COVID-19 Outcomes Vary Widely in Patients With Primary Immunodeficiencies

By Will Boggs MD

October 20, 2020

NEW YORK (Reuters Health) - Clinical outcomes of COVID-19 in patients with primary immunodeficiency diseases range from mild illness to death, according to a small case series.

"We were first surprised that we had only a few cases considering how many immune-deficient persons we see and treat," Dr. Charlotte Cunningham-Rundles of Icahn School of Medicine at Mount Sinai, in New York City, told Reuters Health by email. "I have to assume everyone stayed home."

Data on the clinical impact of SARS-CoV-2 infections in patients with primary immunodeficiency diseases (PID) remain limited, she and her colleagues note in The Journal of Allergy and Clinical Immunology: In Practice.

In their report, the researchers describe the clinical features and outcomes of 16 patients with PID who tested positive for SARS-CoV-2. Among these patients (median age, 44.5 years), nine had common variable immunodeficiency, three had X-linked agammaglobulinemia linked to Bruton tyrosine kinase (BTK) mutations, and three had other PID diagnoses. Seven patients had pre-existing PID-associated autoimmune/inflammatory complications.

Twelve patients required hospitalization, including five who required intensive care. Most of the patients (10/16) required oxygen supplementation. Other treatments included hydroxychloroquine in nine patients, azithromycin in 11 patients, steroids in five patients, and convalescent plasma under expanded access protocols in five patients. Two patients received investigational agents during clinical trials.

The median time from symptom onset to resolution or death was 29 days. Four individuals died, including three who had pre-existing PID-associated autoimmune/inflammatory complications (two of these also had pre-existing PID-associated chronic lung disease) and one who was a kidney transplant recipient. Their ages ranged from 39 to 76 years.

This death rate (25%) was greater than that in the general population with COVID-19 reported at New York City hospitals (10.2%) and similar to COVID-19 outcomes data reported in kidney transplant populations (28%).

Three patients developed detectable serum SARS-CoV-2-specific IgG antibodies, and one patient had detectable SARS-CoV-2 spike protein-specific IgM.

"We were surprised that some of our very immune-deficient subjects actually made antibody that we could detect," Dr. Cunningham-Rundles said. "How long? I don't know, but we will test that, and also if it neutralizes the virus."

"We're still learning," she said.

"A multinational registry, previously established by the International Union of Immunology Sciences (IUIS;, will be crucial for understanding the full clinical and immunological impact of Covid-19 in PIDs," the authors conclude.

SOURCE: The Journal of Allergy and Clinical Immunology: In Practice, online October 8, 2020.