title: Enzyme therapy in Fabry disease: severe adverse events associated with anti-agalsidase cross-reactive IgG antibodies.
abstract: OBJECTIVE
  To report a severe adverse event related to enzyme replacement therapy with agalsidase in an hemizygous male patient treated for Fabry disease.
  
  
  METHODS
  Retrospective analysis of clinical, radiological and biochemical data in a patient who suffered adverse events related to both agalsidase alfa and agalsidase beta treatments.
  
  
  RESULTS
  A hemizygous male patient was first treated for Fabry disease with agalsidase alfa. After more than 1 year of therapy, infusion-related symptoms necessitated systemic steroids and antihistaminic therapy. Decline in kidney function prompted a switch for agalsidase beta. Anaphylactoid shock occurred after the second infusion. No serum IgE antibodies were disclosed. Skin-test reactivity to agalsidase beta was negative. Following a published rechallenge infusion protocol, agalsidase beta was reintroduced, leading to a second anaphylactoid shock episode. Enzyme replacement therapy was stopped and the patient was treated with symptomatic therapy only. This case was referred to the pharmacovigilance department.
  
  
  CONCLUSIONS
  The negativity of immunological tests (specific anti-agalsidase IgE antibodies and skin tests) does not rule out the risk of repeated anaphylactoid shock following agalsidase infusion.
authors.affiliation: Department of Clinical Pharmacy, Bichat-Claude Bernard University Hospital (AP-HP), Paris, France.
authors.forename: Chloe
authors.initials: C
authors.lastname: Tesmoingt
authors.forename: Olivier
authors.initials: O
authors.lastname: Lidove
authors.forename: Axele
authors.initials: A
authors.lastname: Reberga
authors.forename: Marguerite
authors.initials: M
authors.lastname: Thetis
authors.forename: Chloe
authors.initials: C
authors.lastname: Ackaert
authors.forename: Pascale
authors.initials: P
authors.lastname: Nicaise
authors.forename: Philippe
authors.initials: P
authors.lastname: Arnaud
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
mesh_terms: D000328:Adult; D000707:Anaphylaxis / Q000139:chemically induced*; D000888:Antibodies, Anti-Idiotypic / Q000009:adverse effects*; D056947:Enzyme Replacement Therapy / Q000009:adverse effects*; D000795:Fabry Disease / Q000188:drug therapy*; D006801:Humans; D007527:Isoenzymes / Q000009:adverse effects; D008297:Male; D011994:Recombinant Proteins; D012307:Risk Factors; D016896:Treatment Outcome; D000519:alpha-Galactosidase / Q000009:adverse effects
chemical_list: D000888:Antibodies, Anti-Idiotypic; D007527:Isoenzymes; D011994:Recombinant Proteins; C048844:anti-IgG; C000627036:agalsidase alfa; D000519:alpha-Galactosidase; C459420:agalsidase beta