Health-Related Quality of Life in Adult Patients with Common Variable Immunodeficiency Disorders and Impact of Treatment

J Clin Immunol. 2017 Jul;37(5):461-475. doi: 10.1007/s10875-017-0404-8. Epub 2017 May 23.

Abstract

Purpose: Common 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.

Methods: A 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.

Results: Overall, 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.

Conclusion: These 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.

Keywords: Primary immunodeficiency diseases; common variable immunodeficiency; mental health; physical health; quality of life; reported health.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Age of Onset
  • Aged
  • Child
  • Child, Preschool
  • Clinical Decision-Making
  • Common Variable Immunodeficiency / diagnosis
  • Common Variable Immunodeficiency / epidemiology*
  • Common Variable Immunodeficiency / therapy
  • Databases, Factual
  • Disease Management
  • Female
  • Health Surveys
  • Humans
  • Immunoglobulins, Intravenous
  • Infant
  • Infant, Newborn
  • Male
  • Middle Aged
  • Patient Reported Outcome Measures
  • Quality of Life*
  • Severity of Illness Index
  • Sickness Impact Profile*
  • United States / epidemiology
  • Young Adult

Substances

  • Immunoglobulins, Intravenous