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In addition to idiopathic pulmonary fibrosis (IPF), other diffuse interstitial lung diseases (ILD) are also associated with pulmonary fibrosis and occur in a variable proportion of patients, depending on the entity. The name given to this fibrotic component, that may progress despite treatment, is progressive pulmonary fibrosis (PPF). In this context, PPF is not an entity per se but a common clinical condition or behavior that may occur in association with different types of fibrosing diffuse ILDs, compromising patient prognosis. PPF is identified from worsening clinical, physiological, and/or radiological criteria during patient follow-up. Randomized clinical trials in patients with IPF or progressive non-IPF ILD have shown that treatment with antifibrotic drugs, either nintedanib or pirfenidone, slows progression. We are seeing the start of a new era in the clinical management of this subgroup of patients, offering the perfect opportunity for exploring still uncharted territories., (Copyright © 2022 SEPAR. Published by Elsevier España, S.L.U. All rights reserved.)

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., (Copyright © 2020. Publicado por Elsevier España, S.L.U.)

Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis. In 2015, the international consensus of 2011 was updated and new therapeutic recommendations were established, prompting us to update our recommendation for the medical treatment of idiopathic pulmonary fibrosis accordingly. Diagnostic aspects and non-pharmacological treatment will not be discussed as no relevant developments have emerged since the 2013 guidelines., (Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Three percent of rare diseases are pneumopathies. Improvements in survival and quality of life have led to a new situation where patients with rare respiratory diseases want to plan their reproductive lives. The intention of this review is to present the experience accumulated in the field of the reproductive health of these women. In several rare respiratory diseases, a genetic base has been identified. The combination of preimplantation genetic diagnosis, assisted reproduction and molecular biology techniques enable embryos to be studied genetically before being transplanted into the uterus. Therefore, the risk for transmitting a certain disease or chromosome alteration may be avoided in high-risk couples, and prenatal diagnoses may be done by chorionic villus sampling or amniocentesis. As a general rule, contraceptive methods should be personalized by evaluating the general state of female patients as well as their possibilities for pregnancy, complications and the future possibility of lung transplantation. In lymphangioleiomyomatosis and primary pulmonary hypertension, pregnancy is considered a contraindication. In the former, there is a very high risk for pneumothorax and loss of lung function. In the latter, mortality reaches 33%. In cystic fibrosis, it is estimated that each year 4% of patients become pregnant and there is no observed loss in lung function. There are special circumstances in childbirth that should be considered as well as specific anesthesia risks. The present review suggests that the decision about contraceptive methods, pregnancy as a contraindication or conditions for managing a pregnancy should be both individualized and multidisciplinary., (Copyright © 2012 SEPAR. Published by Elsevier España, S.L. All rights reserved.)

Introduction: The six-minute walk test (6MWT) is widely used in evaluating diffuse interstitial lung disease (ILD) and pulmonary hypertension (PH). However, their physiological determining factors have not been well defined., Objective: To evaluate the physiological changes that occur in ILD and PH during the 6MWT, and compare them with the cardiopulmonary exercise test (CPET)., Material and Methods: Thirteen patients with ILD and 14 with PH were studied using the 6MWT and CPET on an ergometer cycle. The respiratory variables were recorded by means of telemetry during the 6MWT., Results: Oxygen consumption (VO(2)), respiratory and heart rate reached a plateau from minute 3 of the 6MWT in both diseases. The VO(2) did not differ from the peak value in the CPET (14+/-2 and 15+/-2 ml/kg/min, respectively, in ILD; 16+/-6 and 16+/-6 ml/kg/min, in PH). The arterial oxygen saturation decreased in both diseases, although it was more marked in ILD (-12+/-5%, p<0,01). The ventilatory equivalent for CO(2) (V(E)/VCO(2)) in PH during the 6MWT was strongly associated with functional class (FC) (85+/-14 in FC III-IV, 44+/-6 in FC I-II; p<0,001)., Conclusions: The 6MWT in ILD and PH behaves like a maximal effort test, with similar VO(2) to the CPET, demonstrating a limit in oxygen transport capacity. Monitoring using telemetry during the 6MWT may be useful for the clinical evaluation of patients with ILD or PH., (Copyright 2009 SEPAR. Published by Elsevier Espana. All rights reserved.)

Peribronchiolar metaplasia is a histologic lesion characterized by fibrosis and bronchiolar epithelial proliferation, affecting peribronchiolar alveolar septa and terminal bronchioles. It has been considered a nonspecific tissue reaction secondary to the action of external factors, such as tobacco smoke and microbes, and is a common histologic finding in several diffuse interstitial lung diseases. Several such cases with clinical, radiologic, and lung function manifestations characteristic of idiopathic interstitial pneumonia have been described recently, all having peribronchiolar metaplasia as the only histologic abnormality. We report 2 cases of interstitial lung disease in which peribronchiolar metaplasia was the only finding of pathology.

Objective: The role of different techniques for mediastinal staging in patients with suspected lung cancer is a subject of debate. The aim of this study was to analyze the diagnostic yield and cost-effectiveness of transbronchial needle aspiration in the mediastinal staging of lung cancer in patients being evaluated in a tertiary hospital., Patients and Methods: This was a retrospective, observational study of the results of transbronchial needle aspiration in patients with suspected lung cancer and mediastinal lymph node involvement. A cost-effectiveness analysis of the systematic use of this technique was also performed., Results: One-hundred ninety-four patients (85% men, 15% women) were evaluated. The diagnosis of lung cancer was confirmed in 157 (81%). Cytology samples obtained by transbronchial needle aspiration were adequate in 147 (76%) of the 194 cases. When only the adequate samples were included in the analysis, transbronchial needle aspiration showed a sensitivity of 88%, specificity of 100%, positive predictive value of 100%, negative predictive value of 64%, and efficiency of 90%. Mediastinoscopy was avoided in 44 (34%) of the 127 patients with localized non-small cell lung cancer, with an estimated saving of euro 119,456., Conclusions: Transbronchial needle aspiration has a high diagnostic yield and obviates the need for mediastinoscopy in a significant percentage of cases. This finding is of diagnostic and economic significance.

Diffuse interstitial lung diseases form a group of respiratory diseases about which many questions remain to be answered. In recent years there have been major advances in the correct diagnostic classification of each disease, and therefore, the essential foundations have been laid for investigation of their pathophysiology. However, both the triggers and the precise mechanisms that lead to irreversible changes in the lung parenchyma remain to be identified. Idiopathic pulmonary fibrosis is the most common diffuse interstitial lung disease and has the worst prognosis. Current treatments are empirical and the response is random; furthermore, they do not improve survival. Consequently, most basic research has focused on the pathophysiology of the disease and on identifying an effective therapeutic approach. The aim of this review is to describe the experimental studies that have begun to open the way towards an understanding of the complex process of fibrosis.

Airway-centered interstitial fibrosis is a little known clinical entity that has only recently been described in the literature. Its pathology is characterized by bronchial fibrosis and localized interstitial pulmonary fibrosis around the airways. The disease has been associated with inhalation of a variety of substances, environmental or occupational, organic or inorganic. Clinical signs, radiographic manifestations, and lung function in patients with airway-centered interstitial fibrosis are similar to those of patients with idiopathic interstitial pneumonia. We describe a case of airway-centered interstitial fibrosis related to exposure to fumes from cleaning products.

Objective: Transforming growth factor ss1 (TGF-ss1) is one of the key profibrotic mediators in the pathogenesis of idiopathic pulmonary fibrosis (IPF). The purpose of this study was to investigate the prognostic value of quantifying TGF-ss1 levels in patients with IPF., Patients and Methods: We conducted a prospective study of 29 IPF patients and 27 healthy controls. Enzyme-linked immunosorbent assays were used to quantify TGF-ss1 levels., Results: Mean (SD) TGF-ss1 levels were significantly higher in the IPF patients than in the control subjects (11.1 [7.5] ng/mL vs 4 [2.4] ng/mL; P< .01). Weak inverse correlations were observed between TGF-ss1 levels and both forced vital capacity and total lung capacity. Thirteen IPF patients were evaluated at 8 (1.2) months (range, 5-9 months). The mean TGF-ss1 level was 18.2 (15) ng/mL and there were no significant differences with respect to the initial measurement of 11.1 (7.5) ng/mL. No correlation was observed between changes in respiratory function and changes in TGF-ss1 levels., Conclusions: Although plasma levels of TGF-beta1 were high in the patients with IPF, they do not appear to be a useful prognostic marker of disease activity or therapeutic response.