Reason for request
Favourable opinion for reimbursement in the treatment of patients with moderate to severe pemphigus vulgaris.
What therapeutic improvement?
Slight therapeutic improvement compared to systemic corticosteroid therapy.
Role in the care pathway?
The primary objective of treatment is to achieve and maintain remission. This implies the suppression of blister formation, the healing of skin and/or mucous membrane erosions and the reduction of treatment. The secondary objectives are to control the disease, prevent relapses, limit cumulative corticosteroid doses, reduce circulating antibody levels, limit adverse effects and maintain quality of life.
Rituximab is the first-line induction treatment for moderate to severe pemphigus vulgaris, in combination with short-term (3 to 6 months) low-dose systemic corticosteroid therapy. Rituximab will be used alone (or with topical corticosteroids) in the event of absolute or relative contraindications to systemic corticosteroid therapy.
The use of standard-dose systemic corticosteroid therapy alone or combined from the outset with a conventional immunosuppressant treatment (particularly in the event of increased risk of corticosteroid therapy-related complications and in order to enable rapid withdrawal) may be an alternative in the event of contraindication to rituximab or if this treatment cannot be administered.
The conventional immunosuppressive agents recommended are mycophenolate mofetil (off-label, professional agreement), methotrexate (off-label, professional agreement) or azathioprine. Cyclophosphamide as an intravenous bolus or orally is not recommended as a first-line treatment due to the numerous adverse effects.
Following a first course of rituximab, maintenance treatment may be administered depending on the patient’s response to treatment (see SPC).
Role of the medicinal product in the care pathway
Rituximab, in combination with systemic corticosteroid therapy, is the first-line treatment in moderate to severe pemphigus vulgaris in adults.
The clinical benefit of MABTHERA is substantial in the treatment of patients with moderate to severe pemphigus vulgaris (PV).
Clinical Added Value
MABTHERA provides a minor clinical added value (CAV IV) in the treatment of moderate to severe pemphigus vulgaris.