title: Self-administered hyaluronidase-facilitated subcutaneous immunoglobulin therapy in complicated primary antibody deficiencies.
abstract: Hyaluronidase-facilitated subcutaneous immunoglobulin (fSCIg) is a new immunoglobulin product for replacement therapy in patients with primary antibody deficiencies (PAD). The pre-administration of recombinant human hyaluronidase associated with 10% immunoglobulin allowed the infusion of larger (up to 600 ml) amounts of immunoglobulin at a single infusion site, enabling patients to receive the necessary treatment in a single monthly dose. Here, we report the effectiveness and the tolerability of fSCIg in patients with severe PAD-related comorbidities: refractory autoimmune thrombocytopenia; systemic granulomatous disease; severe enteropathy, and Type I diabetes. We conclude that fSCIg could be a feasible option to improve the adherence to replacement therapy also by patients with severe PAD.
authors.affiliation: Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche & Ospedali Riuniti, Ancona, Italy.
authors.forename: Maria Giovanna
authors.initials: MG
authors.lastname: Danieli
authors.affiliation: Department of Molecular Medicine, Sapienza Medical University, Rome, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Pulvirenti
authors.affiliation: Department of Clinical & Sperimental Medicine AOUP, Pisa, Italy.
authors.forename: Valeria
authors.initials: V
authors.lastname: Rocchi
authors.affiliation: Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche & Ospedali Riuniti, Ancona, Italy.
authors.forename: Ramona
authors.initials: R
authors.lastname: Morariu
authors.affiliation: Department of Molecular Medicine, Sapienza Medical University, Rome, Italy.
authors.forename: Isabella
authors.initials: I
authors.lastname: Quinti
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications / Q000276:immunology / Q000628:therapy*; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000276:immunology / Q000628:therapy*; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000276:immunology / Q000628:therapy*; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000150:complications / Q000276:immunology / Q000628:therapy*; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000276:immunology / Q000628:therapy*; D006801:Humans; D006821:Hyaluronoglucosaminidase / Q000378:metabolism; D007116:Immunization, Passive / Q000379:methods*; D055104:Infusions, Subcutaneous; D008286:Malabsorption Syndromes / Q000150:complications / Q000276:immunology / Q000628:therapy*; D055118:Medication Adherence; D008875:Middle Aged; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications / Q000276:immunology / Q000628:therapy*; D012646:Self Administration
chemical_list: D006821:Hyaluronoglucosaminidase
keywords: X-linked agammaglobulinemia; common variable immunodeficiency; cytopenias; enteropathy; facilitated subcutaneous immunoglobulin; granulomatous disease; immunomodulation, intravenous immunoglobulin; subcutaneous immunoglobulin; thrombocytopenia