Treatment strategies for obliterative bronchiolitis associated with connective tissue diseases: a scoping review
Year: 2026
Type: review
Abstract: Obliterative bronchiolitis associated with connective tissue diseases (OB-CTD) is a severe and progressive pulmonary condition. Characterised by inflammation and fibrosis of the small airways, it leads to significant morbidity. Despite its severity, OB-CTD often remains underdiagnosed and the lack of standardised, evidence-based treatment guidelines poses a substantial clinical challenge. This scoping review consolidates current knowledge of the treatment of OB-CTD. The review aims to raise awareness and stimulate targeted research to improve patient outcomes. Current therapeutic regimens consist of empirical combinations of corticosteroids and various immunomodulatory agents, but their efficacy is inconsistent and supported by limited robust evidence. While supportive care, pulmonary rehabilitation and ultimately lung transplantation are key components of advanced disease management, this review highlights the critical need for effective, validated therapies. Emerging treatments, including antifibrotics, biologic therapies (targeting B-cells or tumour necrosis factor-α), Janus kinase inhibitors and phosphodiesterase-4B inhibitors, show promise but require rigorous investigation in the OB-CTD cohort. To advance the management of this challenging disease, future research must prioritise well-designed, large-scale studies on early diagnostic markers, distinct patient phenotypes, and novel therapeutic interventions.
Source: European Respiratory Review
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W7225266320