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Concise, portable, and user-friendly, The Washington Manual® Pulmonology Subspecialty Consult, 2nd Edition, provides quick access to the essential information needed to evaluate patients on a subspecialty consult service. This edition offers state-of-the-art content on the diagnosis, investigation, and treatment of common acute and chronic lung diseases, including coverage of advancing technologies and therapeutics. Ideal for fellows, residents, and medical students rotating on pulmonology subspecialty services, the manual is also useful as a first-line resource for internists and other primary care providers.

Background: Chronic obstructive pulmonary disease (COPD) has a high incidence rate in China, but the diagnosis rate remains insufficient. This study aimed to explore and compare COPD screening tools for primary healthcare institutions in China. Purpose: Exploring COPD Screening Tools and Their Combined Use for Primary Healthcare Institutions in China. Patients and Methods: From September 2022 to March 2023, a screening for COPD was conducted among residents aged 35 years and above in primary healthcare institutions in Beijing, China. The screening involved the use of the CAPTURE scale, COPD-SQ scale, and peak expiratory flow rate test. Any positive results from these screening tests were followed by further pulmonary function testing to confirm the diagnosis. Sensitivity, specificity, positive predictive value, negative predictive value, and receiver operating characteristic (ROC) curves were calculated for each screening tool alone and in combination. Results: A total of 986 individuals completed the screening tests. The positive rates for the CAPTURE scale, COPD- SQ scale, and peak flow meter screening were 41.78%, 29.11%, and 52.03%, respectively. Of the participants, 166 (24.09%) underwent pulmonary function tests, with an average age of 61.69 [+ or -] 13.68 years. The peak flow meter screening showed the highest sensitivity (83.78%) when used alone, while the COPD-SQ scale exhibited the best specificity (59.69%), positive predictive value (31.58%), and negative predictive value (58.56%). Significant differences (P<0.05) were observed between any two of the three screening tools. Among the combinations, the peak flow meter screening + COPD-SQ scale showed the highest accuracy, with a Youden index of 0.277 and an AUC of 0.638. Conclusion: There is variation in the accuracy of existing screening tools for COPD when used alone. For primary healthcare institutions, the optimal COPD screening tool is the combination of peak flow meter screening and the COPD-SQ questionnaire. If limited by screening equipment conditions, the COPD-SQ questionnaire can be used alone for screening. Keywords: primary health care institutions, chronic obstructive pulmonary disease, COPD, screening questionnaire, peak flow rate test, pulmonary function tests

Bronchiolitis obliterans syndrome (BOS) is a major cause of morbidity and mortality in lung transplantation and allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients. Clinical guidelines recommend lung function monitoring to aid early identification of BOS, but real-world rates of pulmonary function testing (PFT) have not been studied. The purpose of this study was to quantify PFT rates in lung transplantation and allo-HSCT recipients. This longitudinal retrospective study used US data from the IQVIA PharMetrics Plus commercial claims database (January 1, 2006–September 30, 2018) and the Medicare Limited Data Set (January 1, 2010–December 31, 2018). Study recipients had no evidence of transplantation 12 months before transplantation, which was identified by using diagnosis and procedure codes. PFTs were identified by using procedure codes. Outcomes were percentage of recipients who received ≥1 PFT in each follow-up year, including spirometry, lung diffusion capacity, lung function volume test, and plethysmography, including the average number of total and specific tests per recipient. The study identified 367 commercially insured and 1776 Medicare recipients who underwent lung transplantation; 92% and 86% received ≥1 lung function test in the first year after transplantation, respectively. Among recipients observable 3 years after transplant, 85% and 83% received ≥1 PFT. Among 2187 commercially insured and 1864 Medicare recipients who underwent allo-HSCT, 44% and 36% received ≥1 lung function test in the first posttransplant year. In the third year after transplant, only 31% and 26% of observable allo-HSCT recipients underwent any PFT. Morbidity and mortality from BOS remain high in lung transplant and allo-HSCT recipients, but lung function testing in the first posttransplant year is not universal, with substantially lower rates among allo-HSCT recipients. Furthermore, testing rates in all cohorts declined over time. Increased and sustained monitoring could lead to earlier detection of BOS and earlier intervention and treatment.

Background Diagnostic delays are common in patients with interstitial lung disease (ILD). A substantial percentage of patients experience a diagnostic delay in the primary care setting, but the factors underpinning this observation remain unclear. In this multi-center investigation, we assessed ILD reporting on diagnostic test interpretation and its association with subsequent pulmonology referral by a primary care physician (PCP). Methods A retrospective cohort analysis of patients referred to the ILD programs at UC-Davis and University of Chicago by a PCP within each institution was performed. Computed tomography (CT) of the chest and abdomen and pulmonary function test (PFT) were reviewed to identify the date ILD features were first present and determine the time from diagnostic test to pulmonology referral. The association between ILD reporting on diagnostic test interpretation and pulmonology referral was assessed, as was the association between years of diagnostic delay and changes in fibrotic features on longitudinal chest CT. Results One hundred and forty-six patients were included in the final analysis. Prior to pulmonology referral, 66% ( n  = 97) of patients underwent chest CT, 15% ( n  = 21) underwent PFT and 15% (n = 21) underwent abdominal CT. ILD features were reported on 84, 62 and 33% of chest CT, PFT and abdominal CT interpretations, respectively. ILD reporting was associated with shorter time to pulmonology referral when undergoing chest CT (1.3 vs 15.1 months, respectively; p  = 0.02), but not PFT or abdominal CT. ILD reporting was associated with increased likelihood of pulmonology referral within 6 months of diagnostic test when undergoing chest CT (rate ratio 2.17, 95% CI 1.03–4.56; p  = 0.04), but not PFT or abdominal CT. Each year of diagnostic delay was associated with a 1.8% increase in percent fibrosis on chest CT. Patients with documented dyspnea had shorter time to chest CT acquisition and pulmonology referral than patients with documented cough and lung crackles. Conclusions Determinants of ILD diagnostic delays in the primary care setting include underreporting of ILD features on diagnostic testing and prolonged time to pulmonology referral even when ILD is reported. Interventions to modulate these factors may reduce ILD diagnostic delays in the primary care setting.

Background: The significance of the nutritional status in idiopathic pulmonary fibrosis (IPF) is largely unknown. Temporal body weight (BW) change, a dynamic index of nutrition status, can detect the malnutrition more accurately than the conventional single-point body mass index evaluation. Objective: To investigate how the temporal BW change influences the clinical courses of IPF. Methods: This multicenter study enrolled IPF patients from four referral hospitals of interstitial lung diseases in Japan (the Japanese cohort, the derivation cohort) and the Royal Brompton Hospital (the UK cohort, the validation cohort). The annual rate of BW change from the initial presentation was evaluated. A > 5% decrease of BW was defined as a significant BW loss. Results: Twenty-seven out of 124 patients in the Japanese cohort and 13 out of 86 patients in the UK cohort showed significant BW loss. Patients with BW loss showed significantly worse survival in both cohorts. Multivariate analyses revealed that BW loss was an independent factor for decreased survival (Japanese cohort: p = 0.047, UK cohort: p = 0.013). A 6.1% loss of BW was chosen as the optimal cutoff value to predict the 2-year mortality from the initial presentation. The stratified analysis revealed that a 6.1% or greater BW loss could predict worse survival specifically in cases without a greater than 10% decline in forced vital capacity (FVC). Conclusions: BW loss is independently associated with the survival of IPF patients, particularly when a decline in the FVC was not observed. Further studies are needed to understand the mechanisms underlying BW loss in IPF.

Introduction This study we evaluated the association between preoperative clinical, respiratory, and hemodynamic parameters and the post-operative thrombus level in the pulmonary endarterectomy (PEA) material of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Our hypothesis was that whether the level of thrombus is associated with preoperative pulmonary functions. To the best of our knowledge, this is the first study evaluating the relationship between anatomical classification of endarterectomy materials with clinical, respiratory, and hemodynamic parameters. Materials and methods This single-center retrospective cross-sectional study included patients aged 18 and older diagnosed with CTEPH who had PEA at Marmara University School of Medicine Hospital Istanbul between 2013 and 2022. A total of 150 patients with CTEPH who had PEA procedure. Patients whose degree of vascular involvement is unclear were excluded ( n  = 4). Patients whose post-operative pathology compatible other than thrombi (sarcoma, etc.) were excluded n  = 3 (Fig. 2). Patients’ preoperative clinical parameters [New York Heart Association (NYHA) Functional Class (FC), 6-minute walk distance (6MWD)], PFTs, DLCO values, echocardiographic data and hemodynamic parameters measured with right heart catheterization were evaluated along with the thrombi levels of PEA specimens. Results The study included 143 patients diagnosed with CTEPH who had PEA. When the distribution of thrombus materials was examined according to anatomical classification; 18.9% of patients ( n  = 27) were classified as Level 1 (main branch), 40.6% as Level 2 (lobar) ( n  = 58), 32.2% as Level 3 (segmental) ( n  = 46), and 8.4% as Level 4 (subsegmental) ( n  = 12). The mean values of predicted FEV 1 (forced expiratory volume in 1 s) were 2.05 ± 0.55 L (liters) for patients with Level 1 thrombus, 2.29 ± 0.95 L for Level 2, 2.10 ± 0.76 L for Level 3, and 2.86 ± 1.33 L for Level 4 ( p  = 0.053). The DLCOcSB (carbon monoxide diffusion capacity corrected for alveolar volume) values were %60.38 ± 28.98 for patients with Level 1 thrombus, %65.88 ± 20.85 for Level-2, %65.29 ± 18.32 for Level-3, and %85 ± 21.42 for Level-4 ( p  = 0.07). Cardiac indices (L/min/m²) were 2.46 ± 0.84 for Level 1, 2.43 ± 1.14 for Level 2, 2.42 ± 0.76 for Level 3, and 3.04 ± 0.63 for Level 4 ( p  = 0.02). No relationship was observed between the anatomical level of thrombus and 6MWD and the hemodynamic data measured via RHC except cardiac indices (all p values > 0.05). Conclusion Our study did not find a significant relationship between the preoperative parameters of PFTs, DLCO values, and hemodynamic parameters excluding cardiac index with the anatomical classification of thrombi in patients who had PEA for CTEPH. To the best of our knowledge, this is the first study evaluating the relationship between anatomical classification of endarterectomy materials with clinical, respiratory, and hemodynamic parameters.

The development of pulmonary arterial hypertension (PAH) can complicate many interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF). We sought to characterize the prevalence of PAH and its impact on survival in patients with advanced IPF. Retrospective analysis of consecutive IPF patients undergoing pretransplantation right heart catheterization. Lung transplant and IPF referral center. PAH was defined as a mean pulmonary artery pressure (mPAP) of > 25 mm Hg. We compared demographic, spirometric, 6-min walk test (6MWT) results, and survival outcomes between those with PAH and those without PAH. Seventy-nine patients were included in the study. PAH was present in 31.6% of patients (mean [± SD] mPAP, 29.5 ± 3.3 vs 19.1 ± 3.7 mm Hg, respectively). Those patients with PAH had a lower mean diffusing capacity of the lung for carbon monoxide (Dlco) (37.6 ± 11.3% vs 31.1 ± 10.1%, respectively; p = 0.04) and were more likely to require supplemental oxygen (66.7% vs 17.6%, respectively; p < 0.0001). Mean distance walked (143.5 ± 65.5 vs 365.9 ± 81.8 m, respectively; p < 0.001) and mean pulse oximetric saturation nadir (80.1 ± 3.7% vs 88.0 ± 3.5%, respectively; p < 0.001) during the 6MWT were also lower among those with PAH. PAH was associated with a greater risk of death during the study period (mortality rate, 60.0% vs 29.9%, respectively; odds ratio, 2.6; 95% confidence interval [CI], 2.3 to 3.1; p = 0.001). One-year mortality rates were higher in those with PAH (28.0% vs 5.5%, respectively; p = 0.002). As a predictor of mortality, PAH had a sensitivity, specificity, and accuracy of 57.1%, 79.3%, and 73.4%, respectively. There was a linear correlation between mPAP and outcomes with higher pressures associated with a greater risk of mortality (hazard ratio, 1.09; 95% CI, 1.02 to 1.16). FVC and Dlco did not predict outcomes. PAH is common in advanced cases of IPF and significantly impacts survival. A reduced Dlco, supplemental oxygen requirement, or poor 6-min walk performance should raise suspicion of the presence of underlying PAH. Identifying PAH might be an important adjunct in monitoring disease progression, triaging for transplantation, and guiding therapy.

Purpose To investigate the prognostic value of quantitative analysis of CT among patients with idiopathic pulmonary fibrosis (IPF) by quantifying the fibrosis extent and to attempt to provide precise medium-long term prognostic predictions for individual patients. Methods This was a retrospective cohort study that included 95 IPF patients in Zhongshan Hospital, Fudan University. 64 patients firstly diagnosed with IPF from 2009 to 2015 was included as the derivation cohort. Information regarding sex, age, the Gender-Age-Physiology (GAP) index, high-resolution computed tomography (HRCT) images, survival status, and pulmonary function parameters including forced vital capacity (FVC), FVC percent predicted (FVC%pred), diffusing capacity of carbon monoxide (DLCO), DLCO percent predicted (DLCO%pred), carbon monoxide transfer coefficient (KCO), KCO percent predicted (KCO%pred) were collected. 31 patients were included in the validation cohort. The Synapse 3D software was used to quantify the fibrotic lung volume (FLV) and total lung volume (TLV). The ratio of FLV to TLV was calculated and labeled CT FLV/TLV% , reflecting the extent of fibrosis. All the physiological variants and CT FLV/TLV% were analyzed for the dimension of survival through both univariate analysis and multivariate analysis. Formulas for predicting the probability of death based on the baseline CT FLV/TLV% were calculated by logistic regression, and validated by the validation cohort. Results The univariate analysis indicated that CT FLV/TLV% along with DLCO%pred, KCO%pred and GAP index were significantly correlated with survival. However, only CT FLV/TLV% was meaningful in the multivariate analysis for prognostic prediction (HR 1.114, 95% CI 1.047–1.184, P = 0.0006), and the best cutoff was 11%, based on receiver operating characteristic (ROC) curve analysis. The survival times for the CT FLV/TLV%  ≤ 11% and CT FLV/TLV%  > 11% groups were significantly different. Given the CT FLV/TLV% data, the death probability of a patient at 1 year, 3 years and 5 years could be calculated by using a particular formula. The formulas were tested by the validation cohort, showed high sensitivity (88.2%), specificity (92.8%) and accuracy (90.3%). Conclusion Quantitative volume analysis of CT might be useful for evaluating the extent of fibrosis in the lung. The CT FLV/TLV% could be a valuable biomarker for precisely predicting the medium-long term prognosis of individual patients with IPF.

Background. Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease with rapidly progressive evolution and an unfavorable outcome. Nintedanib (NTD) is an antifibrotic drug that has been shown to be effective in slowing down the progression of the disease. The aim of our study was to examine the efficacy, especially in terms of the functional decline, and the safety profile of NTD in patients treated with the recommended dose and subjects who reduced or suspended the therapy due to the occurrence of adverse reactions. Methods. We conducted a real-life retrospective study based on the experience of NTD use in two centers between 2015 and 2022. Clinical data were evaluated at baseline, at 6 and 12 months after the NTD introduction in the whole population and in subgroups of patients who continued the full-dose treatment, at a reduced dosage, and at the discontinuation of treatment. The following data were recorded: the demographic features, IPF clinical features, NTD therapeutic dosage, tolerability and adverse events, pulmonary function tests (PFTs), the duration of treatment upon discontinuation, and the causes of interruption. Results. There were 54 IPF patients who were included (29.6% females, with a median (IQR) age at baseline of 75 (69.0–79.0) years). Twelve months after the introduction of the NTD therapy, 20 (37%) patients were still taking the full dose, 11 (20.4%) had reduced it to 200 mg daily, and 15 (27.8%) had stopped treatment. Gastrointestinal intolerance predominantly led to the dose reduction (13.0%) and treatment cessation (20.4%). There were two deaths within the initial 6 months (3.7%) and seven (13.0%) within 12 months. Compared to the baseline, the results of the PFTs remained stable at 6 and 12 months for the entire NTD-treated population, except for a significant decline in the DLCO (% predicted value) at both 6 (38.0 ± 17.8 vs. 43.0 ± 26.0; p = 0.041) and 12 months (41.5 ± 15.3 vs. 44.0 ± 26.8; p = 0.048). The patients who continued treatment at the full dose or a reduced dosage showed no significant differences in the FVC and the DLCO at 12 months. Conversely, those discontinuing the NTD exhibited a statistically significant decline in the FVC (% predicted value) at 12 months compared to the baseline (55.0 ± 13.5 vs. 70.0 ± 23.0; p = 0.035). Conclusions. This study highlights the functional decline of the FVC at 12 months after the NTD initiation among patients discontinuing therapy but not among those reducing their dosage.