Estudio en fibroblastos humanos del sistema angiotensina en la fisiopatología de la fibrosis pulmonar idiopática / The study of angiotensin system human fibroblasts in the physiopathology of idiopathic pulmonary fibrosis

En el tejido pulmonar de modelos murinos, la angiotensina II induce la proliferación de fibroblastos, su diferenciación a miofibroblastos y la producción de procolágeno tras su unión al receptor I de la angiotensina. Hemos estudiado el comportamiento de fibroblastos pulmonares humanos procedentes de una línea celular comercial tras la estimulación con TGF-β1. Hemos observado que estos fibroblastos, cuando son estimulados, aumentan la expresión de bFGF, colágeno y α-SMA. Tras el bloqueo del receptor de Angiotensina II con Losartan a una concentración de 10 µM y la estimulación con TGF- β1, se produce una disminución, tanto de los niveles de bFGF como de la concentración de colágeno, sin que llegue a alcanzar la significación estadística con respecto a las células no tratadas. En cuanto a la expresión de α-SMA como marcador de transformación a miofibroblastos, no había diferencias entre las células tratadas con TGF-β1 y TGF-β1 más losartán

In murine model lung tissue, angiotensin II induces the proliferation of fibroblasts, their distinction from myofibroblasts and procollagen production after its binding with the type 1 receptor. We have studied the behavior of human lung fibroblasts from a commercial cell line after stimulation with TGF-β1. We observed that those fibroblasts, when stimulated, increased bFGF, collagen and α-SMA expression. After blocking the angiotensin II receptor with losartan at a concentration of 10 µM and stimulation with TGF- β1, there was a decrease in both bFGF levels and collagen concentration, without reaching statistical significance with regard to untreated cells. With regard to α-SMA expression as an indicator of transformation to myofibroblasts, there were no differences between cells treated with TGF-β1 and TGF-β1 with losartan

Producción de factor básico de crecimiento de fibroblastos (Beta-fgf) trasla estimulación de fibroblastos pulmonares humanos in vitro confactor transformador del crecimiento Beta1 (tGf-Beta1). Estudio dosis respuesta / The production of basic fibroblast growth factor following in vitro stimulation of human lung fibroblasts with transforming growth factor Beta1: Dose response Study

Objetivo: Establecer cuál es la dosis de TGF-β1 más adecuada para la estimulación de cultivos de fibroblastos pulmonares humanos y el tiempo necesario de incubación de los mismos para obtener la máxima respuesta. Material y métodos: Diseñamos un estudio de dosis respuesta con TGF-β1 sobre una línea celular de fibroblastos pulmonares humanos. Analizamos la producción de factor básico de crecimiento de fibroblastos (b-FGF) como marcador de estímulo fibrogénico. Para ello se cultivaron fibroblastos humanos procedentes de una línea celular (MRC5) obtenida de la ECCC (European Collection of Cell Culture, UK). Las células se cultivaron en placas de 33cm2, cuando estuvieron confluentes se les estimuló con diferentes dosis de TGF-β1 (Peprotech, USA): 5, 10 ng/ml. Las células se incubaron durante 12, 24, 48 y 72 horas. Se usaron como control células no estimuladas con TGF-β1. Los niveles de factor básico de crecimiento de fibroblastos (b-FGF) se midieron por ELISA (R&D System, Minneapolis, MN). Se analizó la viabilidad celular mediante Azul Trypan a las 24, 48 y 72 horas de la estimulación con TGF-β1. Resultados: La mayor producción de b-FGF se produjo a las 24 horas tras la estimulación con la dosis de 10 ng/ml de TGF-β1, siendo la producción de b-FGF igual a 501 pg/ml. La viabilidad celular tiene su mayor valor a las 48 horas, disminuyendo en horas sucesivas, alcanzando los niveles más bajos a las 72 horas. Conclusiones: Existe un efecto estimulador del TGF-β1 sobre los fibroblastos pulmonares humanos in vitro. Esta acción del TGF-β1 es dosis dependiente y alcanza el nivel máximo de proliferación con la dosis de 10 ng/ml a las 24 horas de su tratamiento (AU)

Objective: To establish the most appropriate dose of TGF-β1 to stimulate human lung fibroblasts cultures and their necessary incubation time to obtain the maximum response. Material and methods: A dose response study was designed with TGF - β1 based on human lung fibroblast cells. The production of basic fibroblast growth factor (b-FGF) was analyzed as a marker for fibrogenic stimulus. Human fibroblasts from a cell line (MRC5) were obtained from the ECCC (European Collection of Cell Culture, UK) and cultivated. Cells were cultured on 33 cm2 plates; once confluent, they were stimulated with various doses of TGF - β1 (Peprotech, USA): 5, 10 ng/ml. The cells were incubated for 12, 24, 48 and 72 hours. Cells not stimulated with TGF - β1 were used as a control. The levels of basic fibroblast growth factor (b-FGF) were measured using ELISA (R&D System, Minneapolis, MN). Cell viability was analyzed using Trypan Blue at 24, 48 and 72 hours following the stimulation with TGF - β1. Results: The greatest production of b-FGF took place 24 hours after the stimulation with the dose of 10ng/ml of TGF - β1, with the production of b-FGF being equal to 501 pg/ml. The cell viability reached its greatest value at the 48 hours, decreasing in the hours thereafter, to reach the lowest levels at 72 hours. Conclusions: TGF-β1 has a stimulating effect on human lugn fibroblasts in vitro. This action of the TGF - β1 is dose dependent and reaches maximum proliferation levels with a dose of 10ng/ml 24 hours following treatment (AU)

Niveles séricos de mesotelina. Valor pronóstico en mesotelioma pleural maligno / Serum levels of mesothelin. Prognosis value in malignant pleural mesothelioma

El mesotelioma pleural maligno (MPM) es un tumor agresivo que surge del epitelio pleural. Se han detectado concentraciones aumentadas de proteínas solubles relacionadas con la mesotelina (SMRP) en suero de pacientes con MPM (..) (AU)

Malignant pleural mesothelioma (MPM) is an aggressive tumour that arises from pleural epithelium. Increased concentrations of soluble mesothelin related proteins (SMRP)

Utility of high-resolution computed tomography and BAL in cryptogenic organizing pneumonia.

BACKGROUND: Cryptogenic organizing pneumonia (COP) is a rare disease, and its diagnosis requires histological confirmation. The objective of our study was to describe the findings of the thoracic high-resolution computed tomography (HR-CT) and bronchoalveolar lavage (BAL) in patients with confirmed COP and evaluate the complementary diagnostic use of BAL and thoracic HR-CT. METHODS: Patients recorded in the registry of interstitial pulmonary diseases between 1991 and 2008 were located and the COP patients selected. RESULTS: We identified 21 patients with histological confirmation of COP. The median age was 58.0 ± 15.9 years, and 61.9% of patients were female. The most frequent thoracic HR-CT profile was patchy infiltrate (71.4%), followed by parenchymatous consolidation (42.9%). The most frequent BAL profile was mixed alveolitis (62%) with lymphocyte predominance, a CD4/CD8 index of 0.4 and foamy macrophages. The effectiveness of transbronchial biopsy was 66.6%. The diagnostic utility of Poletti's BAL criteria gives us a specificity of 88.8%, although the sensitivity obtained was low. The specificity of certain HR-CT profiles is 99%. In addition, we observed a complementary use of the HR-CT and the BAL. CONCLUSIONS: The imaging findings and BAL could be useful for patients with appropriate clinical presentation and for those whose transbronchial biopsy is negative or for whom a confirmatory biopsy cannot be performed.

A haplotype of cyclooxygenase-2 gene is associated with idiopathic pulmonary fibrosis.

BACKGROUND: Cyclooxygenase-2, a key regulatory enzyme in the synthesis of the antifibrotic agent prostaglandin E2, is downregulated in lung tissue from patients with idiopathic pulmonary fibrosis. OBJECTIVE: To investigate the association between COX2.3050 (G --> C), COX2.8473 (C --> T) and COX2.926 (G --> C) single nucleotide polymorphisms (SNP) and the susceptibility to idiopathic pulmonary fibrosis and the progression of the disease. DESIGN: Genetic polymorphisms were analyzed in 121 out of 225 available control subjects and in all of 174 patients with idiopathic pulmonary fibrosis by real time polymerase chain reaction. Logistic regression analysis of covariance and chi-squares test were used for statistical analysis. RESULTS: While analysis of disease development did not find any significant association with single SNP genotype, a haplotype analysis revealed a strong association between the disease development and one haplotype [GC] at loci COX2.3050 and COX2.8473, and suggested a recessive genetic effect of this haplotype. Further analysis concluded that subjects having two copies of [GC] haplotype, or equivalently (GG/CC) genotype at the two SNPs, had an increased risk after adjusting for age and sex. Due to the interaction, this elevated risk increased slowly with age, and the estimated odds ratio (OR) decreased with age from OR = 1.4 at age 30 to OR = 1 at age 74 and OR = 0.96 at age SO. The OR was significantly greater than 1 up to age 66, and not significant for age older than 66. Therefore, the recessive effect of [GC] haplotype increased the risk of IPF of subjects younger than 66 years, but its effect diminished for seniors older than 66. One hundred and forty-nine patients with idiopathic pulmonary fibrosis were followed up for 33.7 +/- 2.1 months. Further analysis of disease progressions, defined by the changes in pulmonary function tests, did not reveal any association with either SNP genotypes or haplotypes. CONCLUSIONS: The carriage of double homozygote (GG/CC) at the SNP loci of COX2.3050 and COX2.8473 polymorphisms may increase the susceptibility to idiopathic pulmonary fibrosis, by approximately 1.4 folds at age 30 and by a smaller fold greater than 1 up to age 66 years, but not the progression of the disease. These findings may help to improve our understanding of idiopathic pulmonary fibrosis pathogenesis and may lead to the development of new therapeutic strategies.

Importancia de los polimorfismos de la ciclooxigenasa-2 en la presentación clínica de la sarcoidosis / Importance of cyclooxigenase-2 polymorphismsin the clinical presentation of sarcoidosis

OBJETIVO: Estudiar la posible relación entre las manifestaciones clínicas de la sarcoidosis y los polimorfismos del gen de laciclooxigenasa-2 (COX-2).MÉTODO: Estudio multicéntrico observacional transversal en el que participaron 7 hospitales de España. Se incluyeron pacientes diagnosticados de sarcoidosis según criterios internacionales. De cada caso se recogió edad, sexo, método diagnóstico, enzima convertidora de angiotensina, pruebas de función respiratoria, estadio radiológico y clínica del paciente en el momento del diagnóstico. Los hallazgos clínicos se agruparon en respiratorios y sistémicos. Los estudios genéticos se realizaron a partir del ADN obtenido de linfocitos de sangre periférica. El ADN se amplificó mediante PCR convencional y los polimorfismos fueron analizados por sondas de hibridación fluorescentes y curvas de disociación. Se determinaron4 variantes alélicas del gen de la COX-2: COX2.5909T>G,COX2.8473T>C, COX2.926G>C y COX2.3050G>C. RESULTADOS: La muestra se compuso de 131 casos de sarcoidosis (63 hombres; edad: 47 ± 15 años), todos con diagnóstico histológico menos 5 casos. El polimorfismo COX2.3050G>C en homocigosis resultó estar significativamente presente entre los pacientes con manifestaciones sistémicas frente al resto de pacientes (4,6% vs 0%;p=0,045). La presencia de manifestaciones sistémicas de la enfermedad estuvo significativamente asociada a los pacientes portadores del alelo C de dicho polimorfismo (34,4% vs. 18,6%; p=0,031; OR:2,3; IC 95%: 1,03-5,12). El resto de polimorfismos estudiados no estuvieron relacionados con la expresión clínica de la enfermedad. CONCLUSIÓN: La presencia de manifestaciones sistémicas parece estar relacionada con los portadores del alelo C del polimorfismoCOX2.3050G>C de la COX-2 (AU)

OBJECTIVE: To study clinical manifestations of sarcoidosis according to cyclooxigenase-2 (COX-2) polymorphisms. METHOD: Observational cross-sectional multicentre trial in which 7 Spanish hospitals participated. Patients diagnosed withs arcoidosis according to international criteria were included. Age, gender, diagnostic method, angiontens in converting enzyme, pulmonary function tests, radiological stage and clinical findings at the moment of the diagnosis were recorded for each case included. Clinical findings were grouped as respiratory or systemic. Genetic studies were performed on DNA extracted from peripheral blood lymphocytes. DNA was amplified by conventional PCR and polymorphisms were studied by Fluorescent Hybridization Probe-Melting Curves. COX-2 polymorphisms genotyped were COX2.5909T>G, COX2.8473 T>C, COX2.926 G>C y COX2.3050 G>C.RESULTS: 131 sarcoidosis patients (63 males, age: 47 ± 15years) were included. All included patients had a histological diagnosis except for 5 patients. COX2.3050G>C homozygote polymorphism resulted to be significantly present in patients with a systemic manifestation of the disease as compared with the rest of the sample(4,6% vs 0%; p = 0,045). Systemic manifestations were significantly associated with allele C carriers of this polymorphism (34.4% vs.18.6%; p = 0.031; OR: 2.3; IC 95%: 1.03 – 5.12). The rest of the studied polymorphisms were not significantly related to the clinical manifestations of the disease. CONCLUSION: Our results suggest that allele C carriers ofCOX2.3050G>C polymorphism are associated with the systemic manifestations of sarcoidosis (AU)

Association of the 3050G>C polymorphism in the cyclooxygenase 2 gene with systemic sarcoidosis.

BACKGROUND: We investigated the potential association between cyclooxygenase-2 (COX-2) gene polymorphisms and clinical manifestations of sarcoidosis. METHODS: This observational cross-sectional study involved seven hospitals in Spain. We diagnosed patients with sarcoidosis according to the International Criteria. The following variables were recorded: age, gender, initial diagnostic methods, serum angiotensin-converting enzyme (ACE) levels, pulmonary function tests, radiological stage, and clinical findings at diagnosis. Manifestations of sarcoidosis were classified as systemic vs. nonsystemic. Genotyping of four COX-2 polymorphisms (COX2.5909T>G, COX2.8473T>C, COX2.926G>C, and COX2.3050G>C) was undertaken on DNA extracted from peripheral blood lymphocytes using fluorescent hybridization probes and melting curves. RESULTS: A total of 131 sarcoid patients (63 males, mean age: 47 +/- 15 years) were studied. One hundred twenty-six of these patients had one or more positive biopsies. The results demonstrated that genotype distribution for the COX2.3050G>C polymorphism was significantly different between patients with systemic sarcoidosis and those with nonsystemic forms (p = 0.046). After adjustment for age, gender, and serum ACE levels, a significant association between the carriage of at least one C allele of the COX2.3050G>C polymorphism and systemic sarcoidosis was observed (odds ratio [OR]: 2.3; 95% confidence interval [CI]: 1.03-5.12, p = 0.031). Other polymorphisms were not associated with either clinical manifestations of the disease or serum ACE levels. CONCLUSIONS: Our results indicate for the first time that the C allele of the COX2.3050G>C polymorphism is associated with systemic sarcoidosis.

Factores relacionados con la infección por Pneumocystis Jirovecii intersticial difusa (EPID) / Factors related to the infection by Pneumocystis Jirovecii in patients with diffuse interstitial pulmonary disease (DIPD)

Objetivo: Hemos realizado un estudio prospectivo para determinar la prevalencia de colonización de Pn. jiroveccii (PnJ) en muestras de LBA en pacientes con EPID y los factores que pueden condicionar esta situación. Material y métodos: Se incluyen 240 pacientes con EPID con una media de edad de 56 años. Se estudió el gen mtLSU rRNA de PnJ mediante PCR anidada. La PCR resultó positiva en el 32% (78 pacientes).Resultados: Sólo el tabaquismo mostró una asociación significativa con la evidencia de colonización. Hematológicamente, la leucocitosis y eosinofilia son parámetros relacionados con ésta. Radiológicamente, en el TACAR no hay ningún hallazgo distintivo y tampoco hay diferencia entre ambos subgrupos (PCR+ vs PCR-) en la distribución de las patologías más frecuentes en nuestro medio: fibrosis pulmonar idiopática, sarcoidosis, bronquiolitis obliterante con neumonía organizativa y conectivopatías. En el estudio de los parámetros del LBA, tampoco se observan diferencias significativas. En el seguimiento, no se han evidenciado complicaciones infecciosas atribuibles a este patógeno. Conclusiones: PnJ coloniza un tercio de la población con EPID sin que se haya definido con claridad ningún factor determinante. En el seguimiento de estos pacientes no se han evidenciado complicaciones infecciosas significativas. Falta por determinar la posible implicación de PnJ en la aceleración del proceso inflamatorio o deterioro funcional en estos pacientes

OObjective: A prospective study was made to determine the prevalence of colonization with Pn. jiroveccii (PnJ) in bronchoalveolar (BAL) samples of patients with DIPD, and the factors that can condition this situation. Material and methods: 240 patients with DIPD were included in the study, with an average age of 56 years. The mtLSU rRNA gene of PnJ was studied by means of nested PCR. The PCR was positive in 32% (78 patients).Results Only tobacco use showed a significant association with the evidence of colonization. Leucocytosis and eosinophilia are parameters related to this phenomenon also. Radiologically, there were no distinctive findings in high-resolution computed tomography (HRCT) nor difference between both sub-groups (PCR+ versus PCR-) in the distribution of the most frequent pathologies in our area: idiopathic pulmonary fibrosis, sarcoidosis, bronchiolitis obliterans with organizing pneumonia and connective tissue diseases. Also, no significant differences were observed in the study of the BAL parameters. Infectious complications attributable to this pathogen have not been demonstrated in the follow-up. Conclusions: PnJ colonises a third of the population with DIPD without any determining factor having been clearly defined so far. Significant infectious complications have not been demonstrated in the follow up of these patients. The possible implication of PnJ in the acceleration of the inflammatory process or functional deterioration in these patients has not been demonstrated

Factores relacionados con la infección por Pneumocystis jirovecii en pacientes con efermedad pulmonar intersticial difusa (EPID) / Factors related to the infection by pneumocystis jirovecii in patients with intestitial pulmonary disease (DIPD)

Objetivo: Hemos realizado un estudio prospectivo para determinarla prevalencia de colonización de Pn. jiroveccii (PnJ) enmuestras de LBA en pacientes con EPID y los factores que puedencondicionar esta situación.Material y métodos: Se incluyen 240 pacientes con EPID conuna media de edad de 56 años. Se estudió el gen mtLSU rRNA dePnJ mediante PCR anidada. La PCR resultó positiva en el 32%(78 pacientes).Resultados: Sólo el tabaquismo mostró una asociación significativacon la evidencia de colonización. Hematológicamente, la leucocitosisy eosinofilia son parámetros relacionados con ésta. Radiológicamente, en el TACAR no hay ningún hallazgo distintivo ytampoco hay diferencia entre ambos subgrupos (PCR+ vs PCR-)en la distribución de las patologías más frecuentes en nuestromedio: fibrosis pulmonar idiopática, sarcoidosis, bronquiolitisobliterante con neumonía organizativa y conectivopatías. En elestudio de los parámetros del LBA, tampoco se observan diferenciassignificativas. En el seguimiento, no se han evidenciado complicacionesinfecciosas atribuibles a este patógeno.Conclusiones: PnJ coloniza un tercio de la población con EPIDsin que se haya definido con claridad ningún factor determinante.En el seguimiento de estos pacientes no se han evidenciado complicacionesinfecciosas significativas. Falta por determinar la posibleimplicación de PnJ en la aceleración del proceso inflamatorio odeterioro funcional en estos pacientes (AU)

Objective: A prospective study was made to determine the prevalenceof colonization with Pn. jiroveccii (PnJ) in bronchoalveolar (BAL)samples of patients with DIPD, and the factors that can condition this situation.Material and methods: 240 patients with DIPD were included in thestudy, with an average age of 56 years. The mtLSU rRNA gene of PnJ wasstudied by means of nested PCR. The PCR was positive in 32% (78patients).Results Only tobacco use showed a significant association with theevidence of colonization. Leucocytosis and eosinophilia are parametersrelated to this phenomenon also. Radiologically, there were no distinctivefindings in high-resolution computed tomography (HRCT) nor differencebetween both sub-groups (PCR+ versus PCR-) in the distribution of themost frequent pathologies in our area: idiopathic pulmonary fibrosis, sarcoidosis,bronchiolitis obliterans with organizing pneumonia and connectivetissue diseases. Also, no significant differences were observed in thestudy of the BAL parameters. Infectious complications attributable tothis pathogen have not been demonstrated in the follow-up.Conclusions: PnJ colonises a third of the population with DIPDwithout any determining factor having been clearly defined so far. Significantinfectious complications have not been demonstrated in the followupof these patients. The possible implication of PnJ in the acceleration ofthe inflammatory process or functional deterioration in these patients hasnot been demonstrated (AU)

Angiotensinogen gene G-6A polymorphism influences idiopathic pulmonary fibrosis disease progression.

Angiotensin II is a growth factor that plays a key role in the physiopathology of idiopathic pulmonary fibrosis (IPF). A nucleotide substitution of an adenine instead of a guanine (G-6A) in the proximal promoter region of angiotensinogen (AGT), the precursor of angiotensin II, has been associated with an increased gene transcription rate. In order to investigate whether the G-6A polymorphism of the AGT gene is associated with IPF development, severity and progression, the present study utilised a case-control study design and genotyped G-6A in 219 patients with IPF and 224 control subjects. The distribution of G-6A genotypes and alleles did not significantly differ between cases and controls. The G-6A polymorphism of the AGT gene was not associated with disease severity at diagnosis. The presence of the A allele was strongly associated with increased alveolar arterial oxygen tension difference during follow-up, after controlling for the confounding factors. Higher alveolar arterial oxygen tension changes over time were observed in patients with the AA genotype (0.37+/-0.7 mmHg (0.049+/-0.093 kPa) per month) compared to GA genotype (0.12+/-1 mmHg (0.016+/-0.133 kPa) per month) and GG genotype (0.2+/-0.6 mmHg (0.027+/-0.080 kPa) per month). G-6A polymorphism of the angiotensinogen gene is associated with idiopathic pulmonary fibrosis progression but not with disease predisposition. This polymorphism could have a predictive significance in idiopathic pulmonary fibrosis patients.

Fibrosis pulmonar idiopática. Marcadores de la actividad de la enfermedad / Idiopathic pulmonary fibrosis. Markers of disease activity

El estudio de los marcadores de la actividad de la fibrosis pulmonar idiopática constituye uno de sus principales problemas, tanto por lo prometedor de sus expectativas como por lo controvertido de sus resultados. Los principales problemas que se han encontrado en su evaluación han sido el cambiante concepto de la enfermedad, así como de su clasificación y del patrón histológico asociado a ella, y la pobre definición de los conceptos de actividad e inflamación pulmonar. Esto ha llevado a replantear la hipótesis inflamatoria de la enfermedad. Entre los marcadores evaluados figuran las pruebas de función respiratoria, pruebas de imagen, como la gammagrafía pulmonar de perfusión con Galio67, y marcadores morfológicos de la biopsia pulmonar o del líquido de lavado broncoalveolar. Sin embargo, en el momento actual no disponemos de evidencia suficiente como para definir un marcador concreto que nos de una idea definitiva de la actividad de la enfermedad (AU)

The study of the markers of activity in idiopathic pulmonary fibrosis (IPF) constitutes one of its main problems, as much by the promising of its expectations like by its controversial results. Some of the difficulties found in the evaluation of IPF have been the changing concept of the disease, its classification, the histological pattern of the disease, and the lack of consistency of activity and pulmonary inflammation definitions. This has led to reframe the inflammatory hypothesis of the disease. Some markers of the IPF activity include: pulmonary function tests, imaging tests like lung perfusion scans with Ga67, and morphologic markers obtained from the pulmonary biopsies and from broncoalveolar lavage fluid. Nevertheless, at present, there is no evidence enough to define an individual marker to assess the activity of the disease (AU)

[Radiographic staging and bronchoalveolar lavage cell counts in sarcoidosis]. / Estadios radiológicos y lavado broncoalveolar en la sarcoidosis.

OBJECTIVE: Sarcoidosis is a multisystem granulomatous inflammatory disease of unknown etiology that mainly affects the lungs and lymph nodes. Bronchoalveolar lavage (BAL) is known to be useful in diagnosis of the disease but its value as a prognostic marker is unclear. The aim of this study was to assess whether there is a characteristic pattern in BAL cell counts according to radiographic stage and determine whether BAL offers information on disease course. PATIENTS AND METHODS: The study included 34 patients with untreated sarcoidosis. Data were collected on the following variables: age, sex, smoking habit, treatment type, radiographic stage, respiratory function, serological parameters, and BAL cell counts. The patients were classified into 3 groups according to functional and radiographic change at 12-month follow-up. RESULTS: No differences in age, sex, or smoking habit were found according to either radiographic stage or disease course. Although the proportion of lymphocytes in BAL fluid was higher in radiographic stage I than in stages II and III, the differences were not statistically significant. The differences in BAL cell counts between groups based on disease course were not statistically significant. CONCLUSIONS: No differences were found in the characteristics of BAL fluid according to radiographic stage. The differential cell count in BAL fluid does not appear to predict the course of sarcoidosis in the first 12 months.

Estadios radiológicos y lavado broncoalveolar en la sarcoidosis / Radiographic staging and bronchoalveolar lavage cell counts in sarcoidosis

Objetivo: La sarcoidosis es una enfermedad inflamatoria granulomatosa multisistémica de causa desconocida que afecta principalmente al pulmón y a los ganglios linfáticos. La utilidad del lavado broncoalveolar (LBA) en el diagnóstico es conocida, pero su valor como marcador pronóstico es controvertido. El objetivo de nuestro estudio es evaluar si existe un patrón característico en la celularidad del LBA según el estadio radiológico de presentación y determinar si el LBA aporta información sobre la evolución de la enfermedad. Pacientes y métodos: Se incluyó en el estudio a 34 pacientes con sarcoidosis no tratados. Se recogieron las siguientes variables: edad, sexo, hábito tabáquico, tipo de tratamiento, estadio radiológico, exploración funcional respiratoria, parámetros serológicos y análisis celular del LBA. Se clasificó a los pacientes en 3 grupos según la evolución funcional y radiológica a los 12 meses. Resultados: No se encontraron diferencias entre la edad, el sexo y el hábito tabáquico ni entre los estadios radiológicos ni entre los grupos según evolución. En el estadio radiológico I el recuento porcentual de linfocitos del LBA fue mayor que en los estadios II y III, pero las diferencias no fueron estadísticamente significativas. Las diferencias en el LBA por grupos evolutivos no fueron estadísticamente significativas. Conclusiones: Al analizar las características del LBA según estadios radiológicos no se encontraron diferencias. El recuento diferencial de células en el LBA no parece predecir el curso de la sarcoidosis durante los primeros 12 meses

Objective: Sarcoidosis is a multisystem granulomatous inflammatory disease of unknown etiology that mainly affects the lungs and lymph nodes. Bronchoalveolar lavage (BAL) is known to be useful in diagnosis of the disease but its value as a prognostic marker is unclear. The aim of this study was to assess whether there is a characteristic pattern in BAL cell counts according to radiographic stage and determine whether BAL offers information on disease course. Patients and methods: The study included 34 patients with untreated sarcoidosis. Data were collected on the following variables: age, sex, smoking habit, treatment type, radiographic stage, respiratory function, serological parameters, and BAL cell counts. The patients were classified into 3 groups according to functional and radiographic change at 12-month follow-up. Results: No differences in age, sex, or smoking habit were found according to either radiographic stage or disease course. Although the proportion of lymphocytes in BAL fluid was higher in radiographic stage I than in stages II and III, the differences were not statistically significant. The differences in BAL cell counts between groups based on disease course were not statistically significant. Conclusions: No differences were found in the characteristics of BAL fluid according to radiographic stage. The differential cell count in BAL fluid does not appear to predict the course of sarcoidosis in the first 12 months

Presentación de adenocarcinoma pulmonar como enfermedad pulmonar intersticial difusa / Pulmonary adenocarcinoma presenting as diffuse interstitial lung disease

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Diagnóstico y tratamiento de las enfermedades pulmonares intersticiales difusas / Diagnosis and treatment of diffuse interstitial lung diseases

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[Metastatic pulmonary calcification: a rare cause of interstitial lung disease]. / Calcificación pulmonar metastásica: una rara causa de enfermedad pulmonar intersticial.

We report the case of a 58-year-old woman with metastatic pulmonary calcinosis who presented with bronchial hyperreactivity. She was receiving calcium and vitamin D supplementation following total bilateral thyroidectomy with parathyroidectomy and had a history of episodes of symptomatic hypercalcemia secondary to exogenous administration. Lung function testing showed slight obstruction that was reversed by bronchodilators. Images showed a bilateral micronodular pattern mainly in the upper fields (x-ray and high resolution computed tomography of the thorax) and abnormal calcium deposition in the lungs (bone scintigraphy). The diagnosis was established by transbronchial biopsy. The clinical course was favorable. Metastatic pulmonary calcinosis is rare and usually asymptomatic and evolution is good. This entity should be taken into account in the differential diagnosis of interstitial lung diseases involving micronodular infiltrates in patients at risk.