Evaluation of sleep, fatigue, and quality of life in patients with primary immunodeficiency.

INTRODUCTION

Primary immunodeficiency (PID) is a heterogeneous group of genetic disorders characterized by recurrent infections and immune dysregulation. Despite advances in therapy, many patients experience impaired quality of life (QoL), fatigue, and sleep disturbances. This study aimed to evaluate sleep quality, fatigue, and health-related QoL in adults with PID and to explore the associations between comorbidities and treatment modalities.

MATERIALS AND

METHODS

This retrospective, observational, single-center study was conducted at the University of Health Sciences, Gülhane Training and Research Hospital between May and August 2024. Forty-nine adult patients with PID, diagnosed according to international criteria, completed validated questionnaires: the Pittsburgh Sleep Quality Index (PSQI), Fatigue Severity Scale (FSS), and SF-36 QoL scale. Clinical and demographic data were retrieved from the medical records. Statistical analyses included t-tests, Mann-Whitney U, chi-square, and Pearson's correlation, with significance set at P < 0.05.

RESULTS

Of 49 patients (55.1% males, mean follow-up 9.9 years), 36.7% had poor sleep quality, 42.9% reported severe fatigue, 49% showed impaired SF-36 physical scores, and 87.8% had preserved mental scores. Poor sleep quality was correlated positively with fatigue (r = 0.623, P < 0.001) and negatively correlated with physical QoL (r = -0.491, P < 0.001). Patients with autoimmune and inflammatory bowel disease had significantly worse PSQI and FSS scores, whereas bronchiectasis was associated with reduced SF-36 physical scores. No differences were observed between the IVIG and SCIG groups in fatigue or mental scores; however, SCIG recipients had significantly higher physical scores (P = 0.018).

CONCLUSION

Patients with PID experience substantial impairment in physical QoL, with fatigue and poor sleep quality frequently coexisting. Comorbidities, such as autoimmunity, bronchiectasis, and inflammatory bowel disease, exacerbate these impairments. SCIG therapy confers better physical outcomes than IVIG therapy. Comprehensive care strategies that address psychosocial and physical health are essential for the management of PID.