12 October 2020

People with rare inherited immune disorders who become infected with COVID-19 have similar disease outcomes to the general population, according to a new study.

However, the researchers found that patients with primary immunodeficiency (PID) have a greater risk of being admitted into intensive care, and that the average age of affected patients was lower than in the general population.

The study was jointly led by Prof Stuart Tangye from the Garvan Institute of Medical Research, Australia, and Prof Isabelle Meyts from KU Leuven, Belgium. They say their findings into the outcomes for 94 people with PID will further our understanding of the components of the immune system that underpin an effective coronavirus immune response.

Prof Tangye, leader of the immunity and inflammation research theme at Garvan, said they wanted to find out the impact of SARS-CoV-2 infection on those individuals with rare immune disorders, who were assumed to be at risk of severe COVID-19.

Because PID is so rare, the study involved global research collaboration across 50 centres. The researchers invited clinical immunologists from around the world who manage patients with inborn errors of immunity to complete a questionnaire if their patients had contracted the SARS-CoV-2 virus. Data was collected from patients in the USA, UK, France, Spain, Italy, Germany, the Netherlands and Latin America.

“The findings show that pre-existing immune deficiencies were generally not found to be a significant risk factor as the rate of fatality from COVID-19 was no higher in this group than the general population,” said Prof Tangye.

Prof Meyts, clinical lead of the primary immunodeficiency care program at University Hospitals Leuven, said: “Some immune defects even appeared to be protective against the dramatic immune pathology that is frequently seen in severe disease. However, our study suggests younger male patients with PIDs are more likely to endure severe COVID-19 and require ICU admission.”

Out of the 94 reported patients, 25 had mild disease and were treated as outpatients, while 59 (63%) were hospitalised. Of those receiving hospital care, 13 were given non-invasive breathing assistance and 15 were admitted to intensive care for invasive ventilation.

Nine of the 94 patients (9.6%) died from COVID-19, which is within the range of global data of COVID-19 case mortality of 1-20%, and they all had existing comorbidities, such as heart failure, chronic kidney or lung disease, and diabetes.

“Our findings warrant a recommendation for further stringent personal protective measures for patients affected by PIDs,” said Prof Meyts.

She said 56% of the patient cohort had a deficiency in their ability to produce antibodies, yet they had similar outcomes to the rest of the cohort. What’s more those who were completely unable to produce antibodies all recovered following infection.

The study also found that patients with gene defects that mean the body is unable to respond to the pro-inflammatory effects of interleukin 6 (IL-6) developed little or no disease when infected with the SARS-CoV-2 virus.

“Our findings suggest that certain forms of immune suppression, which reduce the function of IL-6, are protective against the pathological effects of the cytokine storm frequently observed in patients,” says Prof Tangye.

The researchers say further studies are needed to gain a comprehensive understanding of which components of the immune system are crucial to a successful coronavirus defence.

The study was published in the Journal of Allergy and Clinical Immunology.


Meyts I, Bucciol G, Quinti I, Neven B, Fischer A, Seoane E, Lopez-Granados E, Gianelli C, Robles-Marhuenda A, Jeandel PY, Paillard C, Sankaran VG, Demirdag YY, Lougaris V, Aiuti A, Plebani A, Milito C, Dalm VA, Guevara-Hoyer K, Sánchez-Ramón S, Bezrodnik L, Barzaghi F, Gonzalez-Granado LI, Hayman GR, Uzel G, Mendonça LO, Agostini C, Spadaro G, Badolato R, Soresina A, Vermeulen F, Bosteels C, Lambrecht BN, Keller M, Mustillo PJ, Abraham RS, Gupta S, Ozen A, Karakoc-Aydiner E, Baris S, Freeman A, Yamazaki-Nakashimada M, Scheffler-Mendoza S, Espinosa-Padilla S, Gennery AR, Jolles S, Espinoza Y, Poli MC, Fieschi C, Hauck F, Cunningham-Rundles C, Mahlaoui N; IUIS Committee of Inborn Errors of Immunity, Warnatz K, Sullivan KE, Tangye SG. (2020) “Coronavirus Disease 2019 in patients with inborn errors of immunity: an international study.” J Allergy Clin Immunol, doi:  10.1016/j.jaci.2020.09.010


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