TY - JOUR
T1 - The Italian Registry for Primary Immunodeficiencies (Italian Primary Immunodeficiency Network; IPINet)
T2 - Twenty Years of Experience (1999-2019)
AU - Lougaris, Vassilios
AU - Pession, Andrea
AU - Baronio, Manuela
AU - Soresina, Annarosa
AU - Rondelli, Roberto
AU - Gazzurelli, Luisa
AU - Benvenuto, Alessio
AU - Martino, Silvana
AU - Gattorno, Marco
AU - Biondi, Andrea
AU - Zecca, Marco
AU - Marinoni, Maddalena
AU - Fabio, Giovanna
AU - Aiuti, Alessandro
AU - Marseglia, Gianluigi
AU - Putti, Maria Caterina
AU - Agostini, Carlo
AU - Lunardi, Claudio
AU - Tommasini, Alberto
AU - Bertolini, Patrizia
AU - Gambineri, Eleonora
AU - Consolini, Rita
AU - Matucci, Andrea
AU - Azzari, Chiara
AU - Danieli, Maria Giovanna
AU - Paganelli, Roberto
AU - Duse, Marzia
AU - Cancrini, Caterina
AU - Moschese, Viviana
AU - Chessa, Luciana
AU - Spadaro, Giuseppe
AU - Civino, Adele
AU - Vacca, Angelo
AU - Cardinale, Fabio
AU - Martire, Baldassare
AU - Carpino, Luigi
AU - Trizzino, Antonino
AU - Russo, Giovanna
AU - Cossu, Fausto
AU - Badolato, Raffaele
AU - Pietrogrande, Maria Cristina
AU - Quinti, Isabella
AU - Rossi, Paolo
AU - Ugazio, Alberto
AU - Pignata, Claudio
AU - Plebani, Alessandro
PY - 2020/10
Y1 - 2020/10
N2 - 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.
AB - 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.
U2 - 10.1007/s10875-020-00844-0
DO - 10.1007/s10875-020-00844-0
M3 - Article
C2 - 32803625
VL - 40
SP - 1026
EP - 1037
JO - Journal of Clinical Immunology
JF - Journal of Clinical Immunology
SN - 0271-9142
IS - 7
ER -