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To develop a joint proposal for screening criteria of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) and vice versa, which serves as a guidelines in patient referral between the Rheumatology and Pneumology departments to early detection of these patients.A systematic literature review was carried out on the risk factors for the development of ILD in RA patients, and for the referral criteria to Rheumatology for suspected early RA. Based on the available evidence, screening criteria were agreed using the Delphi method by a panel of pneumologists and rheumatologists with expertise in these pathologies.Screening criteria for ILD in patients with RA and for the early detection of RA in cases with ILD of unknown etiology have been developed. In both cases, a detection strategy was based on clinical risk factors. Recommendations also included the complementary tests to be carried out in the different clinical scenarios and on the periodicity that screening should be repeated.A selective screening strategy is recommended for the first time in the early diagnosis of patients with ILD-RA. This multidisciplinary proposal aims to solve some common clinical questions and help decision-making, although its usefulness to identify these patients with good sensitivity must be confirmed in a validation study.

To develop multidisciplinary recommendations to improve the management of rheumatoid arthritis-related interstitial lung disease (RA-ILD).Clinical research questions relevant to the objective of the document were identified by a panel of rheumatologists and pneumologists selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network (SIGN) criteria. Specific recommendations were made.Six PICO questions were selected, three of which analysed the safety and effectiveness of glucocorticoids, classical synthetic disease-modifying anti-rheumatic drugs (DMARDs) and other immunosuppressants, biological agents, targeted synthetic DMARDs, and antifibrotic therapies in the treatment of this complication. A total of 12 recommendations were formulated based on the evidence found and/or expert consensus.We present the first official SER-SEPAR document with specific recommendations for RA-ILD management developed to resolve some common clinical questions, reduce clinical healthcare variability, and facilitate decision-making for patients.

To develop multidisciplinary recommendations to improve the management of rheumatoid arthritis-related interstitial lung disease (RA-ILD).Clinical research questions relevant to the objective of the document were identified by a panel of rheumatologists and pneumologists selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network (SIGN) criteria. Specific recommendations were made.Six PICO questions were selected, three of which analysed the incidence and prevalence of RA-ILD, associated risk factors, and predictors of progression and mortality. A total of 6 specific recommendations on these topics, structured by question, were formulated based on the evidence found and/or expert consensus.We present the first official SER-SEPAR document with specific recommendations for RA-ILD management developed to resolve some common clinical questions and facilitate decision-making for patients.

Aims: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease that includes acute exacerbations (AE-IPF). AE-IPF lead to a significant decline in lung function, higher mortality rates and use of health resources. This study aims to estimate the economic impact of AE-IPF in Spain. Methods: Prospective, observational, multicentric real world data study of patient records with confirmed IPF, over 12 months of follow-up in 28 centres. Annual IPF-associated costs included direct costs (e.g. primary and secondary care visits, outpatient visits, emergency visits, AE-IPF related costs) and indirect costs (days off work and informal caregiver). Results: A total of 204 consecutive IPF patients were included. Patients were classified according to whether they had suffered at least 1 AE-IPF during the study (22; 10.8 %) or not (166; 81.4%). Patients who suffered ≥1 AE-IPF had a significantly greater use of health resources (e.g. specialized visits, emergency visits, hospitalisation, test). Statistically significant differences were observed regarding the mean (SD) total annual IPF-related cost for patients who experienced ≥1 AE-IPF compared with those without exacerbations: € 31763 (€ 19766.75) vs € 22978 (€ 14831.92); p=0.0399, mainly driven by increased direct health IPF-related costs. Over 12 months, FVC % predicted substantially declined in patients who suffered ≥1 AE-IPF compared to those without exacerbations (relative FVC decline 10.14% vs 2.21%; p= NS, probably due to small sample size). Conclusion: Preventing or avoiding AE-IPF is important in order to reduce the use of health resources and decline of patient9s FVC, thus decreasing the economic cost of IPF.

Introduction: Rheumatoid arthritis (RA) is a systemic autoimmune disease whose main extra-articular organ affected is the lung, sometimes in the form of diffuse interstitial lung disease (ILD) and conditions the prognosis. Methods: A multicenter, observational, descriptive and cross-sectional study of consecutive patients diagnosed with RA-ILD between 2013 and 2018. Demographic, analytical, respiratory functional and evolution characteristics were analyzed to evaluate the predictors of progression and mortality. Results: 106 patients were included. The mean age was 70.2±19.8 years and 51.9% of the patients presented UIP pattern. In the multivariate Cox analysis the age (HR 1.33, 95% CI 1.09-1.62, p=0.0045), the DLCO (%) (HR 0.85, 95% CI 0.73-0.98, p=0.0246), and the final SatO2 (%) in the 6MWT (HR 0.62, 95% CI 0.39-0.99, p=0.0465) were independent predictor variables of mortality, as well as the GAP index (HR 4.65, 95% CI 1.59 - 13.54, p=0.0051) and the CPI index (HR 1.12, 95% CI 1.03 - 1.22, p=0.0092). Also highlight that the diagnostic delay was an independent predictor of mortality (HR 1.11, CI 1.01-1.23, p = 0.035). Conclusions: This is the first study where it is objectified that the diagnostic delay in RA-ILD is associated with an increased mortality just like happens in IPF.

Aims and Objective: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease. The course of IPF includes acute exacerbations (AE-IPF) which lead to a significant decline in lung function and a mortality rate of 85%, with advanced IPF patients at higher risk. This study aims to describe the clinical management of AE-IPF in Spain and their impact on the use of health resources. Methods: Prospective, observational, multicentric real world data study of patient records with confirmed IPF, over 12 months of follow-up in 28 centres. Results: A total of 204 consecutive IPF patients were included: 77% male, average age (SD) 70.8 (7.6) years. Patients were classified by their forced vital capacity (FVC) at baseline (FVC 80% 14.7%). 22 (10.8%) patients suffered 28 AE-IPF (mean (SD): 1.27 (0.63) AE-IPF/patient; mean duration: 16.50 (18.38) days) with higher frequency in patients with advanced IPF: 27.3%, 8.6% and 10.0%, respectively. 75% of the AE-IPF required emergency room visits, 46.4% any specialized care visit and hospitalisation was reported in 75% (mean duration: 8.48 (5.90) days). Management of AE-IPF required X-ray (93.8%), arterial blood gas testing (75%), laboratory tests (57.1%), computed tomography (43.8%) and respiratory virus screening (18.8%). 50% of AE-IPF received pharmacological treatment (32.1% systemic corticosteroids, 7.1% antibiotics for systemic use). Oxygen therapy was needed in 21.4% of AE-IPF. Conclusions: Patients with advanced IPF are at higher risk than those with less advanced disease of suffering AE-IPF, which are associated with a substantial use of health resources.

Aim & objective: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease associated with dyspnoea, cough and impaired quality of life affecting 7700 patients in Spain. Antifibrotic drugs have been shown to reduce the decline in lung function in patients with IPF. Our aim was to describe patient characteristics and impact of baseline lung function on the clinical management of IPF. Method: Prospective, observational, multicentric real-world data study of patient records with confirmed IPF, over 12 months of follow-up in 28 centres. Results: 204 consecutive patients with IPF were included: 77% male, average age (SD) 70.8 (7.6) years. At baseline, forced vital capacity (FVC) was 80% in 10.8%, 74.5% and 14.7% of patients, antifibrotics were administered in 82.8% of patients and non-pharmacological treatments in 20.1%. On average, patients attended 4.7, 2.98 and 2.4 primary care visits and 4.9, 3.2 and 2.9 pulmonologist visits during the study, by baseline FVC. Hospitalisations were more frequent in lower baseline FVC patients (20%, 6.4%, 7.4%, respectively). At 12 months, absolute lung capacity was decreased by 2.14% (95% CI: -3.90 to -0.37) relative to baseline. Absolute FVC decline in the total sample was >10% in 17.1% of patients, from 10% to 5% in 22.1% and 10% decline in 0%, 18.9% and 15% of patients, respectively. Conclusion: Healthcare resource use in patients with preserved FVC is lower. Hence, it would be advisable to start IPF treatment as soon as possible to delay progression and control healthcare costs.

Resumen Antecedentes y objetivo La mayoria de los estudios sobre enfermedad pulmonar obstructiva cronica (EPOC) excluyen a pacientes octogenarios, por lo que la enfermedad no esta bien caracterizada en esta franja etaria. El objetivo de este trabajo es estudiar las caracteristicas clinicas de los pacientes octogenarios con EPOC y la utilidad de los indices pronosticos mas empleados en la enfermedad en esta edad. Pacientes y metodo Estudio retrospectivo de pacientes consecutivos atendidos en una consulta entre 2009 y 2017. Se estudiaron parametros de funcion pulmonar, distribucion de fenotipos clinicos, historial de ingresos, mortalidad, comorbilidades y la utilidad de los indices de Charlson, BODEX, COTE y CODEX para predecir mortalidad. Resultados La muestra consto de 698 sujetos, 82 (11,7%) ≥ 80 anos. El seguimiento medio fue de 47,9 ± 21,8 meses. En los pacientes octogenarios la gravedad de la EPOC medida por el FEV1% o por el indice BODEX fue similar a la de sujetos mas jovenes, pero la disnea fue mayor. En estos pacientes fue mas frecuente el fenotipo bronquitis cronica y el exacerbador frecuente, y menos prevalente el enfisema. Los octogenarios tenian mas prevalencia de comorbilidad cardiovascular y de insuficiencia renal. Los ingresos hospitalarios y la mortalidad fueron mayores en los octogenarios. La mayoria de los indices pronosticos tuvieron utilidad para predecir mortalidad en los pacientes ancianos, y en ambos grupos etarios el indice CODEX fue el mejor a este respecto. Conclusion Los pacientes octogenarios con EPOC tienen unas caracteristicas diferenciales que podrian implicar un abordaje terapeutico diferente. Los indices pronosticos son utiles para predecir mortalidad en esta poblacion.

Resumen Introduccion La enfermedad del pulmon de granjero (EPG) es una forma frecuente de neumonitis por hipersensibilidad, posiblemente infradiagnosticada. El objetivo de este estudio es describir las caracteristicas clinicas, la evolucion y los factores que influyen en el pronostico de los pacientes con EPG. Pacientes y metodos Estudio retrospectivo que incluyo a todos los pacientes diagnosticados de EPG por haber presentado una exposicion ambiental de riesgo, una clinica, una funcion pulmonar y una radiologia compatible, en los que se demostro sensibilizacion antigenica y/o una anatomia patologica concordante. Resultados Se incluyeron 75 pacientes con EPG, 50 con forma aguda o subaguda (FAS) y 25 con forma cronica (FC). Un 44% de los pacientes (n = 33) fue diagnosticado durante los meses de marzo y abril, especialmente aquellos con FAS en comparacion con las FC (52 vs. 28%; p = 0,0018). En las FAS la DLco presento una mejoria durante el seguimiento (p = 0,047). La determinacion de anticuerpos IgG especificos fue positiva en 39 pacientes (78%) con FAS (44% de ellos frente a Aspergillus ) y en 12 con FC (48%). La realizacion de la evitacion antigenica (OR 9,26, IC 95% 1,3-66,7, p = 0,026) y la administracion de tratamiento inmunodepresor (OR 16,13, IC 95% 1,26-200, p = 0,033) fueron los factores predictores de mejor evolucion de la enfermedad. Conclusiones La EPG presenta un inicio con predominio estacional en nuestro medio. Las FC presentan habitualmente determinacion de anticuerpos IgG especificos negativos, a diferencia de las FAS, donde los anticuerpos frente Aspergillus son las mas frecuentes. La evitacion antigenica y el tratamiento inmunodepresor son posibles predictores de mejor evolucion de la enfermedad.

Introduction Farmer's lung disease (FLD) is a common form of hypersensitivity pneumonitis possibly underdiagnosed in our midst. The aim of this study was to describe clinical characteristics, evolution and factors that influence the prognosis of patients with FLD. Patients and methods A retrospective study that included all patients diagnosed with FLD presenting an environmental exposure risk, a clinic, lung function and a compatible radiology, in which antigen sensitization was demonstrated and/or a concordant pathology. Results We selected 75 patients with FLD, 50 with acute or subacute form (ASF) and 25 with chronic form (CF). Forty-four percent of patients (n = 33) were diagnosed during the months of March and April, especially those with ASF compared to CF (52 vs. 28%; p = 0.0018). In the ASF group, DLco showed an improvement during follow-up ( p = 0.047). The determination of specific IgG antibodies was positive in 39 patients (78%) with ASF (44% of them against Aspergillus ) and CF 12 (48%). The realization of antigenic avoidance (OR 9.26, 95% CI 1.3–66.7, p = 0.026) and the administration of immunosuppressive therapy (OR 16.13, 95% CI 1.26–200, p = 0.033) were predictors of better disease progression. Conclusions FLD is predominantly seasonal in our environment. CF usually has a negative specific IgG antibodies unlike ASF, where antibodies against Aspergillus are the most common. The realization of antigenic avoidance and immunosuppressive treatment are possible predictors of better disease progression.

Little is known about survival and prognostic factors in chronic obstructive pulmonary disease (COPD) due to biomass smoke exposure (BS-COPD). (1) To determine the value of two indices of COPD severity: BODEx (body mass index, obstruction, dyspnea, and previous severe exacerbations) and Global Initiative for Chronic Obstructive Lung Disease (GOLD) categories system (ABCD) to predict all-cause mortality in BS-COPD, compared with COPD due to tobacco (T-COPD); (2) to verify the usefulness of 2 comorbidity indices, Charlson index and COTE (COPD comorbidity index); and (3) to put side by side the value of these indices. 612 consecutive COPD patients were retrospectively studied. Prognostic factors were evaluated taking into account the exposure to biomass or tobacco smoke. The relative predictive values of the prognostic indices were compared using receiver-operating characteristic analysis. Mortality in the BS-COPD and T-COPD groups was not significantly different, when sex was taken into account. BODEx, Charlson and COTE indices—but not type of exposure—predicted mortality in multivariate analysis. The value of the C-statistic for the BODEx index was not different than that of GOLD ABCD for BS-COPD, but was higher for T-COPD. The discriminatory value of the Charlson index was higher than that of COTE for BS-COPD, but no significant differences were found for T-COPD. Multidimensional indices of COPD severity and comorbidity predict all-cause mortality in BS-COPD. The behavior of the different indices is different for BS-COPD and T-COPD.

Background and objective Most studies on chronic obstructive pulmonary disease (COPD) exclude octogenarian patients. Therefore, the disease is not well characterized in this age group. The objective of this study is to analyze the clinical characteristics of octogenarian patients with COPD and the usefulness of the prognostic indexes used most frequently in this age group. Patients and method Retrospective study of consecutive patients seen at a clinic between 2009 and 2017. The following variables were analyzed: lung function parameters, distribution of clinical phenotypes, income history, mortality, comorbidities and usefulness of the Charlson, BODEX, COTE and CODEX indexes to predict mortality. Results The sample comprised 698 patients, 82 aged (11.7%) ≥ 80 years old. Mean follow-up time was 47.9 ± 21.8 months. In octogenarian patients, the severity of the COPD, assessed by means of the FEV1% or BODEX index, was similar to that of younger patients, but dyspnea was worse in the elderly group. In these patients, the chronic bronchitis and frequent exacerbator phenotypes were the most frequent, whilst the emphysema phenotype was the least common. Octogenarians had a greater prevalence of cardiovascular comorbidities and renal diseases. Moreover, hospital admissions were more frequent and mortality was higher in these elderly patients. Most prognostic indexes were useful in predicting mortality in elderly patients. CODEX was the most useful index to predict mortality, both in octogenarian and younger patients. Conclusion Octogenarian patients with COPD have differential characteristics which could imply the need for different therapeutic approaches. Prognostic indexes are useful for predicting mortality in this population.

This report describes the general characteristics, objectives and organizational aspects of the registries of rare respiratory diseases included in the National Registry of Rare Diseases of the Research Institute for Rare Diseases (ISCIII), in order to publicize their existence and encourage the participation of professionals. Information is collected on the following conditions: alpha-1 antitrypsin deficiency, idiopathic tracheal stenosis, adult pulmonary Langerhans' cell histiocytosis, lymphangioleiomyomatosis, alveolar proteinosis, and sarcoidosis.

Resumen En el presente trabajo se describen las caracteristicas generales, objetivos y aspectos organizativos de los registros de enfermedades respiratorias minoritarias integrados en el Registro Nacional de Enfermedades Raras del Instituto de Investigacion de Enfermedades Raras (IIER), con el objetivo de dar a conocer su existencia y fomentar la participacion de los profesionales. Se recoge informacion sobre registros de las siguientes enfermedades: deficit de alfa-1 antitripsina, estenosis traqueal idiopatica, histiocitosis pulmonar de celulas de Langerhans del adulto, linfangioleiomiomatosis, proteinosis alveolar y sarcoidosis.

Resumen Introduccion La exposicion al humo de biomasa es un factor de riesgo para enfermedad pulmonar obstructiva cronica (EPOC). Se ignora si la EPOC por biomasa y por tabaco tienen caracteristicas diferentes. Objetivo Buscar diferencias clinicas entre ambos tipos de enfermedad. Metodos Estudio observacional retrospectivo de 499 pacientes diagnosticados de EPOC por biomasa o por tabaco. Se compararon ambos grupos respecto a variables clinicas. Resultados Ciento veintidos sujetos (24,4%) fueron clasificados en el grupo de biomasa y 377 (75,5%) en el de tabaco. El porcentaje de varones fue mas alto en el grupo de tabaco (91,2% vs 41,8%, p 1 % fueron superiores en el grupo de biomasa (29,4 ± 5,7 vs 28,0 ± 5,1; p = 0,01 y 55,6 ± 15,6 vs 47,1 ± 17,1; p Conclusion Existen diferencias clinicas entre la EPOC por humo de biomasa y por tabaco, aunque podrian ser atribuibles en parte a desigualdades de sexo entre ambos grupos.

Resumen Objetivo Analizar el significado de la presencia de linfocitos en las muestras de puncion transbronquial aspirativa (PTA) de adenopatias mediastinicas (AM) en pacientes con sospecha de cancer de pulmon (CP). Metodos Estudio observacional retrospectivo que evalua el valor predictivo negativo (VPN) de las muestras de PTA con evidencia de linfocitos pero sin celulas atipicas. Resultados Se realizaron 266 PTA a 252 pacientes con AM patologicas. En 115 PTA se evidencio la presencia de metastasis ganglionares (43%), y 94 (35%) fueron consideradas como no valorables (ausencia de material citologico evaluable o presencia exclusiva de celulas epiteliales bronquiales). De las 57 muestras de PTA restantes que contenian linfocitos sin atipias (21%), en 15 no se pudo confirmar el diagnostico; en 32 se confirmo mediante tecnicas diagnosticas alternativas y en 10 mediante seguimiento clinico-radiologico. El VPN de las 32 muestras confirmadas con tecnicas diagnosticas alternativas fue del 84% y descendio al 76% cuando se incluyeron las 10 PTA en las que se disponia de seguimiento clinico-radiologico. Conclusiones La presencia de linfocitos sin atipias en la muestra de PTA no excluye la invasion neoplasica del ganglio analizado.

Aim To evaluate the clinical relevance of the presence of lymphocytes in transbronchial needle aspiration (TBNA) samples from pathological mediastinal lymph nodes in patients with suspected lung cancer. Methods Retrospective observational study evaluating the negative predictive value (NPV) of TBNA samples with evidence of lymphocytes but no atypical cells. Results A total of 266 TBNA were performed in 252 patients with pathological mediastinal lymphadenopathy. One hundred and fifteen TBNA samples showed evidence of malignant cells (43%) and 94 (35%) samples were considered inadequate (absence of adequate cytological material or exclusive presence of bronchial epithelial cells). Out of the 57 TBNA samples remaining that contained lymphocytes without atypia (21%), 15 could not be confirmed. Thirty-two TBNA samples were confirmed with alternative diagnostic techniques and 10 were confirmed after clinical and radiological follow-up. The NPV of the 32 samples that were confirmed with alternative diagnostic techniques was 84%, which decreased to 76% when we included the 10 TBNA samples confirmed after clinical and radiological follow-up. Conclusions The presence of lymphocytes without atypia in TBNA samples does not rule out the neoplastic invasion of the lymph node analyzed.

Farmer's lung disease (FLD) is a form of hypersensitivity pneumonitis (HP) caused by inhaling microorganisms from hay or grain stored in conditions of high humidity in the agricultural workplace. It is probably underdiagnosed, especially in northern Spain, where climatic conditions favor the development of this disease. According to previous studies, the most common antigens are usually thermophilic actinomycetes and fungi. The epidemiology of the disease is not well known, and is based on studies conducted by Central European and Asian groups. The clinical presentation may vary, differentiating the chronic (exposure to lower concentrations of the antigen over a longer period time) and the acute forms (after exposure to high concentrations of the antigen). In patients with respiratory symptoms and agricultural occupational exposure, radiological, lung function and/or anatomical pathology findings must be compatible with FLD, bronchoalveolar lavage must show lymphocytosis, and tests must find sensitivity to the antigen. The main treatment is avoidance of the antigen, so it is essential to educate patients on preventive measures. To date, no controlled studies have assessed the role of immunosuppressive therapy in this disease. Corticosteroid treatment has only been shown to accelerate resolution of the acute forms, but there is no evidence that it is effective in preventing disease progression in the long-term or reducing mortality.

Endobronchial metastases (EBM) secondary to extrapulmonary primary tumors are rare. The most common solid malignant tumors associated with EBM are breast, renal and colorectal carcinomas. This case report describes the first documented case of EBM from anal canal carcinoma. This neoplasm constitutes less than 1% of colorectal tumors. The clinical presentation of this entity is variable, being asymptomatic in more than 50% of cases. Generally, EBM are diagnosed in advanced stages and the survival after the diagnosis is poor. It is therefore an exceptional clinical manifestation in which bronchoscopy has an important role in the diagnosis and the treatment.

Resumen Las metastasis endobronquiales (MEB) de tumores primarios extratoracicos son infrecuentes. Los tumores de organos solidos que con mas frecuencia se han asociado a MEB son el carcinoma de mama, el renal y el colorrectal. En esta nota clinica se describe el primer caso documentado de MEB de carcinoma del canal anal, neoplasia que constituye menos del 1% del total de tumores colorrectales. La presentacion clinica de esta entidad es variable y es asintomatica en mas del 50% de los casos. Generalmente las MEB suelen diagnosticarse en fases avanzadas, por lo que la supervivencia es baja. Se trata pues de una manifestacion clinica excepcional en que la broncoscopia tiene un importante papel tanto en el diagnostico como en el tratamiento.

Varon de 56 anos, trabajador en un almacen sin contacto directo con productos toxicos. Sin animales de compania. Ex fumador desde hacia 18 anos con dosis acumulada de 40 paquetes/ano. Antecedentes de hipertension arterial en tratamiento con hidroclorotiazida, diabetes mellitus tipo 2 en tratamiento con metformina y dislipemia en tratamiento con atorvastatina. Consulto por un cuadro clinico caracterizado por disnea progresiva y tos seca de 3 anos de evolucion. La exploracion fisica mostraba escasos crepitantes secos bibasales a la auscultacion respiratoria. El resto de la exploracion fisica no mostro alteraciones. Los analisis sanguineos mostraron PCR de 2 mg/dl, hemoglobina de 14,7 g/dl y hematocrito del 46%. Los marcadores tumorales y los de autoinmunidad estaban dentro de los parametros de la normalidad. La radiografia de torax mostro un indice cardiotoracico ligeramente aumentado, y un patron intersticial bilateral y difuso, de predominio en bases pulmonares. La tomografia computarizada toracica de alta resolucion mostro opacidades en vidrio deslustrado con afectacion de todos los lobulos pulmonares, con patron reticular subpleural de predominio posterior. En los lobulos inferiores se observo engrosamiento de los septos intralobulillares asi como discretas bronquiectasias cilindricas de traccion (fig. 1A y B). La exploracion funcional respiratoria evidencio una disminucion de la capacidad de transferencia para el CO: volumen espiratorio forzado en el primer segundo (FEV1)/capacidad vital forzada (FVC): 88%; FEV1: 3.240 ml (98% del valor de referencia); FVC: 3.660 ml (85% del valor de referencia); volumen residual (RV): 2.010 ml (90% del valor de referencia); capacidad de difusion del monoxido de carbono (DLCO): 19,6 ml/mmHg/min (76% del valor de referencia); KCO: 4,2 min/mmHg (77% del valor de referencia). La gasometria arterial basal mostro pH: 7,42; PaO2: 82 mmHg; PaCO2: 38,7 mmHg; DA-a: 22,3 mmHg. Se realizo un ecocardiograma que fue normal, con una fraccion de eyeccion ventricular izquierda del 67%. La fibrobroncoscopia no mostro alteraciones. El estudio de poblaciones celulares del LBA mostro 94% macrofagos y 6% linfocitos. El analisis microbiologico del lavado broncoalveolar fue negativo. Ante estos hallazgos se decidio realizar una biopsia pulmonar por videotoracoscopia, cuyos hallazgos fueron compatibles con neumonia intersticial usual (NIU), confirmandose la sospecha diagnostica de fibrosis pulmonar idiopatica (FPI) (fig. 2A y B). Durante el siguiente ano el paciente presento un ligero empeoramiento de su disnea habitual, que se correspondia con una discreta disminucion de la FVC (3.310 ml, 78% de los valores de referencia). Se inicio tratamiento con pirfenidona a dosis crecientes hasta 2.403 mg/dia con buena tolerancia clinica. A la segunda semana se evidencio en los analisis sanguineos un moderado aumento de las transaminasas (GOT, GPT), que se normalizo en los siguientes analisis sin modificacion previa de las dosis de pirfenidona. El paciente fue tratado durante 5 anos con pirfenidona. No presento mayor progresion de la clinica y se constato estabilizacion de las pruebas funcionales respiratorias, asi como de la extension de la enfermedad en la tomografia computarizada de alta resolucion.

Resumen La metaplasia peribronquiolar es una lesion histologica caracterizada por fibrosis y proliferacion del epitelio bronquiolar, que afecta a las paredes alveolares peribronquiales y a los bronquiolos terminales. Se considera una reaccion tisular inespecifica secundaria a la accion de factores externos, tales como el humo del tabaco y agentes microbianos. Es un hallazgo histologico frecuente en diversas enfermedades pulmonares intersticiales difusas. Recientemente se han descrito algunos casos de enfermedad pulmonar intersticial difusa con manifestaciones clinicas, radiologicas y de funcion respiratoria propias de las neumonias intersticiales idiopaticas en que la unica alteracion histologica era la presencia de metaplasia peribronquial. En el presente articulo se describen 2 casos de neumopatia intersticial donde el unico hallazgo anatomopatologico era la metaplasia peribronquiolar.

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.