Las futuras técnicas diagnósticas en neumología, cambiar el chip / Future Diagnostic Techniques in Pulmonology. A Change in Mindset

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How we will diagnose IPF in the future.

The recent approval of two safe and effective treatments for patients with idiopathic pulmonary fibrosis (IPF) had as a direct consequence the absolute need for an accurate and early diagnosis. The standard approach to IPF diagnosis has proven to be effective and emphasized the importance of clinical and laboratory evaluations to exclude known causes of pulmonary fibrosis. At the same time, chest high-resolution computed tomography (HRCT) has proven to be the crucial initial diagnostic test, by identifying those patients who should undergo surgical lung biopsy to secure a confident diagnosis and an adequate treatment. However, this diagnostic approach showed over the years some limitations. First, many suspected IPF patients present with atypical HRCT appearances and at the same time are unfit (or unwilling) for surgical lung biopsy, therefore making a confident diagnosis of IPF impossible. Although the current recommendations indicate the need for an iterative multidisciplinary process incorporating available clinical, laboratory, imaging and histological features, recent work has identified new tools which might improve the overall accuracy of this process. Genomic techniques have been already applied to molecularly phenotype patients with interstitial lung disease and it is likely that in the near future clinicians will utilize blood or lung-specific molecular markers in combination with other clinical, physiological, or imaging features. The availability of new sampling procedures (e.g. transbronchial cryobiopsies), together with innovative imaging technologies (e.g. microCT) will most likely support and enhance diagnostic efforts, refine prognostic recommendations and ultimately influence therapeutic options.

Advanced medical interventions in pleural disease.

The burden of a number of pleural diseases continues to increase internationally. Although many pleural procedures have historically been the domain of interventional radiologists or thoracic surgeons, in recent years, there has been a marked expansion in the techniques available to the pulmonologist. This has been due in part to both technological advancements and a greater recognition that pleural disease is an important subspecialty of respiratory medicine. This article summarises the important literature relating to a number of advanced pleural interventions, including medical thoracoscopy, the insertion and use of indwelling pleural catheters, pleural manometry, point-of-care thoracic ultrasound, and image-guided closed pleural biopsy. We also aim to inform the reader regarding the latest updates to more established procedures such as chemical pleurodesis, thoracentesis and the management of chest drains, drawing on contemporary data from recent randomised trials. Finally, we shall look to explore the challenges faced by those practicing pleural medicine, especially relating to training, as well as possible future directions for the use and expansion of advanced medical interventions in pleural disease.

Evolving imaging techniques in diagnostic strategies of pulmonary embolism.

Modern non invasive diagnostic strategies for pulmonary embolism (PE) rely on the sequential use of clinical probability assessment, D-dimer measurement and thoracic imaging tests. Planar ventilation/perfusion (V/Q) scintigraphy was the cornerstone for more than two decades and has now been replaced by computed tomography pulmonary angiography (CTPA). Diagnostic strategies using CTPA are very safe to rule out PE and have been well validated in large prospective management outcome studies. With the widespread use of CTPA, concerns regarding radiation and overdiagnosis of PE have paved the way for investigating new diagnostic modalities. V/Q single photon emission tomography has arisen as a highly accurate test and a potential alternative to CTPA. However, prospective management outcome studies are still lacking and are warranted before implementation in everyday clinical practice.

Improving the diagnosis and management of COPD.

COPD is a progressive condition. Therefore, earlier diagnosis allows earlier intervention in particular smoking cessation. Stopping smoking in early middle age where an individual has relatively mild COPD is associated with a slower decline in lung function and reduced mortality. Spirometry should be performed in symptomatic current or former smokers (typically ≥ 10 pack years) who are aged at least 35 where COPD is a likely differential diagnosis. Once airflow obstruction is proven and a diagnosis of COPD established then a measure of COPD severity can be made based on FEV1 expressed as a percentage of predicted value. When an individual with COPD is assessed it is vital that comorbid conditions are considered and management optimised. Cardiovascular disease and diabetes were seen most commonly in people enrolled in the active and sometimes sleep. The COPD Assessment Tool is a simple measure of health status that takes under five minutes to complete. Performing spirometry each year can identify patients with a rapid, progressive decline in lung function and allow this to be addressed. Inhaler technique should be checked at this review and also when a new type of inhaler is commenced.

Fibrosis quística diagnosticada en edad adulta / Cystic fibrosis in adult age

Objetivo. Precisar la prevalencia de enfermos con fibrosis quística (FQ) diagnosticados en edades superiores a los 18 años y analizar sus características clínicas, genéticas y microbiológicas. Pacientes y métodos. Estudio observacional, transversal y descriptivo de todos los pacientes diagnosticados de FQ a edad igual o superior a los 18 años. Las variables analizadas fueron: edad actual, edad al diagnóstico, sexo, nacionalidad, parámetros de función pulmonar, patologías presentes al momento del diagnóstico, características microbiológicas y hallazgos genéticos. Resultados. Se incluyeron 89 pacientes (14,8%, del total de 600 pacientes en seguimiento en las unidades participantes), 45 mujeres (50,6%) y 44 varones (49,4%), con una edad media al diagnóstico de 36,4 años. Ochenta y un pacientes (91%) eran de nacionalidad española. La prueba del sudor fue diagnóstica en 77 de los 89 estudiados (86,5%). Las mutaciones detectadas con mayor frecuencia fueron la F508del/otra y la G542X/otra y los hallazgos clínicos más frecuentes en el momento del diagnóstico fueron las bronquiectasias en 33 pacientes (37,1%) y la esterilidad en 12 (13,5%). Los microorganismos colonizadores más frecuentes fueron Staphylococcus aureus (S.aureus) sensible a meticilina (23,6%) y Pseudomonas aeruginosa (P. aeruginosa) (13,5%). La mayoría de los pacientes presentaban una alteración ventilatoria obstructiva leve y no tenía afectación pancreática. La prueba del sudor con frecuencia ofreció resultados no concluyentes. Conclusiones. La FQ es también una enfermedad de diagnóstico en la edad adulta. Los pacientes diagnosticados en edad adulta presentan una función pulmonar levemente alterada y una baja incidencia de afectación pancreática, por lo que su pronóstico tiende a ser favorable (AU)

Aim. To know the prevalence of the patients diagnosed of cystic fibrosis (CF) older than 18 years old of five specific Spanish Units and to analyze their clinical, genetic and microbiological characteristics. Patients and methods. Observational, cross-sectional, descriptive study of patients diagnosed with CF at age or older than 18 years. The variables analyzed were: current age, age at diagnosis, sex, nationality, lung function parameters, pathologies presented at diagnosis, microbiological features and genetic findings. Results. Eigthy nine patients (14.8% of the total of 600 CF patients followed at the participating units), of which 45 patients were female (50.6%) and 44 were males (49.4%), were included with a mean age at diagnosis of 36.4 years. Eigthy one patients (91%) were Spaniards. The sweat test was diagnostic in 77 (86.5%) of the patients studied. The sweat test was diagnostic in 77 of the 89 patients studied (86.5%). The most frequently detected mutations were F508del/other and G542X/other, and the most frequent clinical findings at diagnosis were the presence of bronchiectasis in 33 patients (37.1%) followed by sterility in 12 patients (13.5%). The most common colonizing organisms were meticillin-sensitive Staphylococcus aureus (S.aureus) (23.6%) and Pseudomonas aeruginosa (P. aeruginosa) (13.5%). Most patients presented a mild obstructive ventilatory defect and had no pancreatic involvement. The sweat test used to be indeterminate. Conclusions. CF is also a disease which diagnosis can be in adulthood. CF patients diagnosed in adulthood have a mild lung function and lower incidence of pancreatic involvement, so their prognosis tends to be favorable (AU)

[Idiopathic pulmonary fibrosis: recent diagnostic and therapeutic advances]. / Fibrose pulmonaire idiopathique: nouveautés diagnostiques et thérapeutiques.

Idiopathic pulmonary fibrosis (IPF) is the most frequent of the idiopathic interstitial pneumonias. It is a progressive disorderwith a poor prognosis. Its diagnosis requires the careful exclusion of potential causes, and a pattern of usual interstitial pneumonia at high-resolution computed tomography or video-assisted surgical lung biopsy. Several recent randomized trials have profoundly modified the therapeutic management of IPF. The combination of prednisone and azathioprine, often prescribed until recently, has been shown to be harmful and is no longer indicated. N-acetylcystein, also used in the past decade, failed to show an efficacy. However, two new antifibrotic drugs, pirfenidone and nintedanib, have for the first time proven effective in slowing disease progression.

[Tracheobronchomalacia in adults: breakthroughs and controversies]. / Trachéobronchomalacie de l'adulte: avancées et controverses.

Tracheobronchomalacia (TBM) in adults is a disease defined by a reduction of more than 50% of the airway lumen during expiration. It encompasses many etiologies that differ in their morphologic aspects, pathophysiological mechanisms and histopathologies. TBM is encountered with increasing frequency, as it is more easily diagnosed with new imaging techniques and diagnostic bronchoscopy, as well as because of its frequent association with Chronic Obstructive Pulmonary Disease (COPD), which represents the most frequent etiology for acquired TBM in adults. A distinction between TBM in association with failure of the cartilaginous part of the airways and TBM affecting only the posterior membranous part is emerging since their physiopathology and treatment differ. The therapeutic management of TBM should be as conservative as possible. Priority should be given to identification and treatment of associated respiratory diseases, such as asthma or COPD. Surgery addressing extrinsic compression (thyroid goiter or tumor, for example) may be necessary. Noninvasive ventilation can be considered in patients with increasing symptoms. Endoscopic options, such as the placement of stents, should only be used as palliative or temporary solutions, because of the high complication rates. Symptomatic improvement after stenting might be helpful in selecting patients in whom a surgical management with tracheobronchoplasty can be useful.

Monitoring and analysis of lung sounds remotely.

Visual and auditory analysis of respiratory sound signals promises improved detection of certain types of lung diseases. LabVIEW software was used to design a system that monitors the respiratory activity of the patient. The program developed calculates the respiratory rate, displays the time expanded waveform of the lung sound, and computes the fast Fourier transform and short-time Fourier transform to present the power spectrum and spectrogram respectively. These parameters are transmitted synchronously to the remote station using the Internet for online monitoring of the patient.

New clinical diagnostic procedures for cystic fibrosis in Europe.

In the majority of cases, there is no difficulty in diagnosing Cystic Fibrosis (CF). However, there may be wide variation in signs and symptoms between individuals which encourage the scientific community to constantly improve the diagnostic tests available and develop better methods to come to a final diagnosis in patients with milder phenotypes. This paper is the result of discussions held at meetings of the European Cystic Fibrosis Society Diagnostic Network supported by EuroCareCF. CFTR bioassays in the nasal epithelium (nasal potential difference measurements) and the rectal mucosa (intestinal current measurements) are discussed in detail including efforts to standardize the techniques across Europe. New approaches to evaluate the sweat gland, future of genetic testing and methods on the horizon like CFTR expression in human leucocytes and erythrocytes are discussed briefly.