Health Related Quality of Life in Common Variable Immunodeficiency

PURPOSE: To quantify the health related quality of life in primary immunodeficiency patients. MATERIALS AND METHODS: We used generic health status and general psychological health questionnaires to determine the range of issues that needed to be considered in examining the burden of common variable immunodeficiency (CVID). RESULTS: The health status of patients with CVID was lower than that observed in normal subjects. Overall, Role-Physical and General Health scales correlated with a poorer clinical status. Surprisingly, the duration of disease did not influence health status. Being female, older, General Health Questionnaire-positive and alexithymic proved to be major risk factors associated with a poor health status. Patients with chronic lung disease and chronic diarrhea had the lowest values on the Medical Outcome Study, Short Form SF-36 (SF-36) scales. Disease severity perception was associated with the General Health Questionnaire and alexithymia status. Limitations in daily activities as a result of lower physical health were the major problems facing common variable immunodeficiency patients. CONCLUSION: Our data underlined the importance of conducting a periodical health related quality of life assessment on patients with primary antibody deficiencies and, moreover, stressed the necessity of providing psychological support to at risk patients.

Italian network of primary immunodeficiencies

Primary immunodeficiencies are rare diseases, characterized by an increased susceptibility to infections. Early diagnosis and appropriate treatment are critical for reducing morbidity and mortality. Given the rarity of these diseases, the awareness of these disorders by physicians is often insufficient, leading to delayed diagnosis and inappropriate treatment which are the major causes of severe long term complications. In an attempt to address and resolve these problems an Italian Network on primary immunodeficiencies has been established with the aim to increase the awareness of these disorders among physicians and to provide the best clinical assistance to all patients on the national territory