Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is defined as a chronic fibrosing interstitial pneumonia limited to the lung, of unknown cause, with poor prognosis and few treatment options. In recent years there has been an increase in their prevalence, probably due to the optimization of diagnostic methods and increased life expectancy. The ATS/ERS Consensus (2000) established the diagnostic criteria and recommendations for the assessment of the disease course and treatment. Later studies have helped to redefine diagnostic criteria and treatment options. In 2011, an international consensus was published, establishing diagnostic criteria and new treatment strategies. These guidelines have been updated with the newest aspects of diagnosis and treatment of idiopathic pulmonary fibrosis. A level of evidence has been identified for the most relevant questions, particularly with regard to treatment options.     

Translation to Spanish and Validation of the Specific Saint George's Questionnaire for Idiopathic Pulmonary Fibrosis

Introduction

Interstitial lung disease (ILD) is associated with low exercise tolerance, dyspnea, and decreased health-related quality of life (HRQL). Idiopathic pulmonary fibrosis (IPF) is one of the most prevalent in the group. A specific version of the Saint George's questionnaire (SGRQ-I) has been developed to quantify the HRQL of IPF patients. However, this tool is not currently validated in the Spanish language. The objective was to translate into Spanish and validate the specific Saint George's Respiratory Questionnaire for idiopathic pulmonary fibrosis (SGRQ-I).

Racial and Ethnic Disparities in Survival in Lung Transplant Candidates with Idiopathic Pulmonary Fibrosis

Minority patients have worse outcomes than nonminority patients in a variety of pulmonary diseases. We aimed to compare the survival of Black and Hispanic patients to that of others with idiopathic pulmonary fibrosis (IPF). We performed a retrospective cohort study of patients with IPF who were evaluated for lung transplantation at our center. Kaplan-Meier survival curves and Cox proportional hazards models were used to compare survival between groups. Black and Hispanic patients had spirometry, lung volumes and diffusion capacity that were similar to others, but had worse exercise capacity. Minority patients had a significantly increased risk of death compared to others independent of transplantation status (hazard ratio = 3.3, 95% CI 1.2-8.9, p = 0.02). Differences in exercise capacity, pulmonary hemodynamics and socioeconomic factors appeared to account for some of the differences in survival. Black and Hispanic patients with IPF had an increased risk of death following referral for lung transplantation. This finding may be due to differences in disease progression and/or differences in access to medical care among minority patients. Future studies should confirm our findings in a larger cohort. The elimination of racial and ethnic disparities in outcome should be a priority for clinicians and researchers in this field.     

A multidisciplinary proposal for a diagnostic algorithm in idiopathic pulmonary fibrosis: The role of transbronchial cryobiopsy

The diagnosis of idiopathic pulmonary fibrosis (IPF) is a complex process that requires the multidisciplinary integration of clinical, radiological, and histological variables. Due to its diagnostic yield, surgical lung biopsy has been the recommended procedure for obtaining samples of lung parenchyma, when required. However, given the morbidity and mortality of this technique, alternative techniques which carry a lower risk have been explored. The most important of these is transbronchial cryobiopsy—transbronchial biopsy with a cryoprobe—which is useful for obtaining lung tissue with less comorbidity. Yield may be lower than surgical biopsy, but it is higher than with transbronchial biopsy with standard forceps. This option has been discussed in the recent clinical guidelines for the diagnosis of IPF, but the authors do not go so far as recommend it. The aim of this article, the result of a multidisciplinary discussion forum, is to review current evidence and make proposals for the use of transbronchial cryobiopsy in the diagnosis of IPF.     

Effect of Sex Differences on Computed Tomography Findings in Adults With Cystic Fibrosis: A Multicenter Study

BackgroundThe survival of women with cystic fibrosis (CF) is lower than that of men by approximately 5 years. While various factors have been put forward to account for this discrepancy, no specific reasons have been established. Our hypothesis was that anatomical-structural involvement is more pronounced in women with CF than in men and that this is reflected in thoracic HRCT findings.Material and methodsWe performed a prospective multicentre study, in which adult patients were consecutively included over 18 months. Chest HRCT was performed, and findings were scored by 2 thoracic radiologists using the modified Bhalla system. We also studied respiratory function, applied the CFQR 14+ questionnaire, and collected clinical variables.ResultsOf the 360 patients followed up in the participating units, 160 were eventually included. Mean age was 28 years, and 47.5% were women. The mean ± SD global score on the modified Bhalla score was 13.7 ± 3.8 in women and 15.2 ± 3.8 in men (p = 0.024). The highest scores were observed for sacculations, bronchial generations, and air trapping in women. Women had lower BMI, %FEV₁, %FVC, and %DLCO. Similarly, the results for the respiratory domain in CFQR 14+ were worse in women, who also had more annual exacerbations.ConclusionsThis is the first study to provide evidence of the implication of sex differences in HRCT findings in patients with CF. Women with CF present a more severe form of the disease that results in more frequent exacerbations, poorer functional and nutritional outcomes, deterioration of quality of life, and greater structural damage.     

Relationship Among Radiological Measurements of Anterior Mediastinal Fat and Outcomes of Lung Transplantation in Fibrotic Patients

ObjectiveLung transplantation (LT) for pulmonary fibrosis is related to higher mortality than other transplant indications. We aim to assess whether the amount of anterior mediastinal fat (AMF) was associated to early and long-term outcomes in fibrotic patients undergoing LT.MethodsRetrospective analysis of 92 consecutive single lung transplants (SLT) for pulmonary fibrosis over a 10-year period. AMF dimensions were measured on preoperative CT-scan: anteroposterior axis (AP), transverse axis (T), and height (H). AMF volumes (V) were calculated by the formula: AP × T × H × 3.14/6.According to the radiological AMF dimensions, patients were distributed into two groups: low-AMF (V < 20 cm³) and high-AMF (V > 20 cm³), and early and long-term outcomes were compared by univariable and multivariable analyses.ResultsThere were 92 SLT: 73M/19F, 53 ± 11 [14–68] years old. 30-Day mortality (low-AMF vs. high-AMF): 5 (5.4%) vs. 15 (16.3%), p = 0.014. Patients developing primary graft dysfunction within 72 h post-transplant, and those dying within 30 days post-transplant presented higher AMF volumes: 21.1 ± 19.8 vs. 43.3 ± 24.7 cm³ (p = 0.03) and 24.4 ± 24.2 vs. 56.9 ± 63.6 cm³ (p < 0.01) respectively. Overall survival (low-AMF vs. high-AMF) (1, 3, and 5 years): 85%, 81%, 78% vs. 55%, 40%, 33% (p < 0.001).Factors predicting 30-day mortality were: BMI (HR = 0.77, p = 0.011), AMF volume (HR = 1.04, p = 0.018), CPB (HR = 1.42, p = 0.002), ischaemic time (HR = 1.01, p = 0.009).Factors predicting survival were: AMF volume (HR = 1.02, p < 0.001), CPB (HR = 3.17, p = 0.003), ischaemic time (HR = 1.01, p = 0.001).ConclusionPreoperative radiological assessment of mediastinal fat dimensions and volumes may be a useful tool to identify fibrotic patients at higher risk of mortality after single lung transplantation.     

Clinical Research on Prognostic Evaluation of Subjects With IPF by Peripheral Blood Biomarkers,Quantitative Imaging Characteristics and Pulmonary Function Parameters

IntroductionIdiopathic pulmonary fibrosis (IPF) is an irreversible and progressive fatal interstitial lung disease with a poor prognosis. The aim of this study is to investigate the predictive value of combined blood biomarkers, pulmonary function and quantitative monitoring by computer-aided diagnosis (CAD) system in IPF patients.MethodsPulmonary baseline function and pathological features of 126 patients with IPF were analyzed using spirometry and chest X-ray. Patients were divided into survival group and non-survival group after 5 years follow-up. The relationships the levels of peripheral blood biomarkers, quantitative imaging characteristics and pulmonary function were analyzed between the two groups.ResultsThe baseline level of serum Krebs von den Lungen-6 (KL-6) and C-X-C motif chemokine 13 (CXCL13) were moderately or highly correlated with annual changes in forced vital capacity (FVC), carbon monoxide diffusing capacity (DLCO), total lung capacity (TLC), total interstitial lung disease (ILD) lesions, and the volume changes of reticular. The baseline level of serum KL-6 was higher than the cut-off value of 800.0 U/ml and baseline level of serum CXCL13 was higher than the cut-off value of 62.0 pg/ml. IPF patients with baseline levels of serum KL-6 and CXCL13 lower than the cut-off value had longer median survival time.ConclusionsSerum KL-6 and CXCL13 may be predictive biomarkers for the outcomes of patients with IPF patients and their baseline levels were related to the progression of pulmonary function and quantitative monitoring by CAD system.     

Endobronchial Ultrasound for the Diagnosis of Peripheral Pulmonary Lesions. A Controlled Study With Fluoroscopy

IntroductionFluoroscopy-guided bronchoscopy is usually performed for the diagnosis of peripheral pulmonary lesions (PPL), but the diagnostic yield varies widely among studies. Endobronchial ultrasound (EBUS) can increase the diagnostic yield of bronchoscopic diagnosis of PPL.ObjectiveTo compare the diagnostic yield of fluoroscopy-guided bronchoscopy and EBUS with fluoroscopy-guided bronchoscopy in the study of PPL.MethodsAll patients who underwent bronchoscopy to study PPL from January 2009 to December 2012 were prospectively included. A total of 145 consecutive patients were randomly distributed in two groups: EBUS and fluoroscopy (50 patients, 71.3±8.2 years) or fluoroscopy alone (95 patients, 68±10.5 years). The mean diameter of the lesions was 41.97±19.22 mm. Cytological brushing and transbronchial biopsies were obtained. All procedures were performed under fluoroscopic guidance with intravenous conscious sedation. EBUS was performed using an endoscopic ultrasound system equipped with a 20-MHz radial miniprobe introduced via a guide-sheath. Bronchoscopist, cytologist, study protocol, techniques and tools were the same throughout the whole study.ResultsIn all, 129 (89%) patients had malignant disease. A diagnosis with bronchoscopy was established in 105 (72.4%) patients. EBUS plus fluoroscopy obtained a diagnostic yield in 78% of patients and fluoroscopy alone in 69.5% (non-significant). In contrast, for lesions smaller than 30 mm, EBUS plus fluoroscopy guidance provided significantly greater diagnostic performance than fluoroscopy alone (90% vs 52%; P=.05).ConclusionsBronchoscopy under EBUS plus fluoroscopy guidance is a technique that has become useful for the diagnosis of LPPs, especially those smaller than 30 mm in diameter.     

A Population-Based Cohort Study on Chronic Obstructive Pulmonary Disease in Latin America: Methods and Preliminary Results. The PLATINO Study Phase II

BackgroundThe PLATINO baseline study, conducted from 2003 to 2005 in five Latin American cities (São Paulo, Mexico City, Montevideo, Santiago, Caracas), showed a high prevalence of chronic obstructive pulmonary disease (COPD).Methods/designA follow-up study was conducted in three out of the five centers (Montevideo, Santiago, and São Paulo) after a period of 5, 6 and 9 years, respectively, aimed at verifying the stability of the COPD diagnosis over time, the evolution of the disease in terms of survival, morbidity and respiratory function, and the analyses of inflammatory and genetic biomarkers in the blood. Some questions were added to the original questionnaire and death certificates were obtained from the national official registries.ResultsThe fieldwork has been concluded in the three centers. From the original samples in the PLATINO study phase i, we were able to locate and interview 85.6% of patients in Montevideo, 84.7% in Santiago and 77.7% in São Paulo. Individuals who could not be located had higher education levels in Brazil, and were more likely to be current smokers in Santiago and São Paulo than in Montevideo. The overall quality of spirometries was ≥80% according to American Thoracic Society criteria. The number of deaths was 71 (Montevideo), 95 (Santiago) and 135 (São Paulo), with death certificates obtained from the national mortality registries for 76.1%, 88.3% and 91.8% of cases in Montevideo, Santiago and São Paulo, respectively.ConclusionsThis study shows that it is possible to perform population-based longitudinal studies in Latin American with high follow-up rates and high-quality spirometry data. The adequacy of national mortality registries varies among centers in Latin America.