Clinical management of patients with primary immunodeficiencies during the COVID-19 pandemic

ABSTRACT

Introduction: Patients affected by Inborn Errors of Immunity (IEI) represent a potential group-at-risk in the current COVID-19 pandemic. Studies on large and small cohorts of IEI reported a huge variability clinical manifestations associated to SARS-Cov-2, ranging from asymptomatic, mild, moderate/severe to death. A great impulse to improve remote assistance programs and to switch to home-based treatment to reduce mobility and face to face contacts has been implemented.

Areas covered: The authors completed a comprehensive review of the literature by searching the PubMed database for studies on large and small cohorts and case reports of IEI patients with COVID-19, with the aim to provide useful information for their clinical management during the COVID-19 pandemic.

Expert opinion: Surprisingly, a low number of IEI patients affected by SARS-Cov-2 were reported with a risk to die for COVID-19 overlapping that of the general population. The low number might be explained by the choice of most physicians to inform early in the pandemic about safety measures, to switch most of the IEI patients to home therapy and to remote assistance. The guidelines issued by the scientific societies and periodically updated, represent the best tool for the clinical management of IEI patients.

KEYWORDS:

• Inborn errors of immunity (IEI)
• SARS-CoV-2
• COVID-19
• immunoglobulin replacement
• convalescent plasma
• remote assistance
• home-based treatment
• guidelines
• virus escape mutants

Article highlights

• COVID-19 in IEI patients does not appear to be significantly different from the general population with the exception of a younger age, and a longer length of SARS-Cov-2 positivity.

• Most patients with antibody production defects do not experience severe disease.

• Patients with predominantly T cell defects, and defects of innate immunity are under-represented in the reported cohorts preventing an assessment of any correlation with COVID-19 severity.

• Co-morbid conditions appear to be relative rare, but important in determining outcome.

• IEI patients with COVID-19 should be referred to their consultant specialists due to the high variability of COVID-19 presentation even within the same IEI condition.

Acknowledgments

We thank all MD, nurses, and health personnel taking care of COVID-19 patients over the world.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Additional information

Funding

This paper was funded by Progetto Ateneo Sapienza, 2020.