Pulmonary features and stage of disease in adult patients with hyper-IgE syndrome: a single-centre clinical study and literature review
The progression of pulmonary disease in HIES patients underscores a critical three-step process: initial recurrent infections, development of structural lung damage, and subsequent reinfections that aggravate the damage. This rapid transition from infection to structural damage, especially in patients with STAT3 mutations, highlights the importance of early and aggressive intervention. Managing reinfections after structural lung damage is essential to prevent further deterioration and to improve long-term outcomes.
Authors
- Xuejun Zeng (ORCID: https://orcid.org/0000-0002-6426-7586)
- Juan Wu (ORCID: https://orcid.org/0000-0002-2660-0587)
- Tiange Xie (ORCID: https://orcid.org/0000-0002-7086-7385)
- Yingdong Han (ORCID: https://orcid.org/0000-0002-3997-8044)
- Hong J. Di (ORCID: https://orcid.org/0000-0002-6966-0299)
- Yun Zhang (ORCID: https://orcid.org/0000-0002-6373-0647)
- Na Xu (ORCID: https://orcid.org/0000-0002-2464-5642)
- He Zhao (ORCID: https://orcid.org/0000-0002-3988-2851)
- Ting Zhang (ORCID: https://orcid.org/0000-0002-1899-1784)
- Min Peng (ORCID: https://orcid.org/0000-0001-5189-0013)
- Hongwei Fan
Institutions
- Chinese Academy of Medical Sciences & Peking Union Medical College (CN)
- Peking Union Medical College Hospital (CN)
Publication Details
- Journal
- Orphanet Journal of Rare Diseases
- Published
- 2025-06-03
- DOI
- https://doi.org/10.1186/s13023-025-03749-6
- Citations
- 2
- Primary Topic
- Immunodeficiency and Autoimmune Disorders
- Type
- article
- Field-Weighted Citation Impact
- 1.59
Funders
- National Natural Science Foundation of China
- Peking Union Medical College Hospital
- Peking Union Medical College
- High-level Hospital Construction Project of Guangdong Provincial People's Hospital