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Peer-reviewedCC BY-NC 4.0European Respiratory Review

Treatment strategies for obliterative bronchiolitis associated with connective tissue diseases: a scoping review

  • Rangdol Tenzin Rangdol1,2
  • Ewa Mia˛dlikowska3,4,2
  • Arno Vanstapel5,6
  • Catharina Belge7,8
  • Ellen De Langhe9
  • Laurens De Sadeleer7,8
  • Nico De Crem7,8
  • Wojciech J. Piotrowski3
  • Wim A. Wuyts7,8
9 affiliations
  1. 1Master of Medicine, KU Leuven, Leuven, Belgium
  2. 2Both authors contributed equally and share first authorship
  3. 3Department of Pneumology, Medical University of Lodz, Lodz, Poland
  4. 4Department of Biostatistics and Translational Medicine, Medical University of Lodz, Lodz, Poland
  5. 5Department of Translational Cell and Tissue Research, KU Leuven, Leuven, Belgium
  6. 6Department of Pathology, University Hospitals Leuven, Leuven, Belgium
  7. 7Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), KU Leuven, Leuven, Belgium
  8. 8Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium
  9. 9Department of Rheumatology, University Hospitals Leuven, Leuven, Belgium
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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.

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Cite

BibTeXRIS
BibTeX
@article{rangdol2026treatment,
  title = {{Treatment strategies for obliterative bronchiolitis associated with connective tissue diseases: a scoping review}},
  author = {Rangdol Tenzin Rangdol and Ewa Mia˛dlikowska and Arno Vanstapel and Catharina Belge and Ellen De Langhe and Laurens De Sadeleer and Nico De Crem and Wojciech J. Piotrowski and Wim A. Wuyts},
  journal = {European Respiratory Review},
  year = {2026},
  month = nov,
  doi = {10.1183/16000617.0073-2026},
  url = {https://doi.org/10.1183/16000617.0073-2026},
  note = {CC BY-NC 4.0},
}
RIS
TY  - JOUR
TI  - Treatment strategies for obliterative bronchiolitis associated with connective tissue diseases: a scoping review
AU  - Rangdol Tenzin Rangdol
AU  - Ewa Mia˛dlikowska
AU  - Arno Vanstapel
AU  - Catharina Belge
AU  - Ellen De Langhe
AU  - Laurens De Sadeleer
AU  - Nico De Crem
AU  - Wojciech J. Piotrowski
AU  - Wim A. Wuyts
PY  - 2026
DA  - 2026/11/04
JO  - European Respiratory Review
DO  - 10.1183/16000617.0073-2026
UR  - https://doi.org/10.1183/16000617.0073-2026
AB  - 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.
SN  - 0905-9180
SN  - 1600-0617
ER  -