Health-related quality of life, social and psychological well-being of 109 adult patients with genetic lipodystrophy
Résumé
Introduction
Lipodystrophy syndromes are rare diseases characterized by a generalized or partial lipoatrophic morphotype and metabolic complications. Data on health-related quality of life and impact of genetic lipodystrophy on social or psychological well-being are lacking.
Patients and Methods
Patients with genetic lipodystrophy were recruited throughout the French national reference network for rare diseases of insulin secretion and insulin sensitivity. Patients completed a self-reported questionnaire exploring their physical, psychological and social well-being and perceived impact of the disease. Descriptive analyses and comparison with general population norms were conducted.
Results
Out of 175 eligible patients, 109 (84% of women) were included, either with familial partial (n=93) or congenital generalized (n=16) lipodystrophy. Health-related quality of life based on physical and mental scores was significantly decreased as compared to the French general population of similar age and gender (p<0.001 for both). Forty-one percent of patients reported moderate or severe depression and 69% dealt with chronic pain. Half of respondents had taken tranquilizers, sleeping pills or antidepressants over their life. Female participants with genetic lipodystrophy were more frequently unemployed due to health issues as compared to the general population. Social discrimination was highly prevalent (73%), coming, in 34% of cases, from health professionals. More than half of affected women reported a very negative impact of lipodystrophy on body image, significantly associated with depressive symptoms.
Conclusion
This study highlights the need of psycho-social support in patients with lipodystrophy. An integrated approach and evaluation of psychological and physical symptoms by physicians should be made available to organize specialized care and set up specific therapeutic educational programs.