Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL)

Andersen, J. B.; Midttun, Knut; Feragen, Kristin Billaud

doi:10.1186/s41687-019-0101-x

Cited by 9 publications

(6 citation statements)

References 49 publications

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“…QoL is a multidimensional concept that encompasses several elements such as the physical, psychological and social well-being of individuals. The main point of QoL is to assess an individual’s perception of the impact of illness on his/her life at a similar level to that of the clinical factors in the disease’s prognosis [ 67 ]. RRTI has a critical effect on the QoL of CVID patients.…”

Section: Discussionmentioning

confidence: 99%

Trained Immunity Based-Vaccines as a Prophylactic Strategy in Common Variable Immunodeficiency. A Proof of Concept Study

et al. 2020

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Background. A major concern in the care of common variable immunodeficiency (CVID) patients is the persistence of subclinical or recurrent respiratory tract infections (RRTI) despite adequate trough IgG levels, which impacts the quality of life (QoL) and morbidity. Therefore, the development of new approaches to prevent and treat infection, especially RRTI, is necessary. Objectives. We conducted a clinical observational study from May, 2016 to December, 2017 in 20 CVID patients; ten of these patients had a history of RRTI and received the polybacterial preparation MV130, a trained immunity-based vaccine (TIbV) to assess its impact on their QoL and prognosis. Methods. Subjects with RRTI received MV130 for 3 months and were followed up to 12 months after initiation of the treatment. The primary endpoint was a reduction in RRTI at the end of the study. We analyzed the pharmacoeconomic impact on the RRTI group before and after immunotherapy by estimating the direct and indirect costs, and assessed CVID-QoL and cytokine profile. Specific antibody responses to the bacteria contained in MV130 were measured. Results. The RRTI-group treated with TIbV MV130 showed a significant decrease in infection rate (p = 0.006) throughout the 12 months after initiation of the treatment. A decrease in antibiotic use and unscheduled outpatient visits was observed (p = 0.005 and p = 0.002, respectively). Significant increases in anti-pneumococcus and anti-MV130 IgA antibodies (p = 0.039 both) were detected after 12 months of MV130. Regarding the CVID QoL questionnaire, an overall decrease in the score by more than 50% was observed (p < 0.05) which demonstrated that patients experienced an improvement in their QoL. The pharmacoeconomic analysis showed that the real annual direct costs decreased up to 4 times per patient with the prophylactic intervention (p = 0.005). Conclusion. The sublingual administration of the TIbV MV130 significantly reduced the rate of respiratory infections, antibiotic use and unscheduled visits, while increasing specific IgA responses in CVID patients. Additionally, the CVID population felt that their QoL was improved, and a decrease in expenses derived from health care was predicted.

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Section: Discussionmentioning

confidence: 99%

Trained Immunity Based-Vaccines as a Prophylactic Strategy in Common Variable Immunodeficiency. A Proof of Concept Study

et al. 2020

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“…One reason for the lack of correlation could also be the choice of a survey tool. As discussed previously, few studies have sought to validate quality of life surveys in patients with IEI 6 , 35 , and these have focused on humoral immune deficiencies, primarily in adult patients. This survey was chosen for its broad utilization in a range of diagnoses, in addition to the simplicity and short length.…”

Section: Discussionmentioning

confidence: 99%

The Integration of Patient-Reported Quality of Life and Systemic Biomarkers in Patients with Immune Dysregulation

LaBere,

Chu,

Platt

et al. 2023

Preprint

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Background Patient-reported quality of life measurements are an important method for improving the treatment of patients with a variety of diseases. These tools have been minimally investigated in patients with inborn errors of immunity (IEI). Patients with IEI may have immune dysregulation and autoimmune-mediated multi-system organ involvement, making treatment optimization vitally important. Routine laboratory and radiologic testing are typically used for treatment monitoring; however, these modalities have the potential to miss early organ damage. T follicular helper cells are T cells that contribute to antibody production and are known to be expanded in patients with active autoimmunity. We hypothesized that a combination of patient-reported quality of life measurements, in addition to T follicular helper cell percentages, would help us to better understand the level of disease activity in patients with IEI and autoimmunity.Methods Patients with immune dysregulation were consented to provide a blood sample and to complete a questionnaire. The Centers for Disease Control HRQOL-14 tool was utilized for the questionnaire portion, and T follicular helper cell levels were measured from whole blood using surface staining and flow cytometry analysis. Patient disease activity was abstracted from the patient medical record, and this was compared to the questionnaire and whole blood assay results.Results A total of 20 patients participated in the study; 8 patients had active disease and the remaining were found to be quiescent. There was no significant difference between the patient-reported general health ratings based on sex, age, disease activity, or category of immune dysregulation (p > 0.05). The cTfh percentages were expanded in patients with active disease as compared to those with quiescent (p < 0.05). However, there was no significant correlation between cTfh percentage and patient-reported unhealthy days from the questionnaire (R2 = 0.113, p > 0.05).Conclusions Patients with active immune dysregulation were found to have expanded cTfh percentages as compared to those with quiescent disease, however this was not reflected in patient-reported quality of life questionnaires. Better understanding of disease activity and the patient experience is vital to optimize appropriate treatments and outcomes for patients with IEI and immune dysregulation, and more investigation is needed.

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“…When the patients have not consented to such consultations, their psychoemotional condition is worth assessing using appropriate scales, for example, Beck, Hamilton, and QLQ. [61][62][63][64][65][66][67][68]…”

Section: Psychological and Psychiatric Evaluationmentioning

confidence: 99%

Protocols of Standard of Care for Adult Patients with Primary Antibody Deficiencies Will Improve Timing of Diagnosis, Survival, and Quality of Life

Napiórkowska-Baran¹,

Więsik-Szewczyk²,

Ziętkiewicz³

et al. 2022

IJAAI

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The majority of primary immunodeficiencies (PIDs) are antibody deficiencies (PADs), and not all of them are rare diseases; As an example, Caucasian individuals suffer from selective IgA deficiency at a frequency of 1:500.In addition to infections, symptomatic patients with PAD are more likely to develop neoplastic, autoimmune, and allergic diseases. In the event that PAD is neglected or delayed for more than ten years, complications develop, eventually resulting in death. No studies have been conducted to devise and report detailed ready-to-use protocols for managing PAD to date.This study aimed to propose protocols and guidelines for the adult PAD patients’ standard care. Preparing the protocol, we considered the frequency and type of laboratory tests, imaging, endoscopic examinations, specialist consultations, and standardized recommendations for further care in the place of residence. As a result of the proposed monitoring scheme, patients can be provided with complete care in terms of their underlying conditions and comorbidities, as well as early detection of complications. This protocol will serve as a guide for physicians dealing with these patients and enable comparisons of patient groups across a variety of treatment centers, even far away from each other. A national consultant in the field of clinical immunology verified the protocol mainly developed by Polish experts from reference immunology centres for adults.

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Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL)

Cited by 9 publications

References 49 publications

Trained Immunity Based-Vaccines as a Prophylactic Strategy in Common Variable Immunodeficiency. A Proof of Concept Study

Trained Immunity Based-Vaccines as a Prophylactic Strategy in Common Variable Immunodeficiency. A Proof of Concept Study

The Integration of Patient-Reported Quality of Life and Systemic Biomarkers in Patients with Immune Dysregulation

Protocols of Standard of Care for Adult Patients with Primary Antibody Deficiencies Will Improve Timing of Diagnosis, Survival, and Quality of Life

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