Better Treatment Recommendations Needed for Children

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by Margarida Maia |

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Childhood-onset Sjögren’s

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Current studies investigating treatment strategies for children with Sjögren’s syndrome have poor quality evidence for the development of clinical recommendations, a recent study has found.

As such, these children are still prescribed medication based on their doctors’ opinions, not on evidence-based treatment recommendations.

“Future research is required to guide treatment recommendations in this rare disease,” the researchers wrote.

The study, “Treatment strategies for Sjögren’s syndrome with childhood onset: a systematic review of the literature,” was published in the journal Rheumatology.

Sjögren’s syndrome is an autoimmune disorder that primarily affects the glands producing saliva and tears. The underlying cause of Sjögren’s is not fully known, which is one reason why current treatments focus on relieving symptoms and preventing long-term complications of the disease.

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Although Sjögren’s syndrome mostly affects people older than 40, a few hundred cases of the disease have been reported in children. Yet, childhood-onset Sjögren’s is poorly characterized and, due to the scarcity of studies in children, treatment strategies follow those recommended for adults.

Recommendations to guide treatment in children and adolescents with Sjögren’s syndrome “are not available due to the rarity of disease and the paucity of research in this patient population,” the researchers wrote.

The team of researchers in the U.K. set out to investigate the medications used to treat childhood-onset Sjögren’s syndrome, as well as their efficacy, to determine if current studies could help develop treatment recommendations.

A search through available medical literature identified 43 studies (34 case reports, eight case series, and one pilot study) describing pharmacological treatment in people with childhood-onset Sjögren’s syndrome.

In total, the studies had data 137 patients, 88% of whom were female and whose ages ranged from 2.6 to 17 years.

Overall, disease-modifying anti-rheumatic drugs (DMARDs), a class of medicines commonly used to treat inflammatory arthritis and other related diseases, were prescribed to 62% of all children.

The most commonly used was hydroxychloroquine — a medication better known for preventing and treating malaria — which was prescribed to 34% of children. The most common symptoms for which it was used were swelling of the parotid gland (a gland that produces saliva), joint pain, and kidney problems.

The response to hydroxychloroquine treatment was favorable in 18 of 46 children, but since this therapy often was used in combination with other medications, it was hard to link any benefits to hydroxychloroquine alone.

A biological DMARD, rituximab, was indicated mainly for mucosa-associated lymphoid tissue lymphoma and complications related to the kidneys or the nervous system. It also seemed effective at controlling the psychiatric symptoms of the condition.

Additional DMARD medications included azathioprine, mycophenolate mofetil, cyclosporine A,  cyclophosphamide, and sulfasalazine, which were prescribed for a number of overlapping symptoms. A small subset of patients also received topical treatments for oral dryness and eye symptoms.

About half of the patients (52%), many of whom had severe symptoms, received corticosteroids for various clinical indications. In most cases, corticosteroids were administered in combination with additional immunosuppressive medications. In general, corticosteroids were found to result in clinical improvements.

Based on the available data, the researchers concluded “therapeutic decisions are based on clinicians’ expertise and preference.”

Moreover, because there are no validated measures of disease activity, “the response to treatment was entirely assessed based on clinician opinion,” the researchers added.

Despite the lack of ground evidence, damage to various organs in the body “is likely to respond to a combination of strong immunosuppression and high steroid doses,” the researchers wrote, adding that children should be included in future clinical trials “before good evidence-based treatment recommendations for this patient population can be made.”