Facial angiofibroma associated with tuberous sclerosis complex

Active Ingredient: Sirolimus

Indication for Sirolimus

Population group: only children (1 year - 12 years old) , adolescents (12 years - 18 years old) , adults (18 years old or older)
Therapeutic intent: Curative procedure

Sirolimus is indicated for the treatment of facial angiofibroma associated with tuberous sclerosis complex in adults and paediatric patients aged 6 years and older.

For this indication, competent medicine agencies globally authorize below treatments:

For patients 6-11 years old up to 1.2 mg sirolimus per day and for patients ≥12 years old up to 1.6 mg sirolimus per day

For:

Dosage regimens

Regimen A, in case that patient age in years is ≥ 6 and patient age in years is ≤ 11

Cutaneous, 1.2 milligrams sirolimus, divided 2 times daily.

Regimen B, in case that patient age in years is ≥ 12

Cutaneous, 1.6 milligrams sirolimus, divided 2 times daily.

Detailed description

This medicinal product should be applied to the affected area twice daily (in the morning and at bedtime). The application should be limited to skin areas with angiofibroma.

A dose of 0.25 mg sirolimus should be administered per 50 cm² lesion in the face.

The maximum recommended daily dose in the face is:

  • Patients aged 6-11 years should apply up to 1.2 mg sirolimus per day.
  • Patients aged ≥12 years should apply up to 1.6 mg sirolimus per day.

The dose should be equally divided for two administrations.

Missed dose

If the first dose was missed in the morning, the application should be done immediately upon realisation of the fact provided this was before dinner of the same day. Otherwise only the application in the evening should be administered on that day. If the application in the evening was missed this should not be taken at a later point in time.

Elderly

No dose adjustment is required in elderly patients (≥65 years).

Dosage considerations

For cutaneous use only.

Application should be limited to areas of facial angiofibroma lesions.

A thin layer of gel should be administered to the affected skin and rubbed in gently. The application site should not be occluded.

The gel should not be applied around the eyes and on the eyelids.

In case no treatment effect appears, administration with Hyftor should be discontinued after 12 weeks.

Hands should be washed carefully before and after administration of the gel to ensure that no gel remains on the hands that may be accidentially ingested or trigger exposure to sirolimus of any other part of the body or other persons.

Active ingredient

Sirolimus

Sirolimus inhibits T-cell activation induced by most stimuli, by blocking calcium-dependent and calcium-independent intracellular signal transduction. Studies demonstrated that its effects are mediated by a mechanism that is different from that of ciclosporin, tacrolimus, and other immunosuppressive agents.

Read more about Sirolimus

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