Abstract
PurposeCommon variable immunodeficiency disorder (CVID) is a primary immunodeficiency disease (PIDD) often associated with severe and chronic infections. Patients commonly receive immunoglobulin (Ig) treatment to reduce the cycle of recurrent infection and improve physical functioning. However, how Ig treatment in CVID affects quality of life (QOL) has not been thoroughly evaluated. The purpose of a recent Immune Deficiency Foundation (IDF) mail survey was to assess the factors that are associated with QOL in patients with CVID receiving Ig treatment.MethodsA 75-question survey developed by the IDF and a 12-item Short Form Health Survey (SF-12) to assess QOL were mailed to adults with CVID. Mean SF-12 scores were compared between patients with CVID and the general US adult population normative sample.ResultsOverall, 945 patients with CVID completed the surveys. More than half of the patients (54.9%) received intravenous Ig and 44.9% received subcutaneous Ig treatment. Patients with CVID had significantly lower SF-12 scores compared with the general US population regardless of sex or age (p < 0.05). Route of IgG replacement did not dramatically improve QOL. SF-12 scores were highest in patients with CVID who have well-controlled PIDD, lacked physical impairments, were not bothered by treatment, and received Ig infusions at home.ConclusionThese data provide insight into what factors are most associated with physical and mental health, which can serve to improve QOL in patients in this population. Improvements in QOL can result from early detection of disease, limiting digestive system disease, attention to fatigue, and implementation of an individual treatment plan for the patient.
Highlights
Electronic supplementary material The online version of this article contains supplementary material, which is available to authorized users.Primary immunodeficiency diseases (PIDD) comprises a group of over 300 disorders affecting immune system function
Individuals with the following criteria were identified via the Immune Deficiency Foundation (IDF) patient database: adult patients with primary immunodeficiency disease (PIDD), parent/caretakers of persons with PIDD, and persons from the IDF 2013 National Patient Survey identified as current users of Ig (n = 1083)
Mean SF-12 scores were compared between adult patients with common variable immunodeficiency (CVID) (n = 945) and the general United States (US) adult population normative sample (2009; N = 6045)
Summary
Primary immunodeficiency diseases (PIDD) comprises a group of over 300 disorders affecting immune system function. The most common, accounting for 35–63% of cases, is common variable immunodeficiency (CVID) [1,2,3], which is characterized by deficiencies in immunoglobulin (Ig) quantity and quality. The average time from symptom onset to diagnosis ranges from 4.0 to 12.4 years [2, 5] (depending upon the method of the population surveyed); this delay in diagnosis may primarily reflect patients with CVID (accounting for 63% of the PIDD diagnoses in this survey) who present uniformly across the age continuum and are. The incidence of CVID is likely underreported, with patients often misdiagnosed or undiagnosed [7, 8]. Increasing educational efforts and awareness have potentially resulted in increasing prevalence of the disorders [9]
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