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How should I initiate treatment for a patient diagnosed with pemphigus vulgaris, and what are the first-line therapies?
Answer
Initiation of treatment for pemphigus vulgaris should begin promptly with systemic corticosteroids as the mainstay first-line therapy to control disease activity and prevent progression. High-dose oral prednisolone is typically used initially to induce remission, often combined with adjuvant immunosuppressive agents to reduce corticosteroid exposure and side effects.
First-line therapies include systemic corticosteroids combined with steroid-sparing agents such as azathioprine or mycophenolate mofetil. Rituximab, a monoclonal anti-CD20 antibody, has emerged as a highly effective first-line treatment option, either alone or in combination with corticosteroids, and is increasingly recommended due to its ability to induce sustained remission and reduce corticosteroid dependence.
Early use of rituximab as first-line therapy has been shown to achieve sustained remission without corticosteroids in many patients, improving long-term outcomes and safety profiles compared to corticosteroids alone. This approach is supported by recent clinical trial follow-up data demonstrating durable remission with rituximab (Tedbirt et al., 2024).
Management should be initiated under specialist dermatology care with close monitoring for treatment response and adverse effects. Adjunctive therapies such as topical corticosteroids may be used for localized lesions but are insufficient as monotherapy in pemphigus vulgaris.
In summary, the current integrated approach to initiating treatment for pemphigus vulgaris involves systemic corticosteroids combined with immunosuppressants, with rituximab increasingly recognized as a first-line option to achieve sustained remission and minimize corticosteroid exposure (Popescu et al., 2019; Antiga et al., 2023; Tedbirt et al., 2024).
Key References
- CKS - Vitiligo
- CKS - Itch in pregnancy
- CKS - Palliative care - oral
- CKS - Psoriasis
- CG153 - Psoriasis: assessment and management
- (Popescu et al., 2019): Pemphigus vulgaris - approach and management.
- (Antiga et al., 2023): S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).
- (Tedbirt et al., 2024): Sustained Remission Without Corticosteroids Among Patients With Pemphigus Who Had Rituximab as First-Line Therapy: Follow-Up of the Ritux 3 Trial.
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