's in Children

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 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.