Linear Scleroderma in Children When Mycophenolate Mofetil or Abatacept Has Not Worked

Clinical Scenario

Children under 18 years with linear localized scleroderma (LoS) and severe skin and/or musculoskeletal involvement — affecting fat tissue, fascia, muscle, joints, bones, or presenting as widespread skin involvement. Linear types of LoS are more prevalent in childhood than in adults, and early potent systemic therapy is important to prevent late complications such as contractures, growth disturbances, and limb deformities.

Previous Treatment — Failure Condition

This protocol applies after second-line systemic therapy — mycophenolate mofetil, mycophenolic acid, or abatacept — has not achieved the expected response: reduction in peripheral erythema and reduction in skin sclerosis were not reached. The protocol below addresses management in this refractory situation.

Treatment Approach (Partial Overview)

For refractory paediatric linear scleroderma, Janus kinase (JAK) inhibition is among the targeted approaches that may be considered on a case-by-case basis — the full protocol specifies the agents, selection criteria, and additional options for specific clinical presentations.

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References

DOI: 10.1111/ddg.15328

While the limited type of LoS (morphea) primarily occurs in adults, linear types are more prevalent in childhood.

However, in all other cases and for active linear LoS in childhood, potent systemic therapy should be initiated as early as possible to prevent potential late complications (contractures, growth disturbances, limb deformities, etc.).

Although prospective and controlled studies are still lacking, the authors of this guideline think that JAK inhibitors may be considered on a case-by-case basis, weighing all known risks, in cases of refractory LoS after the use of MTX, MMF, or abatacept.

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