Quality Evidence Lacking for Juvenile Sjögren Syndrome Treatment

Pharmacologic strategies for the management of juvenile Sjögren syndrome (SS) are varied and prescribed on the basis of clinician opinion, according to a systematic review published online in Rheumatology.

“This methodologically robust systematic review of the literature demonstrates that there is currently no standardized treatment regime for childhood-onset SS,” researchers reported, “and that the therapeutic decisions are based on clinicians’ expertise and preference and very likely derived from adult SS studies.”

The investigation included a total 43 studies and reports addressing pharmacologic treatment of SS with childhood onset: 34 case reports, 8 mini case series, and a single pilot study. Girls made up 88% of cases, and the studies had very low confidence levels.

The review revealed a handful of general therapeutic trends, researchers reported. Hydroxychloroquine was prescribed for unilateral or bilateral parotid swelling. In patients with arthritis and arthralgia, hydroxychloroquine was prescribed in association with methotrexate and nonsteroidal anti-inflammatory drugs.

For children with severe presentations of SS, long courses of corticosteroids, such as oral prednisone and intravenous methylprednisolone, were common. Rituximab was used mainly for mucosa-associated lymphoid tissue lymphoma, the review found, as well as for renal and nervous system complications. In select patients with extraglandular manifestations, other conventional disease-modifying antirheumatic drugs were used.

“At present,” researchers wrote, “there are no good-quality studies in SS with childhood onset to enable any clinical recommendations for strict selection of therapies. As the disease is rare in children compared with adults, further research into establishing validated classification criteria and disease outcome measures tailored for young people with SS is required.”

—Jolynn Tumolo

Reference:
Doolan G, Faizal NM, Foley C, et al. Treatment strategies for Sjögren’s syndrome with childhood onset: a systematic review of the literature. Rheumatology. 2022;61(3):892-912.