Featured articlesProgression pattern of restrictive allograft syndrome after lung transplantation
Section snippets
Definition of CLAD and RAS
CLAD and RAS were defined as described previously.1 In brief, baseline forced expiratory volume in 1 second (FEV1) was defined according to criteria recommended by the International Society for Heart and Lung Transplantation,4 and then the baseline values of other PFT parameters were taken as the average of the parameters measured at the time of the best FEV1 measurements. CLAD was defined as an irreversible drop of FEV1 to <80% of baseline. RAS was defined as a condition in which restrictive
Results
Patient demographics of the 25 RAS cases examined are shown in Table 1. Among them, 3 patients initially showed BOS phenotype and then developed RAS. Although the number of patients with cystic fibrosis was relatively large in this study, the original diagnosis was not significantly different among BOS, RAS and non-CLAD patients in our previous study using a larger cohort.1
In all RAS cases analyzed, patients had at least 1 episode of acute exacerbation. None of the patients showed a steady
Case 1
A 62-year-old man received cadaveric bilateral lung transplantation for chronic obstructive pulmonary disease (COPD) (Figure 4). Although his early post-transplant course was uneventful, he had 3 episodes of acute exacerbation. After the first exacerbation, PFT showed a decline over time, whereas, after the second episode, PFT showed partial recovery. On CT scan, there were diffuse bilateral GGO and consolidation at the time of exacerbation. GGO resolved to some extent during intervals between
Case 2
A 28-year-old man with cystic fibrosis received cadaveric bilateral lung transplantation (Figure 5). His post-transplant recovery was excellent except for complications of supraventricular tachycardia and intracardiac thrombus formation. His chest remained clear until he had an initial episode of acute exacerbation, accompanied by extensive bilateral GGO, bilateral pneumothoraces and pneumomediastinum (Figure 5C). Although PFT indicated temporary recovery and the radiographic abnormalities
Case 3
A 52-year-old man received bilateral lung transplantation for idiopathic pulmonary fibrosis (Figure 6). His clinical course was excellent until the first episode of acute exacerbation, approximately 1 year after transplantation. His general condition, PFTs and CT scans showed gradual improvement over the next 16 months, when he had a second episode of acute exacerbation. He died 868 days after transplantation due to respiratory failure.
Discussion
In this study we have documented the progression pattern of RAS. In general, patients suffer multiple episodes of acute exacerbation (a “stair-step” progression pattern) characterized by GGO, with or without consolidation and interstitial shadow on CT scan, and histologic features of DAD on biopsies. Although some episodes of acute exacerbation were associated with acute rejection or infection, there was no uniform explanation for acute exacerbation. After acute exacerbations, patients
Disclosure statement
The authors have no conflicts of interest to disclose.
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2020, Transplantation ReviewsCitation Excerpt :RAS is characterized by inflammation and fibrosis in the alveoli, pleura, and interlobular septum of the lung parenchyma [9]. In addition to the above symptoms, RAS was reported to present with minimal coarse crackles, pleurisy and chest tightness in other studies [16,17,20]. Even when treated routinely, RAS eventually results in respiratory failure due to irreversible deterioration of pulmonary function [21].
Use of CT-SCAN score and volume measures to early identify restrictive allograft syndrome in single lung transplant recipients
2020, Journal of Heart and Lung TransplantationCitation Excerpt :BOS was defined by the classical International Society for Heart and Lung Transplantation definition.10,15 Spirometry was performed according to American Thoractic Society guidelines2 (refer to Supplementary Material available online at www.jhltonline.org). Only 5 out of 17 (28%) patients with RAS had performed TLC measures during follow-up within the period of RAS development, with only very few available measures in 3 of them, which did not allow us to use this criteria for the diagnosis of RAS.