has developed. Immunosuppressant medication has resulted in unacceptable side effects or significant toxicity OR Corticosteroids and/or immunosuppressant agents are contraindicated Statement informing prescribers of the Three months treatment is authorised initially after which time a requirement to have demonstrated clinical clinical response should have been demonstrated. If, in response to previous Ig therapy and then responding patients, disease worsens following cessation of Ig relapsed prior to commencing maintenance therapy, a request for low dose maintenance therapy can be treatment. made. Documentation of clinical effectiveness of initial Ig therapy will be required for further requests. Worsening of EBA disease in responding patients with ongoing flare after ceasing Ig therapy The criteria confirm the worsening of mucosal and /or ophthalmological disease Ongoing flare of mucosal or ophthalmic EBA disease in once Ig therapy is withdrawn in responding previously responding patients patients. A description of current symptoms AND is used for comparison to demonstrate Reduction in severity and/or the number of lesions was response to Ig therapy at initial review. demonstrated in response to initial Ig therapy AND At least one Immunosuppressant medication is to be given Maintenance Ig therapy must be given in concurrently combination with at least one immunosuppressant medication to all patients. Review Criteria Persistent severe EBA refractory to conventional All patients will receive an initial trial of three immunosuppressive therapy months Ig therapy. Outcome measures are defined to indicate to prescribers of the Review is not mandated for this condition. expected clinical response.
Immunosuppressant medication has resulted in unacceptable side effects or significant toxicity OR Corticosteroids and/or immunosuppressant agents are contraindicated Three months treatment is authorised initially after which time a clinical response should have been demonstrated. If, in responding patients, disease worsens following cessation of Ig therapy, a request for low dose maintenance therapy can be made. Documentation of clinical effectiveness of initial Ig therapy will be required for further requests. Worsening of EBA disease in responding patients with ongoing flare after ceasing Ig therapy Ongoing flare of mucosal or ophthalmic EBA disease in previously responding patients AND Reduction in severity and/or the number of lesions was demonstrated in response to initial Ig therapy AND At least one Immunosuppressant medication is to be given concurrently has developed. Statement informing prescribers of the requirement to have demonstrated clinical response to previous Ig therapy and then relapsed prior to commencing maintenance treatment. The criteria confirm the worsening of mucosal and /or ophthalmological disease once Ig therapy is withdrawn in responding patients. A description of current symptoms is used for comparison to demonstrate response to Ig therapy at initial review. Maintenance Ig therapy must be given in combination with at least one immunosuppressant medication to all patients. Review Criteria Persistent severe EBA refractory to conventional immunosuppressive therapy Review is not mandated for this condition. All patients will receive an initial trial of three months Ig therapy. Outcome measures are defined to indicate to prescribers of the expected clinical response.
Epidermolysis-bullosa-acquisita2017.docx