Rationale: Patients can change chronic lung allograft dysfunction (CLAD) phenotype, especially from BOS to mixed phenotype. Our aim was to further characterize these patients. Method: Mixed CLAD was defined as a restrictive physiology with persistent CT opacities, after initial bronchiolitis obliterans syndrome (BOS) diagnosis. The incidence, prognosis, pulmonary function, radiology, pathology, and airway inflammation were compared between patients with restrictive allograft syndrome (RAS) and mixed CLAD. Result: A total of 268 (44%) patients developed CLAD of which 47 (18%) were diagnosed with RAS “ab initio,” 215 (80%) with BOS, and 6 (2%) an undefined phenotype. Twenty-five patients developed a mixed CLAD phenotype (24 BOS to mixed and 1 RAS to mixed). Survival after mixed phenotype diagnosis was comparable (P =.39) to RAS. More emphysema patients developed a mixed phenotype (P =.020) compared to RAS ab initio, while mixed CLAD patients had a lower FEV1 (P <.0001) and FEV1/FVC (P =.0002) at diagnosis compared to RAS ab initio. CT scans in patients with the mixed phenotype demonstrated apical predominance of the opacities (P =.0034) with pleuroparenchymal fibroelastosis on histopathology. Conclusion: We further characterized patients with a mixed phenotype of CLAD. Although the survival after diagnosis was comparable to RAS ab initio patients, there was a difference in demography, pulmonary function, radiology, and pathology.

Additional Metadata
Keywords bronchiolitis obliterans syndrome, chronic lung allograft dysfunction, lung transplantation, mixed CLAD, phenotypes, restrictive allograft syndrome
Persistent URL dx.doi.org/10.1111/ctr.13781, hdl.handle.net/1765/124303
Journal Clinical Transplantation
Citation
Verleden, S.E, von der Thusen, J.H, Van Herck, A. (Anke), Weynand, B. (Birgit), Verbeken, E.K, Verschakelen, J, … Ceulemans, L.J. (Laurens J). (2020). Identification and characterization of chronic lung allograft dysfunction patients with mixed phenotype: A single-center study. Clinical Transplantation. doi:10.1111/ctr.13781