PT - JOURNAL ARTICLE AU - Bos, Saskia AU - Murray, John AU - Marchetti, Monia AU - Cheng, Guang-Shing AU - Bergeron, Anne AU - Wolff, Daniel AU - Sander, Clare AU - Sharma, Akshay AU - Badawy, Sherif M. AU - Peric, Zinaida AU - Piekarska, Agnieszka AU - Pidala, Joseph AU - Raj, Kavita AU - Penack, Olaf AU - Kulkarni, Samar AU - Beestrum, Molly AU - Linke, Andrea AU - Rutter, Matthew AU - Coleman, Courtney AU - Tonia, Thomy AU - Schoemans, Hélène AU - Stolz, Daiana AU - Vos, Robin TI - ERS/EBMT clinical practice guideline on treatment of pulmonary chronic graft-<em>versus</em>-host disease in adults AID - 10.1183/13993003.01727-2023 DP - 2024 Jan 01 TA - European Respiratory Journal PG - 2301727 4099 - https://publications.ersnet.org//content/early/2024/02/08/13993003.01727-2023.short 4100 - https://publications.ersnet.org//content/early/2024/02/08/13993003.01727-2023.full AB - Chronic graft-versus-host disease (cGvHD) is a common complication after allogeneic haematopoietic stem cell transplantation, characterised by a broad disease spectrum that can affect virtually any organ. Although pulmonary cGvHD is a less common manifestation, it is of great concern due to its severity and poor prognosis. Optimal management of patients with pulmonary cGvHD is complicated and no standardised approach is available.The purpose of this joint European Respiratory Society and European Society for Blood and Marrow Transplantation Task Force was to develop evidence-based recommendations regarding the treatment of pulmonary cGvHD phenotype bronchiolitis obliterans syndrome in adults. A multidisciplinary group representing specialists in haematology, respiratory medicine, methodology as well as patient advocates formulated eight PICO (Patient, Intervention, Comparison, Outcome) and two narrative questions. Following the ERS standardised methodology, we conducted systematic reviews to address these questions and used the GRADE (Grading of Recommendations Assessment, Development and Evaluation) approach to develop recommendations.The resulting guideline addresses common therapeutic options (inhalation therapy, fluticasone-azithromycin-montelukast, imatinib, ibrutinib, ruxolitinib, belumosudil, extracorporeal photopheresis and lung transplantation), as well as other aspects of general management, such as lung functional and radiological follow-up and pulmonary rehabilitation, for adults with pulmonary cGvHD phenotype bronchiolitis obliterans syndrome. These recommendations include important advancements that could be incorporated in the management of adults with pulmonary cGvHD, primarily aimed at improving and standardising treatment and improving outcomes.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of Interest: Jonathan Grigg reports lecture honoraria from GSK; outside the submitted work.Conflict of interest: SB is funded by the Paul Corris International Clinical Research Training Scholarship and reports lecture fees from Mallinckrodt and Jazz, travel support from Takeda. JM reports lecture fees from Therakos and Sanofi. MM reports consultancy fees from Gilead and lecture fees from Novartis. GSC reports being site PI of an ongoing multicentre phase II trial for ruxolitinib in BOS after HCT (clinicaltrials.gov NCT03674047). AB reports a scientific committee fee to the institution from Astra Zeneca, travel grant from Boehringer, adjudication board fee from ENANTA, lecture fee to the institution from Novartis, outside the submitted work. DW received honoraria from Sanofi, Incyte, Mallinckrodt and Novartis and a research grant from Novartis. AS reports grants from CRISPR Therapeutics, personal fees from Vertex Pharmaceuticals, Editas Medicine, Sangamo Therapeutics, Spotlight Therapeutics, Medexus Inc. and RCI BMT / NMDP, outside the submitted work; AS is the St. Jude Children's Research Hospital site principal investigator of clinical trials for genome editing of sickle cell disease sponsored by Vertex Pharmaceuticals/CRISPR Therapeutics (NCT03745287), Novartis Pharmaceuticals (NCT04443907) and Beam Therapeutics (NCT05456880). The industry sponsors provide funding for the clinical trial, which includes salary support paid to the institution. AS has no direct financial interest in these therapies. AP reports lecture fees from Novartis, outside the submitted work. KR reports lecture fees from Celgene, Pfizer, Mallinckrodt, Jazz, BMS and Astellas; travel support from Celgene, BMS and Mallinckrodt. OP received honoraria or travel support from Gilead, Jazz, MSD, Novartis, Pfizer and Therakos. He has received research support from Incyte and Priothera. He is a member of advisory boards to Equillium Bio, Jazz, Gilead, Novartis, MSD, Omeros, Priothera, Sanofi, Shionogi and SOBI. OP acknowledges the support of José Carreras Leukämie-Stiftung (3R/2019, 23R/2021), Deutsche Krebshilfe (70113519), Deutsche Forschungsgemeinschaft (PE 1450/7-1, PE 1450/9-1, PE 1450/10-1) and Stiftung Charité BIH (BIH_PRO_549, Focus Group Vascular Biomedicine). TT acts as a senior ERS methodologist. HS reports grants from Novartis and BHS, personal fees from Novartis, Janssen and Sanofi, travel grants from Gilead, Pfizer, EBMT, CIBMTR and BHS. DS reports grants from Curetis and AstraZeneca to the institution, personal fees from CSL Behring, Berlin-Chemie Menarini, Novartis, GlaxoSmithKline, AstraZeneca, Vifor, Merck, Chiesi, Grifols, MSD, Merck, Sanofi, and Pfizer; participation on advisory boards for CSL Behring, Berlin-Chemie Menarini, Novartis, GlaxoSmithKline, AstraZeneca, Vifor, Merck, Chiesi, Grifols, MSD, Merck and Sanofi. RV reports a research grant from Research Foundation-Flanders (FWO), advisory board fees to the institution from AstraZeneca, GSK, Takeda and Zambon, outside the submitted work. CS, SMB, ZP, JP, SK, MB, AL, MR and CC have nothing to disclose.