The Italian Registry for Primary Immunodeficiencies (Italian Primary Immunodeficiency Network; IPINet): Twenty Years of Experience (1999-2019)

Vassilios Lougaris, Andrea Pession, Manuela Baronio, Annarosa Soresina, Roberto Rondelli, Luisa Gazzurelli, Alessio Benvenuto, Silvana Martino, Marco Gattorno, Andrea Biondi, Marco Zecca, Maddalena Marinoni, Giovanna Fabio, Alessandro Aiuti, Gianluigi Marseglia, Maria Caterina Putti, Carlo Agostini, Claudio Lunardi, Alberto Tommasini, Patrizia BertoliniEleonora Gambineri, Rita Consolini, Andrea Matucci, Chiara Azzari, Maria Giovanna Danieli, Roberto Paganelli, Marzia Duse, Caterina Cancrini, Viviana Moschese, Luciana Chessa, Giuseppe Spadaro, Adele Civino, Angelo Vacca, Fabio Cardinale, Baldassare Martire, Luigi Carpino, Antonino Trizzino, Giovanna Russo, Fausto Cossu, Raffaele Badolato, Maria Cristina Pietrogrande, Isabella Quinti, Paolo Rossi, Alberto Ugazio, Claudio Pignata, Alessandro Plebani

Research output: Contribution to journalArticlepeer-review

Abstract

Primary immunodeficiencies (PIDs) are heterogeneous disorders, characterized by variable clinical and immunological features. National PID registries offer useful insights on the epidemiology, diagnosis, and natural history of these disorders. In 1999, the Italian network for primary immunodeficiencies (IPINet) was established. We report on data collected from the IPINet registry after 20 years of activity. A total of 3352 pediatric and adult patients affected with PIDs are registered in the database. In Italy, a regional distribution trend of PID diagnosis was observed. Based on the updated IUIS classification of 2019, PID distribution in Italy showed that predominantly antibody deficiencies account for the majority of cases (63%), followed by combined immunodeficiencies with associated or syndromic features (22.5%). The overall age at diagnosis was younger for male patients. The minimal prevalence of PIDs in Italy resulted in 5.1 per 100.000 habitants. Mortality was similar to other European registries (4.2%). Immunoglobulin replacement treatment was prescribed to less than one third of the patient cohort. Collectively, this is the first comprehensive description of the PID epidemiology in Italy.

Original languageEnglish
Pages (from-to)1026-1037
Number of pages12
JournalJournal of Clinical Immunology
Volume40
Issue number7
DOIs
Publication statusPublished - Oct 2020

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