Changes in health-related quality of life in common variable immunodeficiency: an eight-year journey, including the COVID-19 pandemic

ABSTRACT

Background

Personalized medicine requires the assessment of the impact of health care interventions on Health-Related Quality of Life.

Research design and methods

We run an observational study of HRQoL in 140 CVID patients with biannual assessments over 8  years using a disease-specific tool, the CVID_QoL, and the GHQ questionnaires. Factors influencing changes in HRQoL scores were identified using multiple linear regression models with a stepwise procedure.

Results

Infections frequency, female gender, and chronic enteropathy were associated with worse global CVID_QoL scores. The presence of permanent organ damage and older age contributed to the perception of being at risk of health deterioration, while chronic enteropathy was associated with fatigue. The presence of permanent organ damage was also associated with perceived difficulties in usual activities. The frequency of infections was the main risk factor for difficulties in long-term planning and perceptions of vulnerability. Before COVID-19, improved HRQoL scores were associated with reduced respiratory infections and changes in immunoglobulin replacement route and setting. The COVID-19 pandemic caused a sudden deterioration in all HRQoL dimensions, and a further deterioration in the emotional dimension was observed during the pandemic period. Patients who died during the study had worse CVID_QoL scores at all time points, confirming that HRQoL performance is strongly related to patient outcome.

Conclusions

Periodic HRQoL assessments are needed to capture relevant issues that change over time in patients affected by long-term chronic conditions such CVID, possibly identifying areas of intervention.

KEYWORDS:

• Clinical phenotypes
• common variable immunodeficiency
• COVID-19
• CVID_QoL questionnaire
• general health questionnaire
• health related quality of life
• immunoglobulin replacement

Acknowledgments

We would like to thank patients and their families. We would like also to thank Stefano Tabolli for giving us his suggestions. This work is generated within the European Reference Network for Rare Immunological Dis-orders (ERN-RITA). The views and opinions expressed are those of the authors only and do not necessarily reflect those of the European Union or the European Commission. Neither the European Union nor the European Commission can be held responsible for them. We thank the Jeffrey Modell Foundation for their support.

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.

Author contributions

Conceptualization F Pulvirenti and I Quinti; methodology, F Pulvirenti and I Quinti; formal analysis, F Pulvirenti: investigation, A Villa, M D’Ambrosi, G Cusa, E de la Fuente-Munoz, P Quijada-Morales, and G Garzi; resources, I Quinti; data curation, F Pulvirenti; writing original draft preparation, F Pulvirenti and I Quinti: writing, review and editing, A Villa, P Quijada-Morales; project administration, F Pulvirenti; funding acquisition, I Quinti. All authors have read and agreed to the published version of the manuscript.

Data availability statement

The datasets generated and/or analyzed during the current study are available from the corresponding author on reasonable request to researchers who provide a methodologically sound proposal. The data will be provided after their de-identification, in compliance with applicable privacy laws, data protection, and requirements for consent and anonymization.

Ethics statement

The study was approved by the Ethical Committee of the Sapienza University of Rome, Prot. 316/2016, and Prot. 0279/2021, and performed in accordance with the Good Clinical Practice guidelines, the International Conference on Harmonization guidelines, and the most recent version of the Declaration of Helsinki.

Supplementary material

Supplemental data for this article can be accessed online at https://doi.org/10.1080/1744666X.2024.2368195

Additional information

Funding

The research leading to these results has received funding from the European Union - NextGenerationEU through the Italian Ministry of University and Research under PNRR - M4C2-I1.3 Project PE_00000019 “HEAL ITALIA” to Isabella Quinti CUP: B53C22004000006.