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

V. Lougaris, A. Pession, M. Baronio, A. Soresina, R. Rondelli, L. Gazzurelli, A. Benvenuto, S. Martino, M. Gattorno, A. Biondi, M. Zecca, M. Marinoni, G. Fabio, A. Aiuti, G. Marseglia, M.C. Putti, C. Agostini, C. Lunardi, A. Tommasini, P. BertoliniE. Gambineri, R. Consolini, A. Matucci, C. Azzari, M.G. Danieli, R. Paganelli, M. Duse, C. Cancrini, V. Moschese, L. Chessa, G. Spadaro, A. Civino, A. Vacca, F. Cardinale, B. Martire, L. Carpino, A. Trizzino, G. Russo, F. Cossu, R. Badolato, M.C. Pietrogrande, I. Quinti, P. Rossi, A. Ugazio, C. Pignata, A. 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 - 2020

Fingerprint

Dive into the research topics of 'The Italian Registry for Primary Immunodeficiencies (Italian Primary Immunodeficiency Network; IPINet): Twenty Years of Experience (1999–2019)'. Together they form a unique fingerprint.

Cite this