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.
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 emphysemapatients 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.
Authors: Ilaria Righi; Valentina Vaira; Letizia Corinna Morlacchi; Giorgio Alberto Croci; Valeria Rossetti; Francesco Blasi; Stefano Ferrero; Mario Nosotti; Lorenzo Rosso; Mario Clerici Journal: Front Immunol Date: 2021-08-13 Impact factor: 7.561
Authors: Allan R Glanville; Christian Benden; Anne Bergeron; Guang-Shing Cheng; Jens Gottlieb; Erika D Lease; Michael Perch; Jamie L Todd; Kirsten M Williams; Geert M Verleden Journal: ERJ Open Res Date: 2022-07-25
Authors: Laura Peräkylä; Antti Nykänen; Anneli Piilonen; Risto Kesävuori; Maija Halme; Peter Raivio Journal: PLoS One Date: 2022-10-12 Impact factor: 3.752
Authors: Jens Gottlieb; Geert M Verleden; Michael Perchl; Christina Valtin; Alexander Vallee; Olivier Brugière; Carlos Bravo Journal: PLoS One Date: 2021-12-23 Impact factor: 3.240