Development and Initial Validation of a Questionnaire to Measure Health-Related Quality of Life of Adults with Common Variable Immune Deficiency: The CVID_QoL Questionnaire

Quinti, Isabella; Pulvirenti, Federica; Giannantoni, Patrizia; Hajjar, Joud; Canter, Debra; Milito, Cinzia; Abeni, Damiano; Orange, Jordan S.; Tabolli, Stefano

doi:10.1016/j.jaip.2016.07.012

Cited by 32 publications

(32 citation statements)

References 29 publications

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“…Before and despite adequate Ig replacement, recurrent RTIs are the commonest clinical feature in symptomatic PADs and have great impact on patients' quality of life [43,46,47]. Upper and lower RTIs are included in the list of 10 warning signs for PID promoted by the Jeffrey Model Foundation [48,49].…”

Section: Acute Infectionsmentioning

confidence: 99%

The broad spectrum of lung diseases in primary antibody deficiencies

et al. 2018

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Human primary immunodeficiency diseases (PIDs) represent a heterogeneous group of more than 350 disorders. They are rare diseases, but their global incidence is more relevant than generally thought. The underlying defect may involve different branches of the innate and/or adaptive immune response. Thus, the clinical picture may range from severe phenotypes characterised by a broad spectrum of infections to milder infectious phenotypes due to more selective (and frequent) immune defects. Moreover, infections may not be the main clinical features in some PIDs that might present with autoimmunity, auto-inflammation and/or cancer. Primary antibody deficiencies (PADs) represent a small percentage of the known PIDs but they are the most frequently diagnosed, particularly in adulthood. Common variable immunodeficiency (CVID) is the most prevalent symptomatic PAD.PAD patients share a significant susceptibility to respiratory diseases that represent a relevant cause of morbidity and mortality. Pulmonary complications include acute and chronic infection-related diseases, such as pneumonia and bronchiectasis. They also include immune-mediated interstitial lung diseases, such as granulomatous-lymphocytic interstitial lung disease (GLILD) and cancer. Herein we will discuss the main pulmonary manifestations of PADs, the associated functional and imaging findings, and the relevant role of pulmonologists and chest radiologists in diagnosis and surveillance.

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Section: Acute Infectionsmentioning

confidence: 99%

The broad spectrum of lung diseases in primary antibody deficiencies

et al. 2018

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“…While a cohort of European patients with markedly different levels of access to IgRT showed similar HRQoL [ 6 ], a similar US-based assessment indicated that a regular access to therapy is beneficial in perceived health [ 7 ]. We have previously shown that such an HRQoL elicitation did not reveal any influence of Ig administration schedule in an Italian cohort, using generic and disease-specific instruments [ 8 – 10 ]. As with other chronic conditions, treatment of patients living with CVID could impose demands on daily life to plan and self-care management, as a result of the need to comply with complex therapeutic schedules, while balancing family or job commitments.…”

Section: Introductionmentioning

confidence: 99%

“…Hence, the importance of defining a tailored immunoglobulin treatment plan for each patient’s situation has been shown [ 13 – 15 ], with clear implications on patient well-being [ 16 , 17 ]. Our own work indicates that patients with CVID manifest substantial restrictions and poor HRQoL, which worsen over the time, mainly due to CVID-associated clinical conditions [ 8 – 10 ].…”

Section: Introductionmentioning

confidence: 99%

“…This investigation followed a long period of patients’ education and training aimed to establish the best treatment option. HRQoL was assessed by a recently validated new instrument: the CVID_QoL questionnaires [ 10 ], together with the GHQ-12, a tool to detect nonpsychotic, minor psychiatric disorders, such as depression and anxiety [ 18 ]. The study sought to generate information to help health care professionals to understand factors that may impact on patient’s everyday life and possibly contribute to maximizing patient empowerment and satisfaction with care, while minimizing the impact of illness.…”

Section: Introductionmentioning

confidence: 99%

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Health-Related Quality of Life in Patients with CVID Under Different Schedules of Immunoglobulin Administration: Prospective Multicenter Study

et al. 2019

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Objective We assessed the health-related quality of life (HRQoL) in CVID adults receiving different schedules of immunoglobulin replacement therapy (IgRT) by intravenous (IVIG), subcutaneous (SCIG), and facilitated (fSCIG) preparations. For these patients, IgRT schedule was chosen after a period focused on identifying the most suitable individual option. Methods Three hundred twenty-seven participants were enrolled in a prospective, observational, 18-month study. Participants received IgRT for at least 2 years. The first 6 months were devoted to the educational process during which the choices related to IgRT were regularly re-assessed, and the shift to alternative regimen was permitted. During the following 12 months, clinical data were prospectively collected, and only patients who did not further modify their IgRT schedule were included in the analysis of HRQoL measured by CVID_QoL, a specific instrument, and by GHQ-12, a tool to assess minor psychiatric nonpsychotic disorders. Results Three hundred four patients were included in the analysis. CVID_QoL global score and its dimensions (emotional functioning, relational functioning, gastrointestinal symptoms) were similar in IVIG, SCIG, and fSCIG recipients. Patients receiving IgRT by different routes of administration reported similar capacity to make long-term plans, discomfort due to therapy, and concern to run out of medications. Multivariate analysis revealed the GHQ-12 status, but not the IgRT mode of administration, as the major factor impacting on treatment-related QoL items, and a significant impact of age on discomfort related to IgRT. Conclusions IgRT schedules do not impact the HRQoL in CVID if the treatment is established after an extensive educational period focused on individualizing the best therapeutic regimen. Electronic supplementary material The online version of this article (10.1007/s10875-019-0592-5) contains supplementary material, which is available to authorized users.

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“…About 20 patients in their cohort had severe T-cell defects (low T-cell numbers and opportunistic infections); with these distinct differences, they prefer the term late-onset combined immunodeficiency rather than CVID for these subjects. In a second article on CVID, Quinti et al 11 present a 32-item questionnaire to measure specific health-related quality of life for adults with CVID, which they then validated on 118 subjects, identifying main indicators of reduced quality of life in these subjects. The third original article concerns the diagnosis of methylenetetrahydrofolate dehydrogenase deficiency made in 2 brothers who had lymphopenia, opportunistic and bacterial infections, and macrocytosis.…”

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confidence: 99%