BACKGROUND: There are no studies of patients with primary immunodeficiency states receiving intravenous immunoglobulin (IVIG) therapy that assess health-related quality of life (HRQOL) using a well-standardized and reproducible method. OBJECTIVES: To determine the HRQOL of patients with common variable immunodeficiency (CVID) requiring IVIG therapy, to compare these patients with patients with diabetes mellitus (DM) and congestive heart failure (CHF), to determine the factors that affect HRQOL, and to develop normative data on the HRQOL of these CVID patients, which can be used to follow the effects of future therapies. METHODS: Fifty-eight adults with CVID receiving IVIG therapy completed the Medical Outcomes Study 36-Item Short-Form Health Survey to evaluate their HRQOL and were compared with DM and CHF patients. The impact of demographic, socioeconomic, and disease-related variables and comorbid conditions was examined in the CVID population. RESULTS: Patients with CVID had lower HRQOL scores in all dimensions compared with patients with DM and in 4 of 8 dimensions compared with patients with CHF. Increasing age and female sex were negatively associated with certain aspects of HRQOL. There were no significant effects from other socioeconomic or disease-related variables or comorbid conditions examined. CONCLUSIONS: Patients with CVID receiving IVIG therapy have a significantly worse HRQOL than patients with other chronic illnesses, indicating there is much room for improvement in future therapies for this primary immunodeficiency state. The effects of future therapies can be evaluated by comparison with the normative data developed in this study.
BACKGROUND: There are no studies of patients with primary immunodeficiency states receiving intravenous immunoglobulin (IVIG) therapy that assess health-related quality of life (HRQOL) using a well-standardized and reproducible method. OBJECTIVES: To determine the HRQOL of patients with common variable immunodeficiency (CVID) requiring IVIG therapy, to compare these patients with patients with diabetes mellitus (DM) and congestive heart failure (CHF), to determine the factors that affect HRQOL, and to develop normative data on the HRQOL of these CVIDpatients, which can be used to follow the effects of future therapies. METHODS: Fifty-eight adults with CVID receiving IVIG therapy completed the Medical Outcomes Study 36-Item Short-Form Health Survey to evaluate their HRQOL and were compared with DM and CHFpatients. The impact of demographic, socioeconomic, and disease-related variables and comorbid conditions was examined in the CVID population. RESULTS:Patients with CVID had lower HRQOL scores in all dimensions compared with patients with DM and in 4 of 8 dimensions compared with patients with CHF. Increasing age and female sex were negatively associated with certain aspects of HRQOL. There were no significant effects from other socioeconomic or disease-related variables or comorbid conditions examined. CONCLUSIONS:Patients with CVID receiving IVIG therapy have a significantly worse HRQOL than patients with other chronic illnesses, indicating there is much room for improvement in future therapies for this primary immunodeficiency state. The effects of future therapies can be evaluated by comparison with the normative data developed in this study.
Authors: P Titman; Z Allwood; C Gilmour; C Malcolmson; C Duran-Persson; C Cale; G Davies; H Gaspar; A Jones Journal: J Clin Immunol Date: 2014-07-09 Impact factor: 8.317
Authors: B Bienvenu; G Cozon; C Hoarau; M Pasquet; P Cherin; P Clerson; E Hachulla; J C Crave; J C Delain; R Jaussaud Journal: Orphanet J Rare Dis Date: 2016-06-22 Impact factor: 4.123