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

Abstract

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.

Keywords: CVID_QoL; Health-related quality of life; common variable immunodeficiency; immunoglobulin replacement treatment; patient empowerment.

Fig. 1

Study design flow-chart. Abbreviation: CVID_QoL, common variable immunodeficiency quality of life questionnaire; fSCIG, facilitated subcutaneous immunoglobulin; GHQ-12 GHQ-12, questionnaire; IVIG, intravenously-administered immunoglobulins; IVIG + SCIG, immunoglobulin replacement therapy by combined intravenous and subcutaneous route of administration; IgRT, immunoglobulin replacement treatment; SCIG, subcutaneous Immunoglobulins

Fig. 2

CVID-related clinical conditions (panel a) and mean number of co-morbidities (panel b) observed in CVID grouped according to their IgRT during the observational time. p values by the t test. **p ≤ 0.01. Abbreviation: CVID, common variable immunodeficiency; COPD, chronic obstructive pulmonary diseases; IVIG, intravenously-administered immunoglobulins; SCIG, subcutaneous Immunoglobulins; fSCIG, facilitated subcutaneous immunoglobulin; IVIG + SCIG, immunoglobulin replacement therapy by combined intravenous and subcutaneous route of administration

Fig. 3

GHQ status and CVID_QoL score. Proportion of GHQ-12-positive participants and global. EF, RF, and GSS scores (panel a) recorded for CVID grouped according to the route of IgG replacement treatment. CVID_QoL global. EF, RF, and GSS are expressed as percentage of the respective total score with higher values indicating increasing disability. Scores of eight single items of the CVID_QoL related to the immunoglobulin treatment (panel b). p values by the t test. **p ≤ 0.01. Abbreviation: CVID_QoL, common variable immunodeficiency quality of life questionnaire; EF, emotional functioning; IVIG, intravenously administered immunoglobulins; fSCIG, facilitated subcutaneous immunoglobulin; GHQ-12, GHQ-12 questionnaire; GSS, gastrointestinal and skin symptoms; IgRT, immunoglobulin replacement treatment; IVIG + SCIG, immunoglobulin replacement therapy by combined intravenous and subcutaneous route of administration; Q, question; RF, relational functioning; SCIG, subcutaneous immunoglobulins