ITEM CRITERIAv2.1 PROPOSED REVISIONS TO THE CRITERIA SPECIALIST WORKING RATIONALE FOR ADDITIONS/CLARIFICATIONS Catastrophic anti-phospholipid syndrome Catastrophic antiphospholipid No change Condition syndrome Name Specialty Immunology Immunology No change Category Exceptional circumstances only Exceptional circumstances only No change Catastrophic anti-phospholipid syndrome Catastrophic antiphospholipid No change Specific Conditions syndrome of Insufficient data (). Insufficient data (Category 4a) No change Evidence Justification the rarity of this condition, the evidence level is Justification for recommendation added. for Evidence unlikely to extend beyond case reports and series. Category An international registry of patients with Catastrophic APS (Cervera et al, 2016) was established in 2000 by the European Forum on antibodies and contains data from around 400 patients. Retrospective data analysis demonstrates that patients treated by plasmapheresis and Ig therapy demonstrate superior clinical outcomes. Indication Intravenous immunoglobulin (IVIg) Confirmed diagnosis of CAPS with clinical The Specialist Working Group drafted a more may be appropriate therapy for deterioration post conclusion of steroid and clearly defined indication after consideration catastrophic antiphospholipid of the literature which confirmed the role of plasmapheresis therapy or where therapies are syndrome, a term that describes the contraindicated or plasmapheresis in not available. plasmapheresis in the management of CAPS. accelerated form of antiphospholipid syndrome characterised by
ITEM CRITERIAv2.1 PROPOSED REVISIONS TO THE CRITERIA SPECIALIST WORKING RATIONALE FOR ADDITIONS/CLARIFICATIONS Condition Name Catastrophic antiphospholipid syndrome Catastrophic anti-phospholipid syndrome No change Specialty Immunology Immunology No change Category Exceptional circumstances only Exceptional circumstances only No change Specific Conditions Catastrophic antiphospholipid syndrome Catastrophic anti-phospholipid syndrome No change of Evidence Insufficient data (). Insufficient data (Category 4a) No change Justification for Evidence Category the rarity of this condition, the evidence level is unlikely to extend beyond case reports and series. An international registry of patients with Catastrophic APS (Cervera et al, 2016) was established in 2000 by the European Forum on antibodies and contains data from around 400 patients. Retrospective data analysis demonstrates that patients treated by plasmapheresis and Ig therapy demonstrate superior clinical outcomes. Justification for recommendation added. Indication Intravenous immunoglobulin (IVIg) may be appropriate therapy for catastrophic antiphospholipid syndrome, a term that describes the accelerated form of antiphospholipid syndrome characterised by Confirmed diagnosis of CAPS with clinical deterioration post conclusion of steroid and plasmapheresis therapy or where therapies are contraindicated or plasmapheresis in not available. The Specialist Working Group drafted a more clearly defined indication after consideration of the literature which confirmed the role of plasmapheresis in the management of CAPS.
Catastrophic-anti-phospholipid-syndrome2017.docx