Potential biomarkers for diagnosis and disease evaluation of idiopathic pulmonary fibrosis / 中华医学杂志(英文版)

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease characterized by progressive lung fibrogenesis and histological features of usual interstitial pneumonia. IPF has a poor prognosis and presents a spectrum of disease courses ranging from slow evolving disease to rapid deterioration; thus, a differential diagnosis remains challenging. Several biomarkers have been identified to achieve a differential diagnosis; however, comprehensive reviews are lacking. This review summarizes over 100 biomarkers which can be divided into six categories according to their functions: differentially expressed biomarkers in the IPF compared to healthy controls; biomarkers distinguishing IPF from other types of interstitial lung disease; biomarkers differentiating acute exacerbation of IPF from stable disease; biomarkers predicting disease progression; biomarkers related to disease severity; and biomarkers related to treatment. Specimen used for the diagnosis of IPF included serum, bronchoalveolar lavage fluid, lung tissue, and sputum. IPF-specific biomarkers are of great clinical value for the differential diagnosis of IPF. Currently, the physiological measurements used to evaluate the occurrence of acute exacerbation, disease progression, and disease severity have limitations. Combining physiological measurements with biomarkers may increase the accuracy and sensitivity of diagnosis and disease evaluation of IPF. Most biomarkers described in this review are not routinely used in clinical practice. Future large-scale multicenter studies are required to design and validate suitable biomarker panels that have diagnostic utility for IPF.

Prevalencia y letalidad de enfermedades pulmonares intersticiales en la Región de Los Ríos, Chile / Prevalence and lethality of interstitial pulmonary diseases in the Region of Los Ríos, Chile

BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) is a subtype of interstitial lung disease (ILD) that significantly affects people's quality of life, whose prevalence and mortality has increased. AIM: To determine the prevalence and lethality of ILD in the Los Ríos Region between the years 2018 and 2019. MATERIAL AND METHODS: Review of a database of all patients attended at an outpatient clinic of the regional hospital. Those patients with the diagnosis of ILD and seen between 2018 and 2019 were selected. Mortality and its causes were verified with death certificates. RESULTS: In the study period, 339 cases with ILD, aged 71 ± 10 years (64% women) were identified. The calculated ILD prevalence was 84 cases per 100,000 inhabitants, with a higher predominancy in Futrono and Paillaco communes. IPF and Connective Tissue Diseases were the predominant subtypes. Overall lethality was 18%, with more deaths among patients with IPF (n = 31). CONCLUSIONS: In the Los Ríos Region, ILDs have a relevant prevalence and lethality.

Clinical evaluation on idiopathic pulmonary fibrosis / 中南大学学报(医学版)

Idiopathic pulmonary fibrosis (IPF) is a chronic fatal pulmonary disease characterized by complex illness condition. There is no effective treatment at present except lung transplantation. The comprehensive evaluation is helpful for the management of patients with IPF in hierarchical stages. Therefore, it is very important to evaluate IPF by various independent factors. At present, the commonly used methods for clinical evaluation on IPF include assessment of health-related quality of life, assessment of physiological function, assessment of imaging, assessment of laboratory examination, and multi-dimensional assessment system. However, there are different advantages and disadvantages on diverse evaluation methods for the evaluation of IPF.

Transplante pulmonar em pacientes com fibrose pulmonar familiar / Lung transplant in patients with familial pulmonary fibrosis

RESUMO Objetivo A fibrose pulmonar familiar (FPF) é definida como uma doença pulmonar intersticial idiopática que afeta dois ou mais membros da mesma família. Nesses pacientes, os resultados têm sido insatisfatórios, apresentando alto risco de morte e disfunção crônica do enxerto pulmonar (CLAD) após o transplante de pulmão. O objetivo do presente estudo foi comparar o resultado de curto e longo prazo do transplante de pulmão em pacientes com FPF e pacientes transplantados por outras doenças pulmonares intersticiais. Métodos Foram coletados retrospectivamente dados clínicos pré e pós-transplante de 83 pacientes com fibrose pulmonar submetidos a transplante de pulmão. Os pacientes foram divididos em aqueles com fibrose pulmonar familiar (n = 9, grupo FPF) e aqueles com fibrose pulmonar não familiar (n = 74, grupo controle). Resultados O grupo FPF foi composto de quatro mulheres e cinco homens, sendo 44,5% ex-fumantes. A maioria apresentou tomografia computadorizada e evidência patológica de pneumonia intersticial usual. Os pacientes com FPF tiveram níveis significativamente menores de hemoglobina e hematócrito. Não foram observadas outras diferenças nas características pré e pós-transplante em relação ao grupo controle. A evolução clínica pós-operatória foi semelhante nos dois grupos. Não foi constatada diferença significativa na sobrevida de um ano livre de CLAD e na sobrevida em geral. Conclusão A evolução pós-transplante de pacientes com FPF foi semelhante à dos pacientes com fibrose pulmonar não familiar, embora mais pacientes com FPF tivessem anemia pré-transplante. O resultado a curto e longo prazo foi comparável em ambos os grupos. O transplante de pulmão provou ser uma opção válida para pacientes com FPF, assim como para pacientes com outros tipos de fibrose pulmonar.

ABSTRACT Objective Familial pulmonary fibrosis (FPF) is defined as an idiopathic interstitial lung disease affecting two or more members of the same family; poor outcome with high risk of death and chronic lung allograft dysfunction (CLAD) after lung transplant has been reported in these patients. The present study aimed to compare the short- and long-term outcome of lung transplants in patients with FPF and patients transplanted because of other interstitial lung diseases. Method Clinical pre- and post-transplant data from 83 consecutive patients with pulmonary fibrosis who underwent lung transplant at our centre were collected retrospectively. Patients were divided into those with familial (n=9 FPF group) and those with non-familial pulmonary fibrosis (n=74 controls). Results The FPF group was composed of 4 females and 5 males; 44.5% were ex-smokers. The majority presented their CT scan and pathology evidence of usual interstitial pneumonia. Patients with FPF had significantly lower pre-transplant levels of haemoglobin and haematocrit. No other differences in pre- and post-transplant characteristics were observed concerning controls. The clinical post-operative course was similar in the two groups. No significant difference in one-year CLAD-free survival and overall survival was observed. Conclusion The post-transplant course of patients with FPF was similar to patients with non-familial pulmonary fibrosis, although more patients with FPF had pre-transplant anaemia. Short- and long-term outcome was comparable in both groups. Lung transplant proved to be a valid option for patients with FPF as it was for patients with other types of pulmonary fibrosis.

¿Cómo se sospecha una fibrosis pulmonar idiopática en la atención primaria de salud? / How is idiopathic pulmonary fibrosis suspected in primary health care?

La fibrosis pulmonar idiopática (FPI) es una enfermedad pulmonar de sintomatología inespecífica. La tos crónica y disnea progresivas en adultos mayores fumadores o exfumadores son los síntomas y características clínicas más frecuentes. Por lo tanto, es usual que el diagnóstico sea tardío. La atención primaria constituye el primer contacto del paciente con el sistema de salud. Por esto es necesaria la entrega de toda la información posible a los médicos, enfermeras y kinesiólogos que atienden adultos con problemas respiratorios, ya que sólo se diagnosticará FPI si se piensa en FPI.

Idiopathic pulmonary fibrosis (IPF) is a lung disease of nonspecific symptomatology. Progressive chronic cough and dyspnea in older smokers or ex-smokers are the most frequent symptoms and clinical features. Therefore, it is usual for the diagnosis to be late. Primary care constitutes the patient's first contact with the health system. Therefore, it is necessary to deliver all possible information to physicians, nurses and physical therapists who care for adults with respiratory problems, since IPF will only be diagnosed if FPI is considered.

Diagnóstico diferencial de la fibrosis pulmonar idiopática / Differential diagnosis of idiopathic pulmonary fibrosis

Para el diagnóstico certero de fibrosis pulmonar idiopática (FPI) es de vital importancia la presencia de un patrón tomográfico definitivo de neumonía intersticial usual (NIU), en un contexto clínico adecuado. El interrogatorio dirigido, el uso de cuestionarios validados, una evaluación reumatológica acuciosa y exámenes complementarios son importantes para descartar causas secundarias de fibrosis pulmonar como neumonitis por hipersensibilidad (NHS), enfermedades del tejido conectivo (ETC), toxicidad por drogas y algunas neumoconiosis que pueden imitar el patrón radiológico y muchas veces dificultar un diagnóstico adecuado de FPI.

For the accurate diagnosis of idiopathic pulmonary fibrosis (IPF), the presence of a definitive tomographic pattern of usual interstitial pneumonia (UIP) is of vital importance, in an appropriate clinical context. Targeted interrogation, the use of valid questionnaires, an acute rheumatologic evaluation and complementary examinations are important to rule out secondary causes such as hypersensitivity pneumonitis (HP), connective tissue diseases (CTD), drug toxicity and some pneumoconiosis that can mimic the radiological pattern and often hinder a clear diagnosis of IPF.

Evaluación reumatológica del paciente con enfermedad pulmonar intersticial / Rheumatological evaluation of the patient with interstitial lung disease

Las Enfermedades del Tejido Conectivo (ETC) comprenden un grupo heterogéneo de patologías multisistémicas de origen autoinmune. La Enfermedad pulmonar intersticial (EPI) asociada a ETC (EPI-ETC) es frecuente y empeora el pronóstico de la ETC. Las EPI-ETC representan aproximadamente 15-30% del total las EPI y se presentan con las mismas formas histopatológicas y radiológicas descritas para las EPI idiopáticas. Esto pone en evidencia la importancia de incorporar en forma rutinaria a reumatología en el comité multidisciplinario para el diagnóstico y manejo de las EPI.

Connective Tissue Diseases (CTD) comprise a heterogeneous group of multisystemic pathologies of autoimmune origin. Interstitial lung disease (ILD) associated with CTD (CTD-ILD) is common and and it worsens the prognosis of CTD. CTD-ILD represent approximately 15-30% of the universe of ILD and have the same histopathological and radiological forms described for idiopathic ILD. This highlights the importance of routinely incorporate a rheumatologist into the multidisciplinary committee for the diagnosis and management of ILD.

Discusión multidisciplinaria para el diagnóstico de la fibrosis pulmonar idiopática / Multidisciplinary discussion for the diagnosis of idiopathic pulmonary fibrosis

Antes de la publicación de la clasificación ATS/ERS 2002 de las neumonías intersticiales idiopáticas (NII), la evaluación histopatológica se consideraba la referencia de oro para el diagnóstico de las enfermedades pulmonares intersticiales (EPI). Sin embargo, varios estudios posteriores mostraron que las concordancias interobservador entre anatomopatólogos expertos torácicos eran sorprendentemente pobres ya que las apariencias histopatológicas pueden superponerse entre entidades distintas. Por lo anterior, se hace necesario un nuevo sistema diagnóstico que sirva de patrón de oro en pacientes con EPI. Es así como nace el concepto de discusión multidisciplinaria, para referirse a una reunión que permita la integración de todos los datos clínicos, radiológicos y patológicos disponibles para un paciente individual y así poder determinar un diagnóstico de trabajo.

Prior to the publication of the 2002 ATS / ERS classification of idiopathic interstitial pneumonias (IIP), the histopathological evaluation was considered the gold standard for the diagnosis of interstitial lung diseases (ILD). However, several subsequent studies showed that interobserver concordances between expert lung pathologists were surprisingly poor, since histopathological appearances may overlap between different entities. Therefore, a new diagnostic system that serves as a gold standard in patients with ILD became necessary. This is how the concept of multidisciplinary discussion was born, to refer to a meeting that allows the integration of all the clinical, radiological and pathological data available for an individual patient and thus be able to determine a working diagnosis.

¿Cómo realizar el seguimiento de los pacientes con fibrosis pulmonar idiopática? / How to monitor patients with idiopathic pulmonary fibrosis?

La fibrosis pulmonar idiopática (FPI) se ha clasificado en enfermedad leve o temprana-moderada-severa o Avanzada, sin puntos de corte en parámetros clínicos, funcionales o imagenológicos. No existe aún consenso en cual es el principal parámetro que se debe medir. Si bien las variables funcionales como la capacidad vital forzada (CVF), capacidad de difusión de monóxido de carbono (DLCO) y test de caminata de 6 minutos se han utilizado de forma rutinaria en la practica clínica y en los principales estudios clínicos de tratamiento muchas veces no son representativos de la evolución clínica. Por lo anterior se han desarrollado, índices o puntajes compuestos como la escala GAP (Gender-Age-Physiology) que podrían ser útiles en el seguimiento de los pacientes.

Idiopathic pulmonary fibrosis (IPF) has been classified as mild or early - moderate - severe or advanced disease, with no cut-off points in clinical, functional or imaging parameters. There is no consensus yet on which is the main parameter to be measured although the functional variables such as forced vital capacity (FVC), carbon monoxide diffusion capacity (DLCO) and 6-minute walk test, have been routinely used in clinical practice and in the main clinical studies of treatment, are often not representative of the clinical evolution. Therefore, composite indices or scores such as the GAP (Gender-Age-Physiology) scale have been developed that could be useful in the follow-up of patients.

Enfermedades pulmonares intersticiales. Una perspectiva actual / Update on interstitial lung diseases

Interstitial lung diseases are a broad, diverse, challenging group of diseases, most of them chronic whose prognosis is not good. In the last two decades there have been considerable advances in the knowledge of the epidemiology, pathological and genetic bases and treatment of several of these diseases. This article summarizes and presents updated information about their classification, new knowledge on genetics and treatments in idiopathic pulmonary fibrosis, advances in the diagnosis and management of hypersensitivity pneumonitis and a review of the broad spectrum of interstitial diseases associated with connective tissue diseases. Several clinical trials are currently underway whose results will be available in the coming years and will provide more information and tools to improve the treatment of these patients.

Clinical and pathological aspects of idiopathic pulmonary fibrosis in cats / Aspectos clínicos e patológicos em felinos com fibrose pulmonar idiopática

Interstitial lung diseases are a group of diffuse parenchymal lung diseases that include interstitial lung fibrosis. The aim of this study is to characterize the clinical and pathological findings of idiopathic pulmonary fibrosis in three cats and to investigate possible etiological agents through bacteriological and mycological exams and immunohistochemistry. All three cats were female and aged from 10 to 14 years old, they presented with a clinical history of weight loss and dyspnea. The radiographic changes were similar in all cats and included increased pulmonary radiopacity with a mixed bronchointerstitial pattern progressing to an alveolar pattern. Two cats died during lung biopsy procedures. At necropsy, the lesions were limited to the pulmonary parenchyma and were firm, hypocrepitant with a multinodular appearance on the pleural surface; they failed to completely collapse when the thorax was opened. In the pleural region, there were multifocal star-shaped scarring lesions, with parenchymal retraction. Microscopically, all three cats had multifocal-to-coalescing fibrosis, type II pneumocyte hyperplasia, hypertrophy or hyperplasia of the smooth muscle tissue of terminal bronchioles and an accumulation of macrophages within the alveolar spaces. There was no growth on bacteriological or mycological cultures, and the immunohistochemical evaluations for the presence of viral etiological agents (FIV, FeLV, FCoV, FCV and FHV-1) were also negative.(AU)

As enfermidades pulmonares intersticiais são um grupo de doenças difusas do parênquima pulmonar, nas quais a fibrose pulmonar está incluída. O objetivo deste trabalho é caracterizar os achados clínicos e patológicos da fibrose pulmonar idiopática em três gatas, e avaliar possíveis agentes etiológicos através dos exames bacteriológicos, micológicos e imuno-histoquímicos. As três gatas tinham entre 10 e 14 anos de idade e histórico clínico de emagrecimento e dispneia. As alterações radiográficas observadas foram similares, com aumento de radiopacidade difuso dos campos pulmonares de padrão misto broncointersticial e eventualmente alveolar. Dois felinos morreram durante procedimento de biópsia pulmonar. No exame de necropsia as lesões eram exclusivas no parênquima pulmonar os quais estavam firmes, hipocreptantes, com aspecto levemente multinodular em superfície pleural e não colapsaram após a abertura da cavidade torácica. Em região pleural havia lesões cicatriciais de aspecto estrelar multifocais, com retração do parênquima. Microscopicamente, todos os gatos apresentaram fibrose multifocal a coalescente, hiperplasia dos pneumócitos do tipo II e hiperplasia e hipertrofia do músculo liso de bronquíolos terminais e acúmulo de macrófagos no interior de espaços alveolares. Não houve crescimento nas culturas bacteriana e micológica, e os exames de imuno-histoquímica para avaliação de possíveis agentes virais (FIV, FeLV, FCoV, FCV e FHV-1) foram negativos em todos os felinos.(AU)

Imaging in idiopathic pulmonary fibrosis: diagnosis and mimics

Idiopathic pulmonary fibrosis is a chronic disease of unknown etiology that usually has a progressive course and is commonly associated with a poor prognosis. The main symptoms of idiopathic pulmonary fibrosis, including progressive dyspnea and dry cough, are often nonspecific. Chest high-resolution computed tomography is the primary modality used in the initial assessment of patients with suspected idiopathic pulmonary fibrosis and may have considerable influence on subsequent management decisions. The main role of computed tomography is to distinguish chronic fibrosing lung diseases with a usual interstitial pneumonia pattern from those presenting with a non-usual interstitial pneumonia pattern, suggesting an alternative diagnosis when possible. A usual interstitial pneumonia pattern on chest tomography is characterized by the presence subpleural and basal predominance, reticular abnormality honeycombing with or without traction bronchiectasis, and the absence of features suggestive of an alternative diagnosis. Idiopathic pulmonary fibrosis can be diagnosed according to clinical and radiological criteria in approximately 66.6% of cases. Confirmation of an idiopathic pulmonary fibrosis diagnosis is challenging, requiring the exclusion of pulmonary fibroses with known causes, such as asbestosis, connective tissue diseases, drug exposure, chronic hypersensitivity pneumonitis, and other forms of idiopathic interstitial pneumonitis. The histopathological hallmark of usual interstitial pneumonia is a heterogeneous appearance, characterized by areas of fibrosis with scarring and honeycombing alternating with areas of less affected or normal parenchyma. The aim of this article was to review the clinical, radiological, and pathological features of idiopathic pulmonary fibrosis and of diseases that might mimic idiopathic pulmonary fibrosis presentation.

Fibrosis pulmonar idiopática en un ecuatoriano adulto de la provincia de Riobamba / Idiopathic lung fibrosis in an adult Ecuadorian from Riobamba province

Se presenta el caso clínico de un paciente de 52 años, expuesto a las cenizas del volcán Tungurahua en erupción, quien acudió a la Consulta de Medicina Familiar Comunitaria del Cantón Guano, provincia ecuatoriana de Riobamba, por presentar cuadro de tos productiva en horario matutino, mucoide de coloración blanco amarillenta, toma del estado general, fiebre vespertina, pérdida de apetito y peso. Según los resultados radiográficos y tomográficos presentó signos sugestivos de tuberculosis pulmonar; los esputos BAAR directos y los cultivos negativos. Los hallazgos anatomopatológicos permitieron confirmar el diagnóstico de fibrosis pulmonar idiopática.

The case report of a 52 years patient is presented, exposed to the ashes of Tungurahua volcano in eruption, who went to the Community Family Medicine Department of the Canton Guano, Ecuadorian province of Riobamba, for presenting productive cough in the morning, mucoid of yellowish white coloration, bad general state, evening fever, appetite and weight loss. According to the radiographic and topographic results, he had suggestive signs of lung tuberculosis; direct BAAR sputa and negative cultures. The pathological findings allowed to confirm the diagnosis of idiopathic lung fibrosis.

Trasplante pulmonar en pacientes portadores de fibrosis pulmonar: caracterización de una cohorte de 87 pacientes / Lung transplantation in 87 patients with idiopathic pulmonary fibrosis

Pulmonary fibrosis is a progressive disease. Lung transplantation is the only effective therapy for a group of patients. Objective: To evaluate results of lung transplantation for fibrosis up to a 5 years of follow up. Methodology: Retrospective review of clinical records of patients subjected to lung transplantation from Clínica Las Condes and Instituto Nacional del Tórax. Demographic data, type of transplant, baseline and post-transplant spirometry and 6 min walked distance (6MWT), early and late complications and long-term survival rate were analyzed. Results: From 1999 to 2015, 87patients with pulmonary fibrosis were transplanted, in average they were 56 years old, 56% were male, 89% of patients were subjected to a single lung transplant. 16% of them were in urgency. Baseline and 1-3-5 years for FVC were 49-73-83 and 78% of the reference values and for 6MWT were 280, 485, 531 and 468 meters respectively. Predominant complications < 1 year post-transplant were: acute rejection (30%) and infections (42%). Complications after 1 year of transplantation were chronic graft dysfunction (DCI) 57% and neoplasms (15%). The main causes of mortality > 1 year were DCI (45%) and neoplasms (11%). The estimated 1, 3 and 5 year survival rate were 84, 71 and 58% respectively. Conclusions: Lung transplantation in patients with pulmonary fibrosis improves their quality of life and survival rate. The monopulmonary technique is efficient in the long term. Acute rejection and infection were the most common early complications and chronic graft dysfunction was the prevalent long-term complication.

La Fibrosis pulmonar es una enfermedad progresiva y el trasplante constituye una terapia efectiva para un grupo de pacientes. Objetivo: Evaluar los resultados del trasplante pulmonar por fibrosis a 5 años. Metodología: Revisión retrospectiva de registros de trasplante pulmonar de la Clínica Las Condes e Instituto Nacional del Tórax. Se analizaron datos demográficos, tipo de trasplante, función pulmonar basal y post-trasplante, complicaciones precoces y tardías y sobrevida a largo plazo. Resultados: Entre 1999 y 2015 ambos centros trasplantaron 87 pacientes por fibrosis pulmonar. Los pacientes tenían una edad promedio de 56 años, 56% eran del género masculino y se usó técnica monopulmonar en 89% de ellos. 16% de los pacientes se encontraba en urgencia. Los resultados espirométricos y la distancia caminada en 6 minutos (T6 min) basales y a 1- 3 - 5 años fueron: CVF 49- 73- 83 y 78% del valor teórico y T6 min fue 280, 485, 531 y 468 metros respectivamente. Complicaciones predominantes < 1 año fueron: rechazo agudo 30% e infecciones 42%. Complicaciones > 1 año fueron: disfunción crónica del injerto (DCI) 57% y neoplasias 15%. Las causas de mortalidad > 1 año fueron DCI 45% y neoplasias 11%. La sobrevida estimada a uno, 3 y 5 años fue 84, 71 y 58% respectivamente. Conclusiones: El trasplante en pacientes con fibrosis pulmonar, permite mejorar la calidad de vida y sobrevida de estos pacientes. La técnica monopulmonar es eficiente a largo plazo. En las complicaciones precoces predominaron el rechazo agudo e infecciones y a largo plazo la DCI.

Update on diagnosis and treatment of idiopathic pulmonary fibrosis / Atualização no diagnóstico e tratamento da fibrose pulmonar idiopática

Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation.

A fibrose pulmonar idiopática é um tipo de pneumonia intersticial crônica fibrosante, de causa desconhecida, associada à piora funcional respiratória progressiva e taxas elevadas de mortalidade. Em anos recentes, o interesse e os conhecimentos sobre essa moléstia têm aumentado substancialmente. O presente artigo de revisão aborda de maneira ampla aspectos relacionados ao diagnóstico e tratamento da doença. Nele são listados os critérios atuais para o diagnóstico e são discutidos as diferentes abordagens terapêuticas agora disponíveis, o tratamento sintomático, a ação de novas drogas eficazes em reduzir o ritmo de deterioração funcional pulmonar e as indicações para transplante pulmonar.

Fibrosis pulmonar idiopática / Idiopathic pulmonary fibrosis

La FPI predomina en el sexo masculino, en edades avanzadas, con tos y/o disnea progresivas. Un 5% se presenta como una forma familiar. La tomografía axial computarizada de tórax, fundamental en el diagnóstico, en al menos un 50% hace innecesaria la biopsia. El diagnóstico es conjunto con clínicos, radiólogos y patólogos. La sobrevivencia media es de tres a cinco años desde el diagnóstico. La historia natural es un deterioro progresivo, pero hay formas rápidas y también pueden aparecer exacerbaciones que ensombrecen el pronóstico. Diversas comorbilidades se han descrito como la hipertensión pulmonar, la asociación con enfisema y el reflujo gastroesofágico. Sólo recientemente aparecen fármacos útiles, que son la Pirfenidona y el Nintedanib. El clásico esquema de prednisona, azatriopina y N-acetil cisteina, se ha demostrado ineficaz. Otros recursos que pueden utilizarse como complementos útiles en la enfermedad son el oxígeno, la rehabilitación, las terapias antirreflujo y el manejo sintomático de la tos.

PF appears mainly in aged males, with progressive cough and dyspnea. In 5% of the cases the disease presents as a familial form. CT scan is key in diagnosis of the disease. In no less than 50% biopsy is unnecessary but diagnosis must be made in conjunction with clinician, radiologist and pathologist. Median survival is 3 to 5 years from diagnosis. Natural history is a progressive deterioration but there are fast evolution cases and exacerbation of the disease that make worse the prognosis. Pulmonary hypertension, the association with emphysema and gastroesophageal reflux has been described as comorbidities of the disease. Last year has been published the positive results of therapeuticall trials with two new drugs, Pirfenidone and Nintedanib. The classical regime for IPF with Prednisone, Azathriopine and Acetylcysteine has been showed as useless. Oxygen, Pulmonary rehabilitation, gastroesophageal reflux and cough management are complementary treatment for the disease.

Validez diagnóstica de los volúmenes pulmonares por pletismografía para el diagnóstico clínico y el seguimiento de la fibrosis pulmonar idiopática / Diagnostic validity of lung volumes by plethysmography for clinical diagnosis and follow-up of idiopathic pulmonary fibrosis

INTRODUCCIÓN: La fibrosis pulmonar idiopática es definida como una forma de neumonía intersticial crónica, progresiva, de causa desconocida, limitada a los pulmones, la cual se presenta principalmente en adultos mayores de 65 años. El diagnóstico de la enfermedad incluye la presencia de un patrón radiológico compatible con neumonía intersticial o hallazgos histopatológicos compatibles con neumonía intersticial usual y la exclusión de: enfermedades ocupacionales, toxicidad por drogas o enfermedades del colágeno que puedan producir enfermedad intersticial. La determinación de los volúmenes pulmonares por pletismografía permite identificar la presencia de trastornos restrictivos y la gravedad de estos en los estados patológicos del pulmón. OBJETIVO: Determinar la validez de la medición de los volúmenes pulmonares por pletismografía como apoyo del proceso diagnóstico de la fibrosis pulmonar idiopática y como medida del seguimiento de la progresión de la enfermedad. MÉTODOS: Dos estudios fueron incluidos, estos evaluaban pacientes con fibrosis pulmonar idiopática en los que se realizaron volúmenes pulmonares por pletismografía y se correlacionaron los datos de CPT con la tasa de sobrevida, la progresión de la enfermedad y las exacerbaciones. Se encontró que la media de sobrevida de estos pacientes es de 36 meses proximadamente y se asocia a bajos niveles de capacidad vital forzada y capacidad pulmonar total entre otros. Adicionalmente que pacientes con una reducción combinada de CPT y CV por debajo de los valores predichos presentan un deterioro restrictivo de la función pulmonar y un reducción de la sobrevida. CONCLUSIONES: La medición de los volúmenes pulmonares es un método complementario a la espirometría para determinar las capacidades y volúmenes pulmonares que ayudan a definir la gravedad de las alteraciones obstructivas, restrictivas o ambas, que está comprometiendo al paciente. La evidencia existente no soporta su efectividad en la confirmación diagnóstica de la fibrosis pulmonar idiopática, para la cual existen otras pruebas de mayor utilidad diagnóstica y tampoco soporta su como método de seguimiento de la progresión de la enfermedad.(AU)