Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK

Author:

Shields Adrian M1ORCID,Anantharachagan Ariharan2,Arumugakani Gururaj3,Baker Kenneth4,Bahal Sameer5,Baxendale Helen6,Bermingham William7,Bhole Malini8,Boules Evon9,Bright Philip10,Chopra Charu11,Cliffe Lucy12,Cleave Betsy12,Dempster John13,Devlin Lisa14,Dhalla Fatima15,Diwakar Lavanya16,Drewe Elizabeth12,Duncan Christopher17,Dziadzio Magdalena18ORCID,Elcombe Suzanne19,Elkhalifa Shuayb20ORCID,Gennery Andrew21,Ghanta Harichandrana22,Goddard Sarah16,Grigoriadou Sofia23ORCID,Hackett Scott24,Hayman Grant25,Herriot Richard26,Herwadkar Archana20,Huissoon Aarnoud7,Jain Rashmi15,Jolles Stephen27,Johnston Sarah10,Khan Sujoy28,Laffan James25,Lane Peter1,Leeman Lucy29,Lowe David M305,Mahabir Shanti31,Lochlainn Dylan James Mac15,McDermott Elizabeth12,Misbah Siraj15,Moghaddas Fiona10,Morsi Hadeil15,Murng Sai25,Noorani Sadia32,O’Brien Rachael33,Patel Smita15,Price Arthur31,Rahman Tasneem25,Seneviratne Suranjith30,Shrimpton Anna9,Stroud Catherine19,Thomas Moira34,Townsend Katie25,Vaitla Prashantha12,Verma Nisha30,Williams Anthony22,Burns Siobhan O305,Savic Sinisa3ORCID,Richter Alex G1

Affiliation:

1. Clinical Immunology Service, Institute of Immunology and Immunotherapy, University of Birmingham , UK

2. Lancashire Teaching Hospitals NHS Foundation Trust , Preston, Lancashire , UK

3. Department of Clinical Immunology and Allergy, St James University Hospital, Leeds Teaching Hospital NHS Trust , Leeds , UK

4. NIHR Newcastle Biomedical Research Centre, Newcastle University and Newcastle upon Tyne Hospitals NHS Foundation Trust , Newcastle upon Tyne , UK

5. Department of Immunology, Royal Free London NHS Foundation Trust , London , UK

6. Royal Papworth NHS Foundation Trust , Cambridge , UK

7. University Hospitals Birmingham NHS Foundation Trust , Birmingham , UK

8. The Dudley Group NHS Foundation Trust , Birmingham , UK

9. Clinical Immunology and Allergy Department, Sheffield Teaching Hospitals NHS Foundation Trust , UK

10. Clinical Immunology, North Bristol NHS Trust , Bristol , UK

11. Department of Haematology & Immunology, Royal Infirmary of Edinburgh , NHS Lothian , UK

12. Clinical Immunology and Allergy Department, Nottingham University Hospital NHS Trust , Nottingham , UK

13. Specialist Allergy and Clinical Immunology, University College London Hospitals , London , UK

14. Regional Immunology Service, The Royal Hospitals , Belfast , UK

15. Department of Clinical Immunology, Oxford University Hospitals NHS Foundation Trust , Oxford , UK

16. Department of Immunology, Royal Stoke Hospital , Stoke-on-Trent , UK

17. Translational and Clinical Research Institute, Immunity and Inflammation Theme, Newcastle University , Newcastle upon Tyne , UK

18. Ear Institute, University College London , London , UK

19. Regional Department of Clinical Immunology & Allergy, Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Foundation Trust , UK

20. Immunology Department, Salford Royal NHS Foundation Trust , Manchester , UK

21. Translational and Clinical Research Institute, Newcastle University, and Paediatric Stem Cell Transplant Unit, Great North Children’s Hospital , Newcastle upon Tyne , UK

22. Department of Allergy and Clinical Immunology, University Hospital Southampton NHS Trust, University of Southampton , Southampton , UK

23. Immunology Department, Royal London Hospital, Barts Health NHS Trust , London , UK

24. Paediatric Immunology Department, University Hospitals of Birmingham , Birmingham , UK

25. Clinical Immunology Service, South West London Immunodeficiency Centre, Epsom and St Helier University Hospital NHS Trust , London , UK

26. Immunology Department, Aberdeen Royal Infirmary , Aberdeen , UK

27. Immunodeficiency Centre for Wales, University Hospital of Wales , Heath Park, Cardiff , UK

28. Hull University Teaching Hospitals NHS Trust , Hull , UK

29. University Hospitals Plymouth NHS Trust , Plymouth , UK

30. Institute of Immunity and Transplantation, University College London , London , UK

31. Clinical Immunology and Allergy Department , Leicester Royal Infirmary, Leicester , UK

32. Clinical Immunology Department, Sandwell & West Birmingham Hospitals NHS Trust , Birmingham , UK

33. Department of Clinical Immunology, Frimley Park Hospital , Frimley, Surrey , UK

34. Clinical Immunology Service, NHS Greater Glasgow and Clyde , Glasgow , UK

Abstract

Abstract In March 2020, the United Kingdom Primary Immunodeficiency Network (UKPIN) established a registry of cases to collate the outcomes of individuals with PID and SID following SARS-CoV-2 infection and treatment. A total of 310 cases of SARS-CoV-2 infection in individuals with PID or SID have now been reported in the UK. The overall mortality within the cohort was 17.7% (n = 55/310). Individuals with CVID demonstrated an infection fatality rate (IFR) of 18.3% (n = 17/93), individuals with PID receiving IgRT had an IFR of 16.3% (n = 26/159) and individuals with SID, an IFR of 27.2% (n = 25/92). Individuals with PID and SID had higher inpatient mortality and died at a younger age than the general population. Increasing age, low pre-SARS-CoV-2 infection lymphocyte count and the presence of common co-morbidities increased the risk of mortality in PID. Access to specific COVID-19 treatments in this cohort was limited: only 22.9% (n = 33/144) of patients admitted to the hospital received dexamethasone, remdesivir, an anti-SARS-CoV-2 antibody-based therapeutic (e.g. REGN-COV2 or convalescent plasma) or tocilizumab as a monotherapy or in combination. Dexamethasone, remdesivir, and anti-SARS-CoV-2 antibody-based therapeutics appeared efficacious in PID and SID. Compared to the general population, individuals with PID or SID are at high risk of mortality following SARS-CoV-2 infection. Increasing age, low baseline lymphocyte count, and the presence of co-morbidities are additional risk factors for poor outcome in this cohort.

Publisher

Oxford University Press (OUP)

Subject

Immunology,Immunology and Allergy

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