Use of biologic drug in the treatment of localized scleroderma and systemic sclerosis in children: A scoping review

Abstract

Objective

Biologic drugs are a potential treatment option in resistant cases of juvenile scleroderma. In this review, we aimed to examine previous studies regarding biologic drug use in pediatric patients with localized scleroderma and systemic sclerosis.

Methods

We performed a search on MEDLINE and Scopus for articles involving pediatric localized scleroderma and systemic sclerosis patients treated with biologic drugs.

Results

We identified 17 articles describing 58 pediatric patients with localized scleroderma treated with biologic drugs and 12 articles describing 29 pediatric patients with systemic sclerosis treated with biologic drugs during our literature search. The most frequently used biologic drug in localized scleroderma treatment was abatacept (55.2 %), followed by tocilizumab (48.3 %). These biologic drugs were mainly preferred for treating resistant/progressive skin disease in pediatric patients with localized scleroderma (58.5 % and 68.8 %, respectively). The improvement rates associated with abatacept and tocilizumab were 92.9 % and 77.4 %, respectively. Adverse events were observed in 23.5 % of all localized scleroderma patients. The most frequently used biologic drug in systemic sclerosis treatment was rituximab (51.7 %), followed by tocilizumab (44.8 %). Rituximab was predominantly favored for managing cardiac involvement (45.5 %), whereas tocilizumab was preferred for pulmonary involvement (50 %) in pediatric patients with systemic sclerosis. The improvement rates associated with rituximab and tocilizumab were 72.7 % and 94.1 %, respectively. Adverse events were reported in 40 % of all systemic sclerosis patients.

Conclusion

Our results showed that abatacept and tocilizumab were more frequently used in patients with localized scleroderma, while rituximab and tocilizumab were the predominantly used biologics in patients with systemic sclerosis. The improvement rate with these biologics were quite high with acceptable safety profile.