Struggling with COVID-19 in Adult Inborn Errors of Immunity Patients: A Case Series of Combination Therapy and Multiple Lines of Therapy for Selected Patients
Abstract
:1. Introduction
2. Material and Methods
Patient Cohort and Clinical Data
3. Results
Patient Cohort
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | |
---|---|---|---|---|---|
Diagnosis | CVID | NFKB2 (CVID-like) | XLA | APDS1 | CVID |
Genetic Mutation | Not known | NFKB2 (c.2593G > A; p.Asp865Gly) | BTK (c.994C > T; Arg288Trp) | PI3KCD (c.1573G > A; p.Glu525lys) | Not known |
Age | 50 | 50 | 39 | 49 | 69 |
Sex | F | F | M | M | F |
Comorbidity | RA | Splenomegaly, bronchiectasis; recurrent viral infections | Hypertrophic cardiomyopathy; AF; CKD secondary to nephrolithiasis. C | Bronchiectasis; COPD; EBV-related NHL | GLILD; Bronchiectasis; colonization Aspergillus; Sjogren syndrome; CKD |
Chronic steroid therapy | no | no | no | no | no |
Continuative Immunosuppressant | yes (RTX) | no | no | no | no |
Infection period | 02/2020 | 03/2021 | 08/2021 | 03/2022 | 04/2022 |
Presumptive SARS-CoV-2 variant | Wuhan | Delta | Delta | Omicron | Omicron |
Vaccine doses at infection | 0 | 0 | 0 | 3 (BNT162b2) | 3 (BNT162b2) |
Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | |
Diagnosis | CVID | NFKB2 (CVID-like) | XLA | APDS1 | CVID |
Specific treatment (other than steroids in patients requiring O2 therapy) | 1st line: remdesivir 2nd line: CP 3rd line: CP + remdesivir | 1st line: bamlanivimab 2nd line: CP + remdesivir | 1st line: casirivimab/imdevimab 2nd line: sotrovimab + remdesivir | 1st line: sotrovimab 2nd line: remdesivir 3rd line: CP | 1st line: remdesivr + casirivimab/imdevimab |
Hospital admission | yes | yes | yes | yes | yes |
Severity according to WHO classification | moderate | moderate | severe | severe | moderate |
SARS-CoV-2 related pneumonia | yes | yes | yes | yes | no |
O2 supplementation therapy needed | LFNC | LFNC | HFNC | HFNC | LFNC |
Positivity duration (days) | 260 | 26 | 14 | 79 * | 52 |
IgRT | no | yes | yes | yes | discontinued |
Comments about IgRT | IgRT started after COVID-19 | Interrupted for ADR to IVIG | |||
IgG trough level (mg/dL) during infection course | 380 | 750 | 800 | 800 | 200 |
IgM (mg/dL) | 20 | <4 | 1 | 178 | 12 |
IgA (mg/dL) | 6 | 8 | 1 | 1 | 6 |
B cell% before infection | 0 | 0 | 0 | 0.8 | 0.4 |
Bacterial superinfections | yes | no | no | yes | yes |
Death | no | no | no | yes | 0 |
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Share and Cite
Bez, P.; D’ippolito, G.; Deiana, C.M.; Finco Gambier, R.; Pica, A.; Costanzo, G.; Garzi, G.; Scarpa, R.; Landini, N.; Cinetto, F.; et al. Struggling with COVID-19 in Adult Inborn Errors of Immunity Patients: A Case Series of Combination Therapy and Multiple Lines of Therapy for Selected Patients. Life 2023, 13, 1530. https://doi.org/10.3390/life13071530
Bez P, D’ippolito G, Deiana CM, Finco Gambier R, Pica A, Costanzo G, Garzi G, Scarpa R, Landini N, Cinetto F, et al. Struggling with COVID-19 in Adult Inborn Errors of Immunity Patients: A Case Series of Combination Therapy and Multiple Lines of Therapy for Selected Patients. Life. 2023; 13(7):1530. https://doi.org/10.3390/life13071530
Chicago/Turabian StyleBez, Patrick, Giancarlo D’ippolito, Carla Maria Deiana, Renato Finco Gambier, Andrea Pica, Giulia Costanzo, Giulia Garzi, Riccardo Scarpa, Nicholas Landini, Francesco Cinetto, and et al. 2023. "Struggling with COVID-19 in Adult Inborn Errors of Immunity Patients: A Case Series of Combination Therapy and Multiple Lines of Therapy for Selected Patients" Life 13, no. 7: 1530. https://doi.org/10.3390/life13071530