Plasmapheresis is unavailable Confirmation by histopathology of thrombotic small vessel occlusion OR in at least one or tissue is desirable but should not delay IVIg Plasmapheresis is contraindicated therapy if indicated. Review Review is not mandated for this condition. Given that Ig is one-off treatment in this Criteria setting, review is not mandated, however Clinical effectiveness of Ig therapy can be outcome measures have been defined demonstrated by: including requesting a description of the Survival clinical response to Ig therapy. • The Specialist Working Group discussion noted the importance of gathering data on rare conditions to inform the future review of the Criteria for access and to demonstrate the benefit of Ig therapy. It was noted that it would be useful to dedicate resources to the follow-up of prescribers to record outcome information in future. Dose Confirmation by histopathology of Induction Dose 2 g/kg over 5 days Dosing is unchanged and advice is provided – thrombotic small vessel occlusion in at regarding when re-treatment is indicated. . least one organ or tissue is desirable Retreatment may be required in early relapse or but should not delay IVIg therapy if occurrence of a second episode. indicated. A single treatment is usually sufficient, The aim should be to use the lowest dose possible based on a dose of 2 g/kg divided over that achieves the appropriate clinical outcome for 2–5 days. The potential each patient.
Confirmation by histopathology of thrombotic small vessel occlusion in at least one or tissue is desirable but should not delay IVIg therapy if indicated. Plasmapheresis is unavailable OR Plasmapheresis is contraindicated Review Criteria Review is not mandated for this condition. Clinical effectiveness of Ig therapy can be demonstrated by: • Survival Given that Ig is one-off treatment in this setting, review is not mandated, however outcome measures have been defined including requesting a description of the clinical response to Ig therapy. The Specialist Working Group discussion noted the importance of gathering data on rare conditions to inform the future review of the Criteria for access and to demonstrate the benefit of Ig therapy. It was noted that it would be useful to dedicate resources to the follow-up of prescribers to record outcome information in future. Dose Confirmation by histopathology of thrombotic small vessel occlusion in at least one organ or tissue is desirable but should not delay IVIg therapy if indicated. A single treatment is usually sufficient, based on a dose of 2 g/kg divided over 2–5 days. The potential Induction Dose – 2 g/kg over 5 days Retreatment may be required in early relapse or occurrence of a second episode. The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient. Dosing is unchanged and advice is provided regarding when re-treatment is indicated. .
Catastrophic-anti-phospholipid-syndrome2017.docx