Yonsei Med J.  2012 May;53(3):603-610. 10.3349/ymj.2012.53.3.603.

Health Related Quality of Life in Common Variable Immunodeficiency

Affiliations
  • 1Department of Molecular Medicine, Sapienza University of Rome and Fondazione Eleonora Lorillard Spencer Cenci, Rome, Italy.
  • 2Health Services Research Unit, Istituto Dermopatico Dell'Immacolata IDI-IRCCS, Rome, Italy. ste.tab@idi.it
  • 3Department of Clinical Immunology, Sapienza University of Rome, Rome, Italy.

Abstract

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.

Keyword

Common Variable Immunodeficiency; Health Related Quality of Life; SF-36; General Health Questionnaire-12; GHQ-12

MeSH Terms

Chronic Disease
Common Variable Immunodeficiency/*physiopathology
Female
Health Status
Humans
Male
*Quality of Life
Questionnaires

Figure

  • Fig. 1 SF-36 scales, mean values in (A) CVID patients vs. normal subjects - Italian normative. (B) CVID patients vs. normal subjects (25-34 years). (C) CVID patients vs. normal subjects (35-44 years). (D) CVID patients vs. normal subjects (45-54 years). (E) CVID patients vs. normal subjects (55-64 years). Higher scores denote better health SF-36 scales. PF, physical functioning (limitations in performing physical activities such as bathing or dressing); RP, role-physical (limitations with work and other daily activities as a result of physical health); BP, bodily pain (how severe and limiting is pain); GH, general health (how general personal health is evaluated by the patient); VT, vitality (feeling tired and worn out vs. feeling full of energy); SF, social functioning (interference with normal social activities due to physical or emotional problems); RE, role-emotional (limitations with work and other daily activities as a result of emotional problems); MH, mental health (feeling nervous and depressed vs. peaceful, happy and calm). CVID, common variable immunodeficiency; SF-36, Shot From-36. Normal subjects, Italian Normative11). *p<0.05 t-test.


Reference

1. International Union of Immunological Societies. Primary immunodeficiency diseases. Report of an IUIS Scientific Committee. Clin Exp Immunol. 1999; 118(Suppl 1):1–28.
2. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999; 93:190–197. PMID: 10600329.
3. Park MA, Li JT, Hagan JB, Maddox DE, Abraham RS. Common variable immunodeficiency: a new look at an old disease. Lancet. 2008; 372:489–502. PMID: 18692715.
Article
4. Conley ME, Park CL, Douglas SD. Childhood common variable immunodeficiency with autoimmune disease. J Pediatr. 1986; 108:915–922. PMID: 2423668.
Article
5. Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol. 1999; 92:34–48. PMID: 10413651.
Article
6. Kainulainen L, Varpula M, Liippo K, Svedström E, Nikoskelainen J, Ruuskanen O. Pulmonary abnormalities in patients with primary hypogammaglobulinemia. J Allergy Clin Immunol. 1999; 104:1031–1036. PMID: 10550749.
Article
7. Quinti I, Soresina A, Spadaro G, Martino S, Donnanno S, Agostini C, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007; 27:308–316. PMID: 17510807.
Article
8. Ware JE Jr, Sherbourne CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care. 1992; 30:473–483. PMID: 1593914.
9. Howard V, Greene JM, Pahwa S, Winkelstein JA, Boyle JM, Kocak M, et al. The health status and quality of life of adults with X-linked agammaglobulinemia. Clin Immunol. 2006; 118:201–208. PMID: 16377251.
Article
10. Daly PB, Evans JH, Kobayashi RH, Kobayashi AL, Ochs HD, Fischer SH, et al. Home-based immunoglobulin infusion therapy: quality of life and patient health perceptions. Ann Allergy. 1991; 67:504–510. PMID: 1958004.
11. Apolone G, Mosconi P. The Italian SF-36 Health Survey: translation, validation and norming. J Clin Epidemiol. 1998; 51:1025–1036. PMID: 9817120.
12. Goldberg D. The detection of psychiatric illness by questionnaire. 1972. London: Oxford University Press.
13. Picardi A, Abeni D, Pasquini P. Assessing psychological distress in patients with skin diseases: reliability, validity and factor structure of the GHQ-12. J Eur Acad Dermatol Venereol. 2001; 15:410–417. PMID: 11763380.
Article
14. Bagby RM, Parker JD, Taylor GJ. The twenty-item Toronto Alexithymia Scale--I. Item selection and cross-validation of the factor structure. J Psychosom Res. 1994; 38:23–32. PMID: 8126686.
Article
15. Bagby RM, Taylor GJ, Parker JD. The Twenty-item Toronto Alexithymia Scale--II. Convergent, discriminant, and concurrent validity. J Psychosom Res. 1994; 38:33–40. PMID: 8126688.
Article
16. Bressi C, Taylor G, Parker J, Bressi S, Brambilla V, Aguglia E, et al. Cross validation of the factor structure of the 20-item Toronto Alexithymia Scale: an Italian multicenter study. J Psychosom Res. 1996; 41:551–559. PMID: 9032718.
Article
17. Quinti I, Soresina A, Guerra A, Rondelli R, Spadaro G, Agostini C, et al. Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deficiencies: results from a multicenter prospective cohort study. J Clin Immunol. 2011; 31:315–322. PMID: 21365217.
Article
18. Higginson IJ, Carr AJ. Measuring quality of life: using quality of life measures in the clinical setting. BMJ. 2001; 322:1297–1300. PMID: 11375237.
Article
19. Lohr KN. Advances in health status assessment. Overview of the conference. Med Care. 1989; 27(3 Suppl):S1–S11. PMID: 2921881.
20. Lohr KN. Applications of health status assessment measures in clinical practice. Overview of the third conference on advances in health status assessment. Med Care. 1992; 30(5 Suppl):MS1–MS14. PMID: 1583924.
21. Sweinberg SK, Wodell RA, Grodofsky MP, Greene JM, Conley ME. Retrospective analysis of the incidence of pulmonary disease in hypogammaglobulinemia. J Allergy Clin Immunol. 1991; 88:96–104. PMID: 2071789.
Article
22. Nicolay U, Kiessling P, Berger M, Gupta S, Yel L, Roifman CM, et al. Health-related quality of life and treatment satisfaction in North American patients with primary immunedeficiency diseases receiving subcutaneous IgG self-infusions at home. J Clin Immunol. 2006; 26:65–72. PMID: 16418804.
Article
23. Tcheurekdjian H, Palermo T, Hostoffer R. Quality of life in common variable immunodeficiency requiring intravenous immunoglobulin therapy. Ann Allergy Asthma Immunol. 2004; 93:160–165. PMID: 15328676.
Article
24. Gardulf A, Borte M, Ochs HD, Nicolay U. Vivaglobin Clinical Study Group. Prognostic factors for health-related quality of life in adults and children with primary antibody deficiencies receiving SCIG home therapy. Clin Immunol. 2008; 126:81–88. PMID: 17964220.
Article
25. Winkelstein JA, Conley ME, James C, Howard V, Boyle J. Adults with X-linked agammaglobulinemia: impact of disease on daily lives, quality of life, educational and socioeconomic status, knowledge of inheritance, and reproductive attitudes. Medicine (Baltimore). 2008; 87:253–258. PMID: 18794707.
26. Carrasco Garrido P, de Miguel Díez J, Rejas Gutiérrez J, Centeno AM, Gobartt Vázquez E, Gil de Miguel A, et al. Negative impact of chronic obstructive pulmonary disease on the health-related quality of life of patients. Results of the EPIDEPOC study. Health Qual Life Outcomes. 2006; 4:31. PMID: 16719899.
Article
27. Bridevaux PO, Gerbase MW, Probst-Hensch NM, Schindler C, Gaspoz JM, Rochat T. Long-term decline in lung function, utilisation of care and quality of life in modified GOLD stage 1 COPD. Thorax. 2008; 63:768–774. PMID: 18505800.
Article
28. Voll-Aanerud M, Eagan TM, Wentzel-Larsen T, Gulsvik A, Bakke PS. Respiratory symptoms, COPD severity, and health related quality of life in a general population sample. Respir Med. 2008; 102:399–406. PMID: 18061422.
Article
29. Reilly MC, Gerlier L, Brabant Y, Brown M. Validity, reliability, and responsiveness of the work productivity and activity impairment questionnaire in Crohn's disease. Clin Ther. 2008; 30:393–404. PMID: 18343277.
Article
30. Gilbody SM, House AO, Sheldon TA. Routinely administered questionnaires for depression and anxiety: systematic review. BMJ. 2001; 322:406–409. PMID: 11179161.
Article
31. van Hemert AM, Hengeveld MW, Bolk JH, Rooijmans HG, Vandenbroucke JP. Psychiatric disorders in relation to medical illness among patients of a general medical out-patient clinic. Psychol Med. 1993; 23:167–173. PMID: 8475204.
Article
Full Text Links
  • YMJ
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr