Which Shall I Choose? Lung Transplantation Listing Preference for Individuals with Interstitial Lung Disease and Chronic Obstructive Pulmonary Disease.
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ABSTRACT:
SUBMITTER: Ramos KJ
PROVIDER: S-EPMC6376947 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Although chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) have distinct clinical features, both diseases may coexist in a patient because they share similar risk factors such as smoking, male sex, and old age. Patients with both emphysema in upper lung fields and diffuse ILD are diagnosed with combined pulmonary fibrosis and emphysema (CPFE), which causes substantial clinical deterioration. Patients with CPFE have higher mortality compared with patients who have COPD alone, but results have been inconclusive compared with patients who have idiopathic pulmonary fibrosis (IPF). Poor prognostic factors for CPFE include exacerbation, lung cancer, and pulmonary hypertension. The presence of interstitial lung abnormalities, which may be an early or mild form of ILD, is notable among patients with COPD, and is associated with poor prognosis. Various theories have been proposed regarding the pathophysiology of CPFE. Biomarker analyses have implied that this pathophysiology may be more closely associated with IPF development, rather than COPD or emphysema. Patients with CPFE should be advised to quit smoking and undergo routine lung function tests, and pulmonary rehabilitation may be helpful. Various pharmacologic agents and surgical approaches may be beneficial in patients with CPFE, but further studies are needed.
| S-EPMC8987660 | biostudies-literature
Project description:RationaleBilateral lung transplantation is widely used to treat chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD), on the basis of an expectation of improved survival after transplantation. Yet, waiting list mortality is higher while awaiting bilateral transplantation. The net effect of procedure preference on overall survival is unknown.ObjectivesTo determine whether an unrestricted procedure preference is associated with improved overall outcomes after listing for lung transplantation.MethodsWe performed a retrospective cohort study of 12,155 adults with COPD or ILD listed for lung transplantation in the United States between May 4, 2005, and December 31, 2014. We defined a "restricted" procedure preference as listing for "bilateral transplantation only" and an "unrestricted" procedure preference as listing for any combination of bilateral or single lung transplantation. We used a composite "intention-to-treat" primary outcome that included events both before and after transplantation, defined as the number of days between listing and death, removal from the list for clinical deterioration, or retransplantation.ResultsIn adjusted analyses, an unrestricted procedure preference was associated with a 3% lower rate of the primary intention-to-treat outcome in COPD (adjusted hazard ratio [aHR], 0.97; 95% confidence interval [CI], 0.89-1.07) and a 1% higher rate in ILD (aHR, 1.01; 95% CI, 0.94-1.08). There was no convincing evidence that these associations varied by age, disease severity, or the use of mechanical support. Among those with ILD and concomitant severe pulmonary hypertension, an unrestricted preference was associated with a 17% increased rate of the primary outcome (aHR, 1.17; 95% CI, 0.99-1.39). An unrestricted preference was consistently associated with lower rates of death or removal from the list for clinical deterioration and with higher rates of transplantation. Graft failure rates were similar among those listed with restricted and unrestricted preferences.ConclusionWhen considering outcomes both before and after transplantation, we found no evidence that patients with COPD or ILD benefit from listing for bilateral lung transplantation compared with listing for a more liberal procedure preference. An unrestricted listing strategy for suitable candidates may increase the number of transplants performed without impacting overall survival.
| S-EPMC6376945 | biostudies-literature
Project description:BackgroundAlthough single and double lung transplantation outcomes for chronic obstructive pulmonary disease (COPD) have been investigated, right and left single lung transplants have never been rigorously compared to evaluate disease-specific differences. Single lung transplants for COPD often have hyperinflation of the contralateral native lung, which may be more pronounced in left lung transplants.MethodsUsing the United Network for Organ Sharing registry, we conducted a retrospective cohort study of 5,585 adults who underwent lung transplantation for COPD from May 4, 2005 to June 30, 2017. Subjects were followed until March 2019. Post-transplant survival was compared using Cox proportional hazards and Royston and Parmar's flexible parametric survival models. We adjusted for donor and recipient factors with known or plausible associations with survival.ResultsLung transplant recipients who received a left single lung transplant for COPD had an increased risk of post-transplant death when compared with those who received a right single lung transplant for COPD (hazard ratio [HR]: 1.24, 95% CI: 1.08-1.48, p = 0.002). Survival did not differ significantly between double lung transplant and right single lung transplant recipients (HR: 0.88, 95% CI: 0.77-1.02, p = 0.086). Adjusted 5-year survival was 57.8% (95% CI: 55.7-60.1) for double lung recipients, 56.7% (95% CI: 55.4-58.0) for right single lung recipients, and 50.9% (95% CI: 47.2-55.0) for left single lung recipients.ConclusionsIn COPD, right single lung transplantation was associated with improved post-transplant survival compared with left single lung transplantation, and no significant difference in post-transplant survival compared with double lung transplantation was found. In light of the ongoing donor lung shortage, preferential allocation of right single lungs to patients with COPD should be considered.
| S-EPMC7863775 | biostudies-literature
Project description:COPD (chronic obstructive pulmonary disease) and ILD (interstitial lung disease) are two common respiratory diseases. They share similar clinical traits but require different therapeutic treatments. Identifying the biomarkers that are differentially expressed between them will not only help the diagnosis of COPD and ILD, but also provide candidate drug targets that may facilitate the development of new treatment for COPD and ILD. Due to the irreversible complex pathological changes of COPD, there are very limited therapeutic options for COPD patients. In this study, we analyzed the gene expression profiles of two datasets: one training dataset that includes 144 COPD patients and 194 ILD patients, and one test dataset that includes 75 COPD patients and 61 ILD patients. Advanced feature selection methods, mRMR (minimal Redundancy Maximal Relevance) and incremental feature selection (IFS), were applied to identify the 38-gene biomarker. An SVM (support vector machine) classifier was built based on the 38-gene biomarker. Its accuracy, sensitivity, and specificity on training dataset evaluated by leave one out cross-validation were 0.905, 0.896, and 0.912, respectively. And on independent test dataset, the accuracy, sensitivity, and specificity on were as great as and were 0.904, 0.933, and 0.869, respectively. The biological function analysis of the 38 genes indicated that many of them can be potential treatment targets that may benefit COPD and ILD patients.
| S-EPMC6879656 | biostudies-literature
Project description:In patients with interstitial lung disease (ILD) complicating classical or amyopathic idiopathic inflammatory myopathy (IIM), lung transplantation outcomes might be affected by the disease and treatments. Here, our objective was to assess survival and prognostic factors in lung transplant recipients with IIM-ILD. We retrospectively reviewed data for 64 patients who underwent lung transplantation between 2009 and 2021 at 19 European centers. Patient survival was the primary outcome. At transplantation, the median age was 53 [46-59] years, 35 (55%) patients were male, 31 (48%) had classical IIM, 25 (39%) had rapidly progressive ILD, and 21 (33%) were in a high-priority transplant allocation program. Survival rates after 1, 3, and 5 years were 78%, 73%, and 70%, respectively. During follow-up (median, 33 [7-63] months), 23% of patients developed chronic lung allograft dysfunction. Compared to amyopathic IIM, classical IIM was characterized by longer disease duration, higher-intensity immunosuppression before transplantation, and significantly worse posttransplantation survival. Five (8%) patients had a clinical IIM relapse, with mild manifestations. No patient experienced ILD recurrence in the allograft. Posttransplantation survival in IIM-ILD was similar to that in international all-cause-transplantation registries. The main factor associated with worse survival was a history of muscle involvement (classical IIM). In lung transplant recipients with idiopathic inflammatory myopathy, survival was similar to that in all-cause transplantation and was worse in patients with muscle involvement compared to those with the amyopathic disease.
| S-EPMC10086953 | biostudies-literature
Project description:Adult lung resident stem/progenitor cells, including P63+ progenitor cells, have demonstrated the capacity for regeneration of lung epithelium in precinical models. Here we report a clinical trial of intra-pulmonary P63+ progenitor cell transplantation which included 28 participants with chronic obstructive pulmonary disease (COPD) (stage II~IV). For control group, standard of care (SOC) for COPD treatment was given. For intervention group, in addition to SOC, autologous P63+ progenitor cells were isolated from the airway basal layer via bronchoscopic brushing, cultured for 3~5 weeks, and then transplanted into the lungs via bronchoscopy with 0.7-5.2x10^6 cells per kilogram bodyweight. Eventually 20 patients were evaluated (intervention group n=17; control group n=3). No grade 3~5 adverse events (AE) or serious adverse events occurred in any group. Bronchoscopy-associated AEs were recorded only in intervention groups, while the other AEs were recorded in both groups without showing significant difference. Participants in intervention group displayed significant improvement in diffusing capacity of the lung for carbon monoxide (DLCO) (change from baseline, 24 weeks: +18.2%), showing significant difference comparing to control group (change from baseline, 24 weeks: -17.4%)(p-value=0.008). Furthermore, more participants in intervention group showed > 30 m increase in walking distance within 6 minutes, demonstrating significant difference comparing to control group (p-value=0.036). Transcriptomic analysis of cells isolated from responding and non-responding individuals in the intervention group showed that higher expression of P63 and Nkx homeobox-1 was associated with better efficacy. In conclusion, transplantation of cultured P63+ lung progenitor cells was safe and represented a potential treatment strategy for COPD.
2024-01-20 | GSE236438 | GEO
Project description:BackgroundIn patients with fibrosing interstitial lung disease (fILD), gastroesophageal reflux (GER) is highly prevalent, perhaps because of the effects of lung fibrosis on altering intrathoracic pressure, diaphragm morphology and lower esophageal sphincter (LES) function. For unclear reasons, obstructive sleep apnea (OSA) is also highly prevalent among patients with fILD. We conducted this study to test our hypothesis that, in patients with fILD, OSA would exacerbate diaphragm/LES dysfunction and increase the propensity for-and severity of - GER.MethodsWe identified patients with fILD who underwent screening polysomnogram and pH or pH/impedence probe at our center during the same week. We examined the association between OSA and GER and used logistic regression to determine independent predictors of OSA or GER.ResultsIn 54 included subjects, neither OSA (dichotomous) nor apnea hypopnea index (continuous) predicted the presence of GER. Regardless of body position (upright, recumbent), GER was no more frequent or severe among subjects with OSA vs. those without OSA. Subjects with idiopathic pulmonary fibrosis (IPF) had an odds of GER nearly seven-fold greater than subjects with other forms of fILD (odds ratio = 6.84, 95% confidence interval 1.36-34.43, p = 0.02). For the entire cohort and the subgroup with IPF, there was no correlation between pulmonary physiology and GER.ConclusionsIn fILD, OSA does not appear to promote GER. Research is needed to determine if compensatory mechanisms emanating from the crural diaphragm prevent GER in fILD patients with OSA and to sort out whether GER has a role in the pathogenesis of certain forms of fILD.
| S-EPMC3362045 | biostudies-literature
Project description:Interstitial lung diseases (ILDs) are chronic, progressive, parenchymal lung diseases with high morbidity and mortality. In recent studies, the prevalence of obstructive sleep apnea (OSA) in patients with ILD has been reported to be high. However, the prevalence and predictive factors of OSA in Korean ILD patients are not well defined. Therefore, the aim of this study was to evaluate the prevalence and predictive factors of OSA in Korean patients with ILD. Clinical data from 86 patients with ILD enrolled from December 2017 to April 2019 at Haeundae-Paik Hospital, Busan, South Korea, were retrospectively analyzed. OSA was monitored with a level 4 portable device and defined as an apnea-hypopnea index of more than 5 per hour of sleep. The median follow-up period was 7 months. The mean age was 69.8 years, and 64% of participants were men. Among the ILDs, idiopathic pulmonary fibrosis (IPF) was the most common (66.3%), followed by connective tissue disease-associated ILD (16.3%) and cryptogenic organizing pneumonia (5.8%). Forty-six ILD patients (53.5%) were diagnosed with OSA, and IPF patients had OSA more frequently (64.9% vs. 31.0%, p = 0.003) than those with other ILDs. Older age (odds ratio [OR], 1.11, 95% CI 1.04-1.19, p = 0.002), higher body weight (OR 1.05, 95% CI 1.01-1.10, p = 0.012), and diabetes mellitus (OR 4.03, 95% CI 1.26-12.91, p = 0.019) were independent risk factors for OSA in the multivariable logistic regression analysis. In the multivariable Cox analysis, an IPF diagnosis was a significant risk factor for one-year mortality (hazard ratio [HR] 7.92, 95% CI: 1.01-61.83, p = 0.048) in ILD patients; however, OSA was not. In conclusion, half of Korean patients with ILD had OSA. Older age, higher body weight, and diabetes mellitus were risk factors for OSA in patients with ILD.
| S-EPMC7535061 | biostudies-literature
Project description:This article reviews the most important articles published in interstitial lung disease, as reviewed during the Clinical Year in Review session at the 2012 annual European Respiratory Society Congress in Vienna, Austria. Since the recent international guidelines for the management of idiopathic pulmonary fibrosis (IPF), important new evidence is available. The anti-fibrotic drug pirfenidone has been recently approved in Europe. Other pharmacological agents, especially nintedanib, are still being tested. The so-called triple combination therapy, anticoagulation therapy and endothelin receptor antagonists, especially ambrisentan, are either harmful or ineffective in IPF and are not recommended as treatment. Although the clinical course of IPF is highly variable, novel tools have been developed for individual prediction of prognosis. Acute exacerbations of IPF are associated with increased mortality and may occur with higher frequency in IPF patients with associated pulmonary hypertension. Interstitial lung disease associated with connective tissue disease has been definitely established to have a better long-term survival than IPF. A subset of patients present with symptoms and/or biological autoimmune features, but do not fulfil diagnostic criteria for a given autoimmune disease; this condition is associated with a higher prevalence of nonspecific interstitial pneumonia pattern, female sex and younger age, although survival relevance is unclear.
| S-EPMC9487426 | biostudies-literature
Project description:Interstitial lung diseases are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. Knowledge achieved in recent years has resulted in the publication of the new classification of idiopathic interstitial pneumonias, according to which there are three groups: major, rare and unclassified. The novelty of the new classification comes from the fact that difficult to classify entities can be treated according to the disease behaviour classification. Idiopathic pulmonary fibrosis is the most lethal amongst the interstitial lung diseases and presents high heterogeneity in clinical behaviour. A number of biomarkers have been proposed in order to predict the course of the disease and group patients with the same characteristics in clinical trials. Early diagnosis and disease stratification is also important in the field of other interstitial lung diseases.
| S-EPMC9487254 | biostudies-literature