Linear Scleroderma in Adults: When Mycophenolate or Abatacept Has Failed

This protocol covers adults (age 18 years or older) with linear localized scleroderma involving severe skin and/or musculoskeletal structures — fat tissue, fascia, muscle, joints, bones, or widespread skin — whose disease has not responded adequately to second-line systemic therapy.

Previous line — failure condition

Second-line systemic therapy with mycophenolate mofetil, mycophenolic acid, or abatacept did not achieve the expected outcomes: reduction in peripheral erythema and reduction in skin sclerosis were not reached. This protocol defines the next step following that documented failure.

Next-step approach (partial overview)

For this refractory situation in adults with severe involvement, the approach involves targeted agents — including options from the Janus kinase inhibitor class and a biologic addressing extracutaneous disease — evaluated on a case-by-case basis. The complete eligibility criteria, agent selection, and clinical decision points remain in the full protocol.

Full regimen details are accessible via the link below.

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References

We recommend methotrexate (MTX) (off-label) as the first-line systemic therapy for LoS with severe skin and/or musculoskeletal involvement.

In subtypes with severe skin or musculoskeletal involvement, systemic therapy with methotrexate is recommended.

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.

According to the authors opinion, tocilizumab should primarily be offered to patients with accompanying or predominant extracutaneous involvement (for example arthritis) if other standard therapies have failed or are contraindicated.

DOI: 10.1111/ddg.15328 View source ↗