ERS/EBMT clinical practice guidelines on treatment of pulmonary chronic graft- versus-host disease in adults
- PMID: 38485149
- DOI: 10.1183/13993003.01727-2023
ERS/EBMT clinical practice guidelines on treatment of pulmonary chronic graft- versus-host disease in adults
Abstract
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 (ERS) 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 and 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 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.
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Conflict of interest statement
Conflict of interest: S. Bos is funded by the Paul Corris International Clinical Research Training Scholarship and reports lecture fees from Mallinckrodt and Jazz, and travel support from Takeda. J. Murray reports lecture fees from Therakos and Sanofi. M. Marchetti reports consultancy fees from Gilead and lecture fees from Novartis. G-S. Cheng reports being site PI of an ongoing multicentre phase II trial for ruxolitinib in BOS after HCT (clinicaltrials.gov NCT03674047). A. Bergeron reports a scientific committee fee (paid to institution) from AstraZeneca, travel grant from Boehringer, adjudication board fee from ENANTA, and a lecture fee (paid to institution) from Novartis, outside the submitted work. D. Wolff received honoraria from Sanofi, Incyte, Mallinckrodt and Novartis and a research grant from Novartis. A. Sharma 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; and 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. A. Sharma has no direct financial interest in these therapies. A. Piekarska reports lecture fees from Novartis, outside the submitted work. K. Raj reports lecture fees from Celgene, Pfizer, Mallinckrodt, Jazz, BMS and Astellas, and travel support from Celgene, BMS and Mallinckrodt. O. Penack received honoraria or travel support from Gilead, Jazz, MSD, Novartis, Pfizer and Therakos, research support from Incyte and Priothera, and is a member of advisory boards to Equillium Bio, Jazz, Gilead, Novartis, MSD, Omeros, Priothera, Sanofi, Shionogi and SOBI. O. Penack 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). T. Tonia acts as a senior ERS methodologist. H. Schoemans reports grants from Novartis and BHS, personal fees from Novartis, Janssen and Sanofi, and travel grants from Gilead, Pfizer, EBMT, CIBMTR and BHS. D. Stolz reports grants from Curetis and AstraZeneca (paid to institution), personal fees from CSL Behring, Berlin-Chemie Menarini, Novartis, GlaxoSmithKline, AstraZeneca, Vifor, Merck, Chiesi, Grifols, MSD, Merck, Sanofi, and Pfizer, and participation on advisory boards for CSL Behring, Berlin-Chemie, Menarini, Novartis, GlaxoSmithKline, AstraZeneca, Vifor, Merck, Chiesi, Grifols, MSD, Merck and Sanofi. R. Vos reports a research grant from Research Foundation-Flanders (FWO), and advisory board fees (paid to institution) from AstraZeneca, GSK, Takeda and Zambon, outside the submitted work. C. Sander, S.M. Badawy, Z. Peric, J. Pidala, S. Kulkarni, M. Beestrum, A. Linke, M. Rutter and C. Coleman have nothing to disclose.
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