Pulmonary Rehabilitation in Inborn Errors of Immunity with Bronchiectasis: A Narrative Review with Implications for High-Consanguinity Settings
5 affiliations
- 1Department of Health and Rehabilitation Sciences, College of Applied Medical Sciences, Prince Sattam bin Abdulaziz University, Al-Kharj 11942, Saudi Arabia
- 2Department of Medical Laboratories, College of Applied Medical Sciences, Shaqra University, Shaqra 11961, Saudi Arabia
- 3Department of Respiratory Therapy, College of Applied Medical Sciences, King Saud bin Abdulaziz University for Health Sciences, Riyadh 14611, Saudi Arabia
- 4King Abdullah International Medical Research Center, Riyadh 11481, Saudi Arabia
- 5Department of Respiratory Services, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh 11426, Saudi Arabia
Abstract
Bronchiectasis is the most frequent chronic structural lung complication of inborn errors of immunity (IEI) and a major driver of morbidity and mortality, even in patients receiving immunoglobulin replacement. Pulmonary rehabilitation (PR), comprising airway clearance, physical activity training and self-management education, is recommended for bronchiectasis in international and national guidelines, yet its evidence base was generated largely in populations in which immunodeficiency was neither characterised nor analysed separately. This narrative review, written from the combined perspectives of clinical immunology and cardiopulmonary rehabilitation, synthesises evidence on rehabilitation in IEI-associated bronchiectasis and considers its relevance to populations with high rates of consanguinity, such as those of the Middle East and North Africa, where autosomal recessive and combined IEIs are over-represented; Saudi Arabia is used as an illustrative example. Evidence specific to IEI is sparse. A single underpowered pilot randomised study (n = 34) of home-based moderate-intensity physical activity in primary immunodeficiency found no between-group difference in infection rates, while observational studies consistently document impaired functional capacity, prominent fatigue, reduced health-related quality of life, and heightened airway and systemic inflammation. Trials of PR in bronchiectasis show short-term gains in functional capacity and quality of life, but none of the trials we examined reported outcomes by aetiology, and no paediatric trials exist. No study has evaluated PR in the IEI categories that predominate in high-consanguinity regions, such as chronic granulomatous disease and the hyper-IgE syndromes, although cohorts from these regions report pulmonary complications as a leading cause of death. Because PR is individually tailored and has a favourable safety profile, its core benefits, including gains in muscle function and cardiorespiratory fitness, are likely to apply regardless of aetiology; the main uncertainties concern how to deliver it safely and how to evaluate it, given that withholding PR from control groups may be unethical. We outline the immunological rationale for rehabilitation in IEI, propose an integrated immunology–rehabilitation care model with personalised delivery, including home-based and tele-rehabilitation to limit cross-infection, and set out a prioritised research agenda that includes pragmatic trial designs, IEI-stratified analyses and immunological endpoints.
Related
- Pulmonary Resection for Bronchiectasis: Perioperative and Follow-Up Outcomes in a VATS-Dominant Cohort
- A Rare Case of Cystic Bronchiectasis With Concurrent Pulmonary Aspergillosis in a Rheumatoid Arthritis Patient Receiving Etanercept.
- Sarcopenia and cognitive impairment after hip fracture in older adults: a narrative review and conceptual rehabilitation framework
- Blood Flow Restriction Training in Stroke Rehabilitation: A Narrative Review of Current Evidence and Future Directions
- Implementation of i‑PARIHS-guided pulmonary rehabilitation in patients receiving oxygen by high‑flow nasal cannula
BibTeX
@article{alruwaili2026pulmonary,
title = {{Pulmonary Rehabilitation in Inborn Errors of Immunity with Bronchiectasis: A Narrative Review with Implications for High-Consanguinity Settings}},
author = {Rawan Alruwaili and Abdullah Albelasi and Mohammed M. Alqahtani},
journal = {Journal of Clinical Medicine},
year = {2026},
month = oct,
doi = {10.3390/jcm15208131},
url = {https://www.mdpi.com/2077-0383/15/20/8131},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Pulmonary Rehabilitation in Inborn Errors of Immunity with Bronchiectasis: A Narrative Review with Implications for High-Consanguinity Settings
AU - Rawan Alruwaili
AU - Abdullah Albelasi
AU - Mohammed M. Alqahtani
PY - 2026
DA - 2026/10/01
JO - Journal of Clinical Medicine
DO - 10.3390/jcm15208131
UR - https://www.mdpi.com/2077-0383/15/20/8131
AB - Bronchiectasis is the most frequent chronic structural lung complication of inborn errors of immunity (IEI) and a major driver of morbidity and mortality, even in patients receiving immunoglobulin replacement. Pulmonary rehabilitation (PR), comprising airway clearance, physical activity training and self-management education, is recommended for bronchiectasis in international and national guidelines, yet its evidence base was generated largely in populations in which immunodeficiency was neither characterised nor analysed separately. This narrative review, written from the combined perspectives of clinical immunology and cardiopulmonary rehabilitation, synthesises evidence on rehabilitation in IEI-associated bronchiectasis and considers its relevance to populations with high rates of consanguinity, such as those of the Middle East and North Africa, where autosomal recessive and combined IEIs are over-represented; Saudi Arabia is used as an illustrative example. Evidence specific to IEI is sparse. A single underpowered pilot randomised study (n = 34) of home-based moderate-intensity physical activity in primary immunodeficiency found no between-group difference in infection rates, while observational studies consistently document impaired functional capacity, prominent fatigue, reduced health-related quality of life, and heightened airway and systemic inflammation. Trials of PR in bronchiectasis show short-term gains in functional capacity and quality of life, but none of the trials we examined reported outcomes by aetiology, and no paediatric trials exist. No study has evaluated PR in the IEI categories that predominate in high-consanguinity regions, such as chronic granulomatous disease and the hyper-IgE syndromes, although cohorts from these regions report pulmonary complications as a leading cause of death. Because PR is individually tailored and has a favourable safety profile, its core benefits, including gains in muscle function and cardiorespiratory fitness, are likely to apply regardless of aetiology; the main uncertainties concern how to deliver it safely and how to evaluate it, given that withholding PR from control groups may be unethical. We outline the immunological rationale for rehabilitation in IEI, propose an integrated immunology–rehabilitation care model with personalised delivery, including home-based and tele-rehabilitation to limit cross-infection, and set out a prioritised research agenda that includes pragmatic trial designs, IEI-stratified analyses and immunological endpoints.
SN - 2077-0383
ER -