ABSTRACT
COVID-19 manifestations range from asymptomatic to life-threatening infections. The outcome in different inborn errors of immunity (IEI) is still a matter of debate. In this retrospective study, we describe the experience of the of the Italian Primary Immunodeficiencies Network (IPINet). Sixteen reference centers for adult or pediatric IEI were involved. One hundred fourteen patients were enrolled including 35 pediatric and 79 adult patients. Median age was 32 years, and male-to-female ratio was 1.5:1. The most common IEI were 22q11.2 deletion syndrome in children (26%) and common variable immunodeficiency (CVID) in adults (65%). Ninety-one patients did not require hospital admission, and among these, 33 were asymptomatic. Hospitalization rate was 20.17%. Older age (p 0.004) and chronic lung disease (p 0.0008) represented risk factors for hospitalization. Hospitalized patients mainly included adults suffering from humoral immunodeficiencies requiring immunoglobulin replacement therapy and as expected had lower B cell counts compared to non-hospitalized patients. Infection fatality rate in the whole cohort was 3.5%. Seroconversion was observed is 86.6% of the patients evaluated and in 83.3% of CVID patients. 16.85% of the patients reported long-lasting COVID symptoms. All but one patient with prolonged symptoms were under IgRT. The fatality rate observed in IEI was slightly similar to the general population. The age of the patients who did not survive was lower compared to the general population, and the age stratified mortality in the 50-60 age range considerable exceeded the mortality from 50 to 60 age group of the Italian population (14.3 vs 0.6%; p < 0.0001). We hypothesize that this is due to the fact that comorbidities in IEI patients are very common and usually appear early in life.
Subject(s)
COVID-19 , Common Variable Immunodeficiency , Adult , COVID-19/complications , COVID-19/epidemiology , Child , Common Variable Immunodeficiency/epidemiology , Female , Hospitalization , Humans , Male , Retrospective Studies , SARS-CoV-2 , Post-Acute COVID-19 SyndromeSubject(s)
Antibodies, Viral/immunology , COVID-19/immunology , COVID-19/virology , Gastrointestinal Tract/virology , Host-Pathogen Interactions , Respiratory System/virology , SARS-CoV-2/immunology , Viral Load , Age Factors , Antibodies, Viral/blood , COVID-19/epidemiology , Gastrointestinal Tract/immunology , Host-Pathogen Interactions/immunology , Humans , Italy/epidemiology , Respiratory System/immunologySubject(s)
Agammaglobulinemia/diagnosis , B-Lymphocytes/immunology , COVID-19/diagnosis , Immunologic Deficiency Syndromes/diagnosis , Lung/diagnostic imaging , Myasthenia Gravis/diagnosis , SARS-CoV-2/physiology , T-Lymphocytes/immunology , Thymoma/diagnosis , Thymus Neoplasms/diagnosis , Continuous Positive Airway Pressure , Fatal Outcome , Humans , Male , Middle Aged , Tomography, X-Ray ComputedSubject(s)
Antibodies, Bacterial/blood , COVID-19/complications , Mucocutaneous Lymph Node Syndrome/complications , Mycoplasma pneumoniae/pathogenicity , Pneumonia, Mycoplasma/complications , SARS-CoV-2/pathogenicity , Severe Acute Respiratory Syndrome/complications , Adolescent , Blotting, Western , COVID-19/diagnosis , COVID-19/pathology , COVID-19/virology , COVID-19 Testing , Child , Child, Preschool , Coinfection , Conjunctivitis/diagnosis , Conjunctivitis/physiopathology , Edema/diagnosis , Edema/physiopathology , Exanthema/diagnosis , Exanthema/physiopathology , Female , Fever/diagnosis , Fever/physiopathology , Humans , Infant , Male , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/pathology , Mucocutaneous Lymph Node Syndrome/virology , Mycoplasma pneumoniae/growth & development , Pneumonia, Mycoplasma/diagnosis , Pneumonia, Mycoplasma/microbiology , Pneumonia, Mycoplasma/pathology , SARS-CoV-2/growth & development , Severe Acute Respiratory Syndrome/diagnosis , Severe Acute Respiratory Syndrome/pathology , Severe Acute Respiratory Syndrome/virology , Stomatitis/diagnosis , Stomatitis/physiopathologyABSTRACT
BACKGROUND: There is uncertainty about the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in individuals with rare inborn errors of immunity (IEI), a population at risk of developing severe coronavirus disease 2019. This is relevant not only for these patients but also for the general population, because studies of IEIs can unveil key requirements for host defense. OBJECTIVE: We sought to describe the presentation, manifestations, and outcome of SARS-CoV-2 infection in IEI to inform physicians and enhance understanding of host defense against SARS-CoV-2. METHODS: An invitation to participate in a retrospective study was distributed globally to scientific, medical, and patient societies involved in the care and advocacy for patients with IEI. RESULTS: We gathered information on 94 patients with IEI with SARS-CoV-2 infection. Their median age was 25 to 34 years. Fifty-three patients (56%) suffered from primary antibody deficiency, 9 (9.6%) had immune dysregulation syndrome, 6 (6.4%) a phagocyte defect, 7 (7.4%) an autoinflammatory disorder, 14 (15%) a combined immunodeficiency, 3 (3%) an innate immune defect, and 2 (2%) bone marrow failure. Ten were asymptomatic, 25 were treated as outpatients, 28 required admission without intensive care or ventilation, 13 required noninvasive ventilation or oxygen administration, 18 were admitted to intensive care units, 12 required invasive ventilation, and 3 required extracorporeal membrane oxygenation. Nine patients (7 adults and 2 children) died. CONCLUSIONS: This study demonstrates that (1) more than 30% of patients with IEI had mild coronavirus disease 2019 (COVID-19) and (2) risk factors predisposing to severe disease/mortality in the general population also seemed to affect patients with IEI, including more younger patients. Further studies will identify pathways that are associated with increased risk of severe disease and are nonredundant or redundant for protection against SARS-CoV-2.