Treatment of Eosinophilic Fasciitis: Immunosuppressive Strategies for Glucocorticoid-Sparing Management
In eosinophilic fasciitis, corticosteroid therapy may be insufficient to achieve complete remission, or long-term use may be limited by adverse effects — requiring the addition of immunosuppressive agents to support disease control.
Clinical context: This protocol addresses patients with eosinophilic fasciitis in whom glucocorticoid-sparing immunosuppressive therapy is indicated — including cases of relapse, inadequate response to corticosteroids alone, or unacceptable corticosteroid-related adverse events. Severe presentations, such as those involving the trunk or respiratory mechanics, are also covered.
Treatment Approach — Partial Overview
The protocol centres on immunosuppressive agents used as glucocorticoid-sparing therapy. Multiple agent options are addressed, and for severe clinical presentations an intensified combination strategy is included. Agent selection, sequencing, dosing, and clinical thresholds are detailed in the full protocol.
References
DOI: 10.1007/s40674-024-00222-6
- Methotrexate (7.5–25 mg/week) is the drug usually recommended as a second-line therapy when corticosteroids alone are not effective for achieving complete remission, when the disease relapses, or due to unacceptable adverse events such as osteoporotic fractures, hyperglycemia or stigmas of hypercortisolism.
- Also, in some clinical situations such as patients who develop some morphea-like lesions, this drug should be considered as a second-line therapy together with the administration of corticosteroids.
- Other immunosuppressive drugs such as azathioprine (1–2 mg/kg/day), cyclosporin (100–150 mg/day), biologic agents (rituximab, tumor necrosis factor blockers, interleukin-6 receptor inhibitors) and hydroxychloroquine are also included as a second-line therapy in EF.
- Mycophenolate mofetil (1 g/12 h) plus intravenous immunoglobulins (0.4 gr/kg/d × 5 days, once a month × 6 months) may be useful in severe situations such as perimyositis or restrictive respiratory failure due to trunk skin induration.
- Thus, mycophenolate mofetil is a good option as a second-line therapy for patients with EF.
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