COVID-19 manifestations range from asymptomatic to life-threatening infections. The outcome in different inborn errors of immunity (IEI) is still a matter of debate. In this retrospective study, we describe the experience of the of the Italian Primary Immunodeficiencies Network (IPINet). Sixteen reference centers for adult or pediatric IEI were involved. One hundred fourteen patients were enrolled including 35 pediatric and 79 adult patients. Median age was 32 years, and maleto-female ratio was 1.5:1. The most common IEI were 22q11.2 deletion syndrome in children (26%) and common variable immunodeficiency (CVID) in adults (65%). Ninety-one patients did not require hospital admission, and among these, 33 were asymptomatic. Hospitalization rate was 20.17%. Older age (p 0.004) and chronic lung disease (p 0.0008) represented risk factors for hospitalization. Hospitalized patients mainly included adults suffering from humoral immunodeficiencies requiring immunoglobulin replacement therapy and as expected had lower B cell counts compared to non-hospitalized patients. Infection fatality rate in the whole cohort was 3.5%. Seroconversion was observed is 86.6% of the patients evaluated and in 83.3% of CVID patients. 16.85% of the patients reported long-lasting COVID symptoms. All but one patient with prolonged symptoms were under IgRT. The fatality rate observed in IEI was slightly similar to the general population. The age of the patients who did not survive was lower compared to the general population, and the age stratified mortality in the 50-60 age range considerable exceeded the mortality from 50 to 60 age group of the Italian population (14.3 vs 0.6%; p < 0.0001). We hypothesize that this is due to the fact that comorbidities in IEI patients are very common and usually appear early in life.

The Impact of SARS-CoV-2 Infection in Patients with Inborn Errors of Immunity: the Experience of the Italian Primary Immunodeficiencies Network (IPINet) / Giuliana Giardino; Cinzia Milito; Vassilios Lougaris; Alessandra Punziano; Maria Carrabba; Francesco Cinetto; Riccardo Scarpa; Rosa Maria Dellepiane; Silvia Ricci; Beatrice Rivalta; Francesca Conti; Antonio Marzollo; Davide Firinu; Emilia Cirillo; Gianluca Lagnese; Caterina Cancrini; Baldassare Martire; Maria Giovanna Danieli; Andrea Pession; Angelo Vacca; Chiara Azzari; Giovanna Fabio; Annarosa Soresina; Carlo Agostini; Giuseppe Spadaro; Raffaele Badolato; Maria Pia Cicalese; Alessandro Aiuti; Alessandro Plebani; Isabella Quinti; Claudio Pignata. - In: JOURNAL OF CLINICAL IMMUNOLOGY. - ISSN 0271-9142. - ELETTRONICO. - 42:(2022), pp. 935-946. [10.1007/s10875-022-01264-y]

The Impact of SARS-CoV-2 Infection in Patients with Inborn Errors of Immunity: the Experience of the Italian Primary Immunodeficiencies Network (IPINet)

Silvia Ricci;Chiara Azzari;Claudio Pignata
2022

Abstract

COVID-19 manifestations range from asymptomatic to life-threatening infections. The outcome in different inborn errors of immunity (IEI) is still a matter of debate. In this retrospective study, we describe the experience of the of the Italian Primary Immunodeficiencies Network (IPINet). Sixteen reference centers for adult or pediatric IEI were involved. One hundred fourteen patients were enrolled including 35 pediatric and 79 adult patients. Median age was 32 years, and maleto-female ratio was 1.5:1. The most common IEI were 22q11.2 deletion syndrome in children (26%) and common variable immunodeficiency (CVID) in adults (65%). Ninety-one patients did not require hospital admission, and among these, 33 were asymptomatic. Hospitalization rate was 20.17%. Older age (p 0.004) and chronic lung disease (p 0.0008) represented risk factors for hospitalization. Hospitalized patients mainly included adults suffering from humoral immunodeficiencies requiring immunoglobulin replacement therapy and as expected had lower B cell counts compared to non-hospitalized patients. Infection fatality rate in the whole cohort was 3.5%. Seroconversion was observed is 86.6% of the patients evaluated and in 83.3% of CVID patients. 16.85% of the patients reported long-lasting COVID symptoms. All but one patient with prolonged symptoms were under IgRT. The fatality rate observed in IEI was slightly similar to the general population. The age of the patients who did not survive was lower compared to the general population, and the age stratified mortality in the 50-60 age range considerable exceeded the mortality from 50 to 60 age group of the Italian population (14.3 vs 0.6%; p < 0.0001). We hypothesize that this is due to the fact that comorbidities in IEI patients are very common and usually appear early in life.
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935
946
Giuliana Giardino; Cinzia Milito; Vassilios Lougaris; Alessandra Punziano; Maria Carrabba; Francesco Cinetto; Riccardo Scarpa; Rosa Maria Dellepiane; Silvia Ricci; Beatrice Rivalta; Francesca Conti; Antonio Marzollo; Davide Firinu; Emilia Cirillo; Gianluca Lagnese; Caterina Cancrini; Baldassare Martire; Maria Giovanna Danieli; Andrea Pession; Angelo Vacca; Chiara Azzari; Giovanna Fabio; Annarosa Soresina; Carlo Agostini; Giuseppe Spadaro; Raffaele Badolato; Maria Pia Cicalese; Alessandro Aiuti; Alessandro Plebani; Isabella Quinti; Claudio Pignata
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2158/1287328
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