Longitudinal Study on Health-Related Quality of Life in a Cohort of 96 Patients with Common Variable Immune Deficiencies

Abstract

Health-related quality of life (HRQoL) in common variable immunodeficiency diseases (CVID) was evaluated by different tools, which were mainly used to compare different schedules of immunoglobulins administration in cross-sectional or short-term longitudinal studies. We assessed the HRQoL and psychological status of CVID patients in a longitudinal study over a 6-year period by a generic, non-disease-specific instrument (SF-36), and by a General Health Questionnaire (GHQ-12) for the risk of depression/anxiety. At baseline, 96 patients were enrolled. After 1 year, a second assessment was performed on 92 patients and, after 6 years, a third assessment was performed on 66 patients. Eighteen patients died during the study time. HRQoL was low, with mental health scales less affected than physical scales. A decline in the score on SF-36 scales was observed between the first and the third assessment for the Physical Functioning, Body Pain, General Health, Social Functioning, and Role-Emotional scales. The General Health scale showed a lower score in these patients, when compared to patients with other chronic diseases. Approximately one-third of the patients were at risk of anxiety/depression at all observation times, a percentage that reached two thirds of the patients, considering only the group of females. Over the 6 years of the study, the health condition of 11/66 patients worsened, passing from “GHQ-negative” to “GHQ-positive”; their score on SF-36 scales also decreased. A decrement of one point in each of the Physical Functioning, Vitality, Social Functioning, and Mental Health SF-36 scales increased the risk of developing anxiety/depression from three to five percent. A negative variation of the Physical Functioning score increased the risk of psychological distress. In a survival analysis with dichotomized variables, Physical Functioning scores <50 were associated with a relative risk (RR) of 4.4, whereas Social Functioning scores <37.5 were associated with a RR of 10.0. In our study, it was the clinical condition, as opposed to the different treatment strategies with immunoglobulins, which had a major role on the deterioration of HRQoL. Moreover, in a quality-of-life evaluation, disorders such as anxiety/depression should be assessed, as they yet often go unrecognized. Our results might be helpful in the interpretation of currently available data on quality of life in CVID patients.

Fatigue and the wear-off effect in adult patients with common variable immunodeficiency

“…However, when fitted into a multivariate model their influence on fatigue became insignificant when paired with the other variables in the predicted model. Similarly, CVID patients with chronic GI and lung disease had lower QoL and higher mortality rate than did those free of such complications [8,16], and while permanent lung damage was not a significant variable in predicting fatigue in multivariate analysis, having permanent lung damage increased the odds of fatigue in a univariate analysis [OR = 1·46, P = 0·045, 95% confidence interval (CI) = 1·01-2·13]. However, previous studies showed that, in the general population, fatigue is reported more frequently by females than in males [10,15].…”

“…We have also shown that patients who report fatigue have a significantly lower quality of life (QoL) than those in a normative sample of the general US population (P < 0·05) [7]. Poor QoL is an independent predictor of mortality in CVID [8], especially in patients with severe noninfectious complications [9]. Importantly, it has been projected that improving the QoL score by one point may reduce the risk of death by 2-3% [8].…”

“…Poor QoL is an independent predictor of mortality in CVID [8], especially in patients with severe noninfectious complications [9]. Importantly, it has been projected that improving the QoL score by one point may reduce the risk of death by 2-3% [8]. Focusing on the drivers of QoL in CVID provides an untapped opportunity to improve outcomes, and potentially survival.…”

Patient’s Experience in Pediatric Primary Immunodeficiency Disorders: Computerized Classification of Questionnaires

“…The diagnosis of PID presents a challenge to pediatricians due to rare incidence and unspecific symptoms, resulting in delayed referral and negative outcome for patients and parents (3, 4). The pre-diagnostic experience of patients and their families is not sufficiently integrated into the diagnostic process.…”

“…Although progress in genetic definition has been made, clinical diagnostics remains a challenging task for the general practitioner (GP) or pediatrician. Early diagnosis is of paramount importance because the delay leads to increased mortality, morbidity, and reduced quality of life (3, 4). Although the time to diagnosis varies, diagnostic delay is common (5–8).…”