title: 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.
authors.affiliation: Health Services Research Unit, Istituto Dermopatico dell'Immacolata (IDI), Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) , Rome , Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Tabolli
authors.affiliation: Health Services Research Unit, Istituto Dermopatico dell'Immacolata (IDI), Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) , Rome , Italy.
authors.forename: Patrizia
authors.initials: P
authors.lastname: Giannantoni
authors.affiliation: Department of Molecular Medicine, Sapienza University of Roma , Rome , Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Pulvirenti
authors.affiliation: Department of Molecular Medicine, Sapienza University of Roma , Rome , Italy.
authors.forename: Fabiola
authors.initials: F
authors.lastname: La Marra
authors.affiliation: Department of Molecular Medicine, Sapienza University of Roma , Rome , Italy.
authors.forename: Guido
authors.initials: G
authors.lastname: Granata
authors.affiliation: Department of Molecular Medicine, Sapienza University of Roma , Rome , Italy.
authors.forename: Cinzia
authors.initials: C
authors.lastname: Milito
authors.affiliation: Department of Molecular Medicine, Sapienza University of Roma , Rome , Italy.
authors.forename: Isabella
authors.initials: I
authors.lastname: Quinti
keywords: GHQ-12; SF-36; common variable immune deficiencies; health-related quality of life; immunoglobulins