Impact of SARS-CoV-2 infection and COVID-19 on patients with inborn errors of immunity

Abel, Laurent; Al-Muhsen, Salah; Aiuti, Alessandro; Al‐Muhsen, Saleh; Al‐Mulla, Fahd; Anderson, Mark S.; Andreakos, Evangelos; Novelli, Antonio; Arias, Andrés A.; Feldman, Hagit Baris; Belot, Alexandre; Biggs, Catherine M.; Bousfiha, Ahmed Aziz; Brodin, Petter; Christodoulou, John; Condino‐Neto, Antônio; Dalgard, Clifton L.; Espinosa-Padilla, Sara; Fellay, Jacques; Flores, Carlos; Franco, José Luis; Froidure, Antoine; Haerynck, Filomeen; Halwani, Rabih; Hammarström, Lennart; Henrickson, Sarah E.; Hsieh, Elena W.Y.; Itan, Yuval; Karamitros, Timokratis; Lau, Yu‐Lung; Mansouri, Davood; Meyts, Isabelle; Mogensen, Trine H.; Morio, Tomohiro; Ng, Lisa F. P.; Notarangelo, Luigi D.; Novelli, Giuseppe; Okada, Satoshi; Özçelık, Tayfun; Pan‐Hammarström, Qiang; Diego, Rebeca Pérez de; Prando, Carolina; Pujol, Aurora; Rénia, Laurent; Resnick, Igor B.; Rodríguez‐Gallego, Carlos; Sancho‐Shimizu, Vanessa; Seppänen, Mikko R. J.; Shcherbina, Anna; Snow, Andrew L.; Soler‐Palacín, Pere; Spaan, András N.; Tancevski, Ivan; Tangye, Stuart G.; Tayoun, Ahmad Abou; Temel, Şehime Gülsün; Turvey, Stuart E.; Uddin, M.J.; Vinh, Donald C.; Zatz, Mayana; Okamoto, Ken‐ichi; Pelin, David S.; Pesole, Graziano; Beek, Diederik van de; Colobran, Roger; Wauters, Joost; Su, Helen C.; Casanova, Jean‐Laurent

doi:10.1016/j.jaci.2022.11.010

Cited by 28 publications

(36 citation statements)

References 179 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…This reinforces the advisability of vaccination in IEI patients, despite SARS-CoV-2 infection, although the role of humoral response is not fully defined for COVID-19 (79,80), but seems important for certain IEI (e.g. CVID) (17).…”

Section: Discussionsupporting

confidence: 53%

“…The comparisons between IEI subgroups (IgRT -which is associated to humoral immunity impairment-, immunosuppressive treatment, or cellular vs. humoral immunodeficiencies) did not show differences in the COVID-19 severity. These are factors commonly associated with worse outcomes (17,(24)(25)(26). The absence of detectable differences in our cohort could be ascribed to the limited samples size or to the young age of the patients, as being young age is a major protective factor for severe COVID-19 (2).…”

Section: Discussionmentioning

confidence: 99%

“…This work was supported by the projects PI18/00223, FI19/00208 and PI21/00211 of LA, integrated in the Plan Nacional de I+D+I and co-financed by the ISCIII -Subdireccioń General de Evaluacioń y Fomento de la Investigacioń Sanitariaand the Fondo Europeo de Desarrollo Regional (FEDER), by Pla Estratègic de Recerca i Innovacióen Salut (PERIS), Departament de Salut, Generalitat de Catalunya (SLT006/17/00199 to LA), by a 2017 Leonardo Grant for Researchers and Cultural Creators, BBVA Foundation (IN [17] _BBM_CLI_0357) to LA, by a 2017 Beca de Investigacioń de la Sociedad Española de Inmunologıá Clıńica Alergologıá y Asma Pediatrica to LA, and by a 2021 Beca de Investigación de la Sociedad Española de Inmunologıá Clıńica, Alergologıá y Asma Pediatrica to AD-M. By a 2022 Convocatòria de Beques de Recerca IRSJD -Carmen de Torres 2022 (2022AR-IRSJD-CdTorres) and CERCA Programme/Generalitat de Catalunya.…”

Section: Data Availability Statementmentioning

confidence: 99%

“…Inborn errors of immunity (IEI) are a heterogeneous group of 485 disorders classified into 10 groups according to the immune components involved (16). A recent report of over 1330 patients with IEI reported so far shows that COVID-19 generally manifests clinically at a younger age, runs a more protracted course, and has a more severe outcome requiring hospitalization and/or ICU admission in many individuals with IEI compared to the epidemiology of SARS-CoV-2 infection in the general population (17). Although patients with IEI have had a heterogeneous clinical evolution during SARS-CoV-2 pandemics (18), in general terms COVID-19 has been less severe in children and young adults (19-21) compared to older IEI patients.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Acute and long-term immune responses to SARS-CoV-2 infection in unvaccinated children and young adults with inborn errors of immunity

et al. 2023

View full text Add to dashboard Cite

PurposeTo describe SARS-CoV-2 infection outcome in unvaccinated children and young adults with inborn errors of immunity (IEI) and to compare their specific acute and long-term immune responses with a sex-, age-, and severity-matched healthy population (HC).MethodsUnvaccinated IEI patients up to 22 years old infected with SARS-CoV-2 were recruited along with a cohort of HC. SARS-CoV-2 serology and ELISpot were performed in the acute phase of infection (up to 6 weeks) and at 3, 6, 9, and 12 months.ResultsTwenty-five IEI patients (median age 14.3 years, min.-max. range 4.5-22.8; 15/25 males; syndromic combined immunodeficiencies: 48.0%, antibody deficiencies: 16.0%) and 17 HC (median age 15.3 years, min.-max. range 5.4-20.0; 6/17 males, 35.3%) were included. Pneumonia occurred in 4/25 IEI patients. In the acute phase SARS-CoV-2 specific immunoglobulins were positive in all HC but in only half of IEI in whom it could be measured (n=17/25): IgG+ 58.8% (10/17) (p=0.009); IgM+ 41.2% (7/17)(p<0.001); IgA+ 52.9% (9/17)(p=0.003). Quantitative response (index) was also lower compared with HC: IgG IEI (3.1 ± 4.4) vs. HC (3.5 ± 1.5)(p=0.06); IgM IEI (1.9 ± 2.4) vs. HC (3.9 ± 2.4)(p=0.007); IgA IEI (3.3 ± 4.7) vs. HC (4.6 ± 2.5)(p=0.04). ELISpots positivity was qualitatively lower in IEI vs. HC (S-ELISpot IEI: 3/11, 27.3% vs. HC: 10/11, 90.9%; p=0.008; N-ELISpot IEI: 3/9, 33.3% vs. HC: 11/11, 100%; p=0.002) and also quantitatively lower (S-ELISpot IEI: mean index 3.2 ± 5.0 vs. HC 21.2 ± 17.0; p=0.001; N-ELISpot IEI: mean index 9.3 ± 16.6 vs. HC: 39.1 ± 23.7; p=0.004). As for long term response, SARS-CoV-2-IgM+ at 6 months was qualitatively lower in IEI(3/8, 37.5% vs. 9/10 HC: 90.0%; p=0.043), and quantitatively lower in all serologies IgG, M, and A (IEI n=9, 1.1 ± 0.9 vs. HC n=10, 2.1 ± 0.9, p=0.03; IEI n=9, 1.3 ± 1.5 vs. HC n=10, 2.9 ± 2.8, p=0.02; and IEI n=9, 0.6 ± 0.5 vs. HC n=10, 1.7 ± 0.8, p=0.002 –respectively) but there were no differences at remaining time points.ConclusionsOur IEI pediatric cohort had a higher COVID-19 pneumonia rate than the general age-range population, with lower humoral and cellular responses in the acute phase (even lower compared to the reported IEI serological response after SARS-CoV-2 vaccination), and weaker humoral responses at 6 months after infection compared with HC.

show abstract

Section: Discussionsupporting

confidence: 53%

Section: Discussionmentioning

confidence: 99%

Section: Data Availability Statementmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Acute and long-term immune responses to SARS-CoV-2 infection in unvaccinated children and young adults with inborn errors of immunity

et al. 2023

View full text Add to dashboard Cite

show abstract

“…The course of COVID-19 in patients with IEI and CVID has been reported in several studies (17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27). Study designs vary and overall mortality of COVID-19 in CVID is difficult to assess, but a recent review by Tangye et al estimated the case fatality rate in CVID to 7% as compared to 1-4% in the general population (28). Notably, known risk factors for severe COVID-19 as age, obesity and chronic lung disease are of significant importance also in CVID (19,(22)(23)(24).…”

Section: Cellular Immunity In Natural Infections In Cvid Covid-19 Mor...mentioning

confidence: 99%

Cellular immunity in COVID-19 and other infections in Common variable immunodeficiency

Løken

Fevang

2023

Front. Immunol.

View full text Add to dashboard Cite

COVID-19 has shed light on the role of cellular immunity in the absence of humoral response in different patient groups. Common variable immunodeficiency (CVID) is characterized by impaired humoral immunity but also an underlying T-cell dysregulation. The impact of T-cell dysregulation on cellular immunity in CVID is not clear, and this review summarizes available literature on cellular immunity in CVID with a particular focus on COVID-19. Overall mortality of COVID-19 in CVID is difficult to assess, but seems not significantly elevated, and risk factors for severe disease mirrors that of the general population, including lymphopenia. Most CVID patients have a significant T-cell response to COVID-19 disease with possible cross-reactivity to endemic coronaviruses. Several studies find a significant but impaired cellular response to basal COVID-19 mRNA vaccination that is independent of an antibody response. CVID patients with infection only have better cellular responses to vaccine in one study, but there is no clear association to T-cell dysregulation. Cellular response wane over time but responds to a third booster dose of vaccine. Opportunistic infection as a sign of impaired cellular immunity in CVID is rare but is related to the definition of the disease. CVID patients have a cellular response to influenza vaccine that in most studies is comparable to healthy controls, and annual vaccination against seasonal influenza should be recommended. More research is required to clarify the effect of vaccines in CVID with the most immediate issue being when to booster the COVID-19 vaccine.

show abstract

The protection of CoronaVac against the infection of wild‐type SARS‐CoV‐2 (WH‐09) or Omicron variant in nude‐hACE2 mice

Lin

Liu

Sun

et al. 2023

Anim Models and Exp Med

View full text Add to dashboard Cite

BackgroundImmunocompromised individuals have an increased risk of severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection and severe outcomes, but we pay less attention to these people. Athymic nude mice are a murine strain with a spontaneous deficiency of the Foxn1 gene, which can result in thymic degeneration or its absence, leading to immunosuppression and a decrease in the number of T cells, and are widely used in preclinical evaluations of disease in immunocompromised populations.MethodsWe investigated the protection of the coronavirus disease 2019 (COVID‐19) inactivated vaccine (CoronaVac) against the infection of wild‐type SARS‐CoV‐2 (WH‐09) or Omicron variant utilizing a hybrid‐type nude‐hACE2 mouse model.ResultsCompared with nude‐hACE2/W mice, the viral load in the brain and lung tissue of nude‐hACE2 mice (nude‐hACE2/WV) infected with WH‐09 after vaccination significantly decreased, and the histopathological changes were also reduced. The viral load in the brain and lung tissue of nude‐hACE2 mice (nude‐hACE2/OV) infected with the Omicron variant after vaccination was lower than that in nude‐hACE2/O, but histopathological symptoms did not improve significantly.ConclusionCoronaVac provides some protection against infection of both WH‐09 and the Omicron variant in the nude‐hACE2 mice. Our findings aimed to provide a reference for vaccination against SARS‐CoV‐2 in immunocompromised populations.

show abstract

Impact of SARS-CoV-2 infection and COVID-19 on patients with inborn errors of immunity

Cited by 28 publications

References 179 publications

Acute and long-term immune responses to SARS-CoV-2 infection in unvaccinated children and young adults with inborn errors of immunity

Acute and long-term immune responses to SARS-CoV-2 infection in unvaccinated children and young adults with inborn errors of immunity

Cellular immunity in COVID-19 and other infections in Common variable immunodeficiency

The protection of CoronaVac against the infection of wild‐type SARS‐CoV‐2 (WH‐09) or Omicron variant in nude‐hACE2 mice

Contact Info

Product

Resources

About