OCTAGAM (immunoglobuline humaine normale) - Dermatomyosite

Key points

Approval of reimbursement for the treatment of active dermatomyositis treated with immunosuppressants, including corticosteroids, or in cases of intolerance to these medicinal products or contraindications to these medicinal products. 

Therapeutic improvement?

Therapeutic improvement in the management of active dermatomyositis in adults.

Role in therapeutic strategy?

Non-medicinal measures are important in the management of adult dermatomyositis, in association with immunomodulator/immunosuppressant treatment, whether they involve the management of pain, psychological impact, disability, undernourishment, therapeutic education, and the key role of rehabilitation/physiotherapy.

The basic treatment is based on corticosteroid therapy, generally associated with methotrexate as a cortisone-sparing agent (off-label).

Other systemic treatments used off-label. such as immunosuppressants (azathioprine, mycophenolate mofetil, ciclosporin, cyclophosphamide), rituximab (glycosylated immunoglobulin) and normal human immunoglobulin (IVIg) and/or plasma exchange, are discussed as second- or third-line treatments depending on the clinical signs and the associated impairments. Skin impairment may progress inconsistently with muscle impairment. In cases of persisting significant skin impairment, topical treatments (dermocorticosteroids, topical tacrolimus 0.03 and 0.1%) and hydroxychloroquine are used (off-label).

IVIg therapies may be proposed to “overcome” a difficult phase, and they help control the disease pending the effect of the new basic treatment.

In the specific case of the treatment of skin impairment, IVIg therapies may be beneficial for patients with a severe form and muscle impairment.

IVIg therapies may be proposed as a first-line approach in cases of severe dermatomyositis with a risk of life--threatening complications, in association with immunosuppressants.

Role of the medicinal product

OCTAGAM 100 mg/ml (normal human Ig), solution for infusion, is reserved for adults with active dermatomyositis, whose clinical muscle severity justifies the use of adjunctive therapy to the standard treatment (corticosteroid and/or immunosuppressant) in the aim of achieving rapid improvement justified by the severity of the impairment and/or in cases of poor tolerance/contraindication to the standard treatment and/or pending its time-to-efficacy (3 to 6 months).

If no treatment effect is observed after 6 months, the treatment must be discontinued. If the treatment is effective, it will be continued long-term at the doctor’s discretion, based on patient response, and response to the maintenance treatment. Nevertheless, IV Ig treatments must only be continued after 6 months in exceptional cases. This therapy should be reserved for resistant forms of dermatomyositis, failing to respond to first-, second- or even third-line immunosuppressant therapies in association with corticosteroids.