Treatment of Linear Scleroderma in Adults When Methotrexate Has Not Achieved Skin Sclerosis Reduction

Clinical scenario

Adults (age 18 years or older) with linear localized scleroderma and severe involvement — affecting skin, fat tissue, fascia, muscle, joints, bones, or presenting as widespread skin changes — whose disease has not adequately responded to first-line systemic therapy.

Previous treatment — failure condition

First-line systemic therapy with methotrexate (with glucocorticosteroids during the active phase) did not achieve the expected reduction in skin sclerosis after at least 8 to 12 weeks of treatment. Escalation to a second line is also indicated where the first-line agent is contraindicated or not tolerated.

Second-line approach (partial overview)

After first-line failure, a second-line systemic therapy is indicated on an off-label basis. The approach draws on a specific class of immunomodulatory agents. The complete regimen — including agent selection, monitoring guidance, and sequencing — is available in the full protocol.

Treatment goals

Clinical improvement with measurable reduction in peripheral erythema and reduction in skin sclerosis.

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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.

We suggest using mycophenolate mofetil, mycophenolic acid, or abatacept as a second-line systemic therapy for LoS in cases of MTX and steroid-refractory courses, contraindication, or intolerance (off-label).

All patients described in the initial study experienced clinical improvement (reduction in peripheral erythema, reduction in sclerosis), and concomitant steroid dose could be significantly reduced in some cases.

DOI: 10.1111/ddg.15328

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