Treatment of Localized Scleroderma with Severe Skin and Musculoskeletal Involvement
This protocol covers adults aged 18 and older with localized scleroderma presenting in a severe form — with deep involvement of fat tissue, fascia, muscle, joints, or bones, widespread skin involvement, or significant musculoskeletal symptoms directly attributable to the disease.
Patients in this group present with severe skin involvement or musculoskeletal manifestations of localized scleroderma — which may include arthritis, myositis, osteitis, or musculoskeletal pain attributable to the condition. The depth and extent of involvement places these patients in a category where systemic therapy is indicated.
Systemic therapy with methotrexate is recommended as the first-line treatment for this presentation. During the active phase of disease, additional systemic glucocorticosteroid therapy may be considered alongside it.
Dosing, duration, tapering strategy, and sequencing details are available in the complete protocol.
Reduction of skin sclerosis, typically assessed from 8 to 12 weeks after treatment initiation.
References
DOI: 10.1111/ddg.15328
- In subtypes with severe skin or musculoskeletal involvement, systemic therapy with methotrexate is recommended.
- We recommend methotrexate (MTX) (off-label) as the first-line systemic therapy for LoS with severe skin and/or musculoskeletal involvement.
- Arthritis, myositis, osteitis, or depending on the severity, musculoskeletal pain symptoms attributable to LoS.
- We recommend a treatment duration of at least 12 months with methotrexate after achieving remission.
- After achieving therapeutic success, a dose reduction may be considered.
- We suggest considering systemic glucocorticosteroid therapy in addition to the systemic treatment with MTX during the active phase of LoS.
- When assessing the effectiveness of the chosen therapeutic concept, it should be noted that the reduction of sclerosis typically takes at least 8 to 12 weeks in most cases.