Diagnóstico precoz de la fibrosis pulmonar idiopática / Early diagnosis of idiopathic pulmonary fibrosis

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Fibrosis pulmonar idiopática: un reto para la atención primaria / Idiopathic pulmonary fibrosis: A challenge for primary care

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Fibrobroncoscopia en una unidad de vigilancia intensiva respiratoria / Fiberoptic bronchoscopy in a respiratory intensive care unit

Objetivo: Describir las indicaciones, rentabilidad diagnóstica y complicaciones de la fibrobroncoscopia (FBS) en una unidad de vigilancia intensiva respiratoria (UVIR). Diseño: Estudio prospectivo observacional. Ámbito: UVIR de 6 camas en un hospital universitario de tercer nivel. Pacientes: Pacientes admitidos en una UVIR a los que se les realizó una FBS. Intervenciones: Ninguna. Variables de interés: Indicaciones y complicaciones de la FBS, técnicas endoscópicas realizadas y tiempo empleado en la FBS. Resultados: Se realizaron 107 (23%) FBS a 69 de los 297 pacientes admitidos en la UVIR. El 68% de las FBS se practicaron a pacientes con ventilación mecánica. La FBS se realizó con fines diagnósticos en 88 ocasiones (82%) y terapéuticos en 19 (18%). La indicación más frecuente para la FBS diagnóstica fue el estudio de infiltrados pulmonares (44 casos; 50%), particularmente en pacientes inmunodeprimidos (24 casos; 27%). Para esta indicación, la rentabilidad diagnóstica de la FBS fue significativamente mejor en los pacientes inmunodeprimidos, respecto a los inmunocompetentes (48% vs 30%; p<0,01). La FBS no causó complicaciones mayores; únicamente se observó un descenso significativo en la PaO2/FiO2 (182±74 vs 163±79; p<0,005) cuando se realizó un lavado broncoalveolar. La mortalidad global en la UVIR fue del 14%; del 25% en los pacientes que precisaron FBS y del 45% en aquellos que precisaron FBS adicionales. Conclusiones: La FBS es un procedimiento seguro y rápido que se utiliza con frecuencia en la UVIR y que contribuye significativamente al manejo clínico. Los pacientes de la UVIR que requieren FBS adicionales tienen una elevada mortalidad (AU)

Objective: To describe the indications, diagnostic performance and safety of fiberoptic bronchoscopy (FOB) performed in a respiratory intensive care unit (RICU). Design: A prospective, observational study was carried out. Setting: A 6-bed RICU in a tertiary university hospital. Patients: Patients admitted to RICU who required FOB. Interventions: None. Main measurements: FOB indications and complications, endoscopic procedures, time required to perform FOB. Results: Sixty-nine out (23%) of the 297 patients admitted to the RICU underwent a total of 107 FOB. Sixty-eight percent of FOB were performed in patients on mechanical ventilation. FOB was performed for diagnostic and therapeutic purposes in 88 (82%) and 19 cases (18%), respectively. The study of pulmonary infiltrates was the main indication for diagnostic FOB (44 cases; 50%), particularly in immunocompromised patients (24 cases; 27%). In immunocompromised patients the diagnostic performance of FOB was significantly higher than in immunocompetent subjects (48% vs 30%; p<0.01). No major complications were recorded. Only a significant drop in PaO2/FiO2 ratio was observed (182±74 vs 163±79; p<0.005) in patients undergoing bronchoalveolar lavage. Overall mortality in patients in the RICU was 14%. In patients requiring a single FOB procedure, mortality was 25%, versus 45% among those requiring more than one FOB procedure. Conclusions: These results show that FOB is used commonly in the RICU. It is a safe and fast procedure that contributes significantly to clinical management. Patients requiring additional FOB during admission to the RICU show high mortality (AU)

[Fiberoptic bronchoscopy in a respiratory intensive care unit]. / Fibrobroncoscopia en una unidad de vigilancia intensiva respiratoria.

OBJECTIVE: To describe the indications, diagnostic performance and safety of fiberoptic bronchoscopy (FOB) performed in a respiratory intensive care unit (RICU). DESIGN: A prospective, observational study was carried out. SETTING: A 6-bed RICU in a tertiary university hospital. PATIENTS: Patients admitted to RICU who required FOB. INTERVENTIONS: None. MAIN MEASUREMENTS: FOB indications and complications, endoscopic procedures, time required to perform FOB. RESULTS: Sixty-nine out (23%) of the 297 patients admitted to the RICU underwent a total of 107 FOB. Sixty-eight percent of FOB were performed in patients on mechanical ventilation. FOB was performed for diagnostic and therapeutic purposes in 88 (82%) and 19 cases (18%), respectively. The study of pulmonary infiltrates was the main indication for diagnostic FOB (44 cases; 50%), particularly in immunocompromised patients (24 cases; 27%). In immunocompromised patients the diagnostic performance of FOB was significantly higher than in immunocompetent subjects (48% vs 30%; p<0.01). No major complications were recorded. Only a significant drop in PaO(2)/FiO(2) ratio was observed (182 ± 74 vs 163 ± 79; p<0.005) in patients undergoing bronchoalveolar lavage. Overall mortality in patients in the RICU was 14%. In patients requiring a single FOB procedure, mortality was 25%, versus 45% among those requiring more than one FOB procedure. CONCLUSIONS: These results show that FOB is used commonly in the RICU. It is a safe and fast procedure that contributes significantly to clinical management. Patients requiring additional FOB during admission to the RICU show high mortality.

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.

Fe de errores de “Implicación de la alteración en la regulación de la ciclooxigenasa-2 sobre la formación de miofibroblastos en la fibrogénesis pulmonar” / No disponible

Introducción: Se ha descrito la existencia de una disminución en la expresión de ciclooxigenasa 2 (COX-2) y de prostaglandina-E2 (PGE-2) en la fibrogénesis pulmonar. La PGE-2 es un mediador antifibrótico. Los miofibroblastos desempeñan un importante papel en la fibrogénesis. Los miofibroblastos pueden formarse a partir de fibroblastos (transición fibroblasto-miofibroblasto) y de células epiteliales (transición epitelio-mesenquimal). El objetivo es estudiar la expresión de COX-2 y la síntesis de PGE2 en la inducción de la formación de miofibroblastos.Métodos: Mediante biopsia pulmonar se obtuvieron fibroblastos normales de sujetos con neumotórax (n=5) y fibroblastos de pacientes con fibrosis pulmonar idiopática (FPI) (n=5). Para el estudio de la función epitelial se utilizaron células epiteliales inmortales A549. Se realizó inmunohistoquímica (IHC) y Western blot para la determinación de COX-2 y α-SMA (marcador de miofibroblastos). Mediante ELISA se estudio la síntesis de PGE2. La proliferación celular se valoró mediante la incorporación nuclear de un análogo de nucleósido.Resultados: Los fibroblastos de la FPI presentan mayores niveles de ß-SMA comparados con los fibroblastos control. Ambos presentan una expresión indetectable de COX-2. Después de la estimulación con interleucina 1ß (IL-1ß), los fibroblastos control expresan mayores niveles de COX-2 que los fibroblastos fibróticos. Los miofibroblastos detectados mediante IHC no expresaron COX-2. Los fibroblastos tratados con TGF-ß1 expresan α-SMA, en forma, dosis y tiempo dependiente, tanto en fibroblastos fibróticos como en controles. A549 tratadas con TGF-ß1 cambian su fenotipo, expresando fibras de estrés relacionadas con la formación de miofibroblastos (α-SMA+). Los miofibroblastos obtenidos de tratar fibroblatos o A549 con TGF-ß1 muestran un descenso de los niveles de COX-2 y PGE-2 tras estimulación con IL-1ß. No hubo variaciones en la proliferación celular(AU)

Conclusiones: Los miofibroblastos se caracterizan por una alteración en la regulación de la expresión de COX-2 y en la síntesis de PGE-2, tanto en los transformados a partir de fibroblastos como de células epiteliales(AU)

Lymphangioleiomyomatosis: a study of 72 patients from the Spanish registry.

BACKGROUND: Pulmonary lymphangioleiomyomatosis (LAM) is a rare lung disease that almost exclusively affects young women of childbearing age. The true incidence and prevalence of LAM are unknown. This study was conducted to evaluate the characteristics of lymphangioleiomyomatosis in Spain. METHODS: Over a 2-year period, a questionnaire designed for this study was collected. This questionnaire included sociodemograhic, clinical, radiological and functional data. Information about the study and this questionnaire were both sent by e-mail to all the participants of the interstitial disease registry of 2004. RESULTS: Seventy-two patients, all of whom were women, were included in the registry, with a mean age of 44.56 +/- 11.1 yr. Sixty-three patients (87.5%) presented the sporadic LAM and 9 (12.5%) presented LAM associated with tuberous sclerosis (LAM-TS). LAM diagnosis was confirmed with an open lung biopsy in 57 patients (79.2%) and was performed with thoracic HRCT compatible with LAM diagnosis in the other 15 cases. The most frequent symptom was dyspnoea (90%) followed by cough (44.4%). Almost 40% of patients presented renal angiomyolipomas in the study and the most frequent spirometric pattern was obstructive in more than half of the patients. Most patients with LAM-TS (88.8%) had renal angiomyolipomas compared with 31.7% in the sporadic LAM group. CONCLUSION: The characteristics of the Spanish population affected with LAM are similar to those of other countries. Most patients were symptomatic, had a history of previous pneumothorax and presented abnormal radiological findings and pulmonary function tests.

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.

Losartan attenuates bleomycin induced lung fibrosis by increasing prostaglandin E2 synthesis.

BACKGROUND: The angiotensin system has a role in the pathogenesis of pulmonary fibrosis. This study examines the antifibrotic effect of losartan, an angiotensin II type 1 receptor antagonist, in bleomycin induced lung fibrosis and its possible implication in the regulation of prostaglandin E(2) (PGE(2)) synthesis and cyclooxygenase-2 (COX-2) expression. METHODS: Rats were given a single intratracheal instillation of bleomycin (2.5 U/kg). Losartan (50 mg/kg/day) was administrated orally starting one day before induction of lung fibrosis and continuing to the conclusion of each experiment. RESULTS: Losartan reduced the inflammation induced by bleomycin, as indicated by lower myeloperoxidase activity and protein content in the bronchoalveolar lavage fluid. Collagen deposition induced by bleomycin was inhibited by losartan, as shown by a reduction in the hydroxyproline content and the amelioration of morphological changes. PGE(2) levels were lower in fibrotic lungs than in normal lungs. Losartan significantly increased PGE(2) levels at both 3 and 15 days. A reduction in COX-2 expression by bleomycin was seen at 3 days which was relieved by losartan. CONCLUSIONS: The antifibrotic effect of losartan appears to be mediated by its ability to stimulate the production of PGE(2). Losartan, which is already widely used clinically, could be assessed as a new treatment in lung fibrosis.

Mucin genes have different expression patterns in healthy and diseased upper airway mucosa.

BACKGROUND: Mucus hyper-secretion is a feature of several airways diseases such as chronic rhinosinusitis, asthma, and cystic fibrosis (CF). Since mucins are major components of mucus, the knowledge of their distribution and regulation in nasal tissues is likely to improve mucus hyper-secretion therapy. OBJECTIVE: The aim of this study was to evaluate and compare mucin gene expression at epithelial and glandular levels, and to identify potential mucin expression patterns for specific upper airways pathologies. METHODS: Immunohistochemistry for MUC1, MUC2, and MUC4-MUC8 mucins was performed on healthy nasal mucosa (NM; n=12), bilateral nasal polyps (NP; n=38), NP from CF patients (n=10), and antrochoanal (AC) polyps (n=11). MUC2, MUC4, MUC5AC, and MUC6 mRNA expression were also analysed by in situ hybridization. RESULTS: MUC1, MUC4, and MUC5AC mucins were highly expressed in the epithelium and their expression pattern was similar in all NP types, MUC1 and MUC4 being increased and MUC5AC decreased compared with NM. MUC8 was highly detected at both epithelial and glandular levels with marked variability between groups. MUC5B was mainly detected in glands and the expression in all polyp types was higher than in NM. Moreover, MUC5B expression was higher in NP epithelia from CF patients than in bilateral NP and healthy NM. Although MUC2 expression was low, especially in AC polyps, it was detected in most samples. In NM, MUC6 and MUC7 were scarcely detected and MUC7 expression was restricted to glands. CONCLUSIONS: These results suggest that NP have a different pattern of mucin expression than healthy NM and that CF polyps (increased MUC5B) and AC polyps (decreased MUC2) have a different mucin expression pattern than bilateral NP.

Effect of desloratadine on epithelial cell granulocyte-macrophage colony-stimulating factor secretion and eosinophil survival.

BACKGROUND: Second-generation antihistamines are H(1) receptor antagonists and may have additional anti-inflammatory effects. OBJECTIVE: The aims of the study were to evaluate the effect of desloratadine (DL) on cytokine secretion by epithelial cells from both nasal mucosa (NM) and polyps (NP), and on eosinophil survival primed by epithelial cell secretions. METHODS: Epithelial cells were cultured and stimulated with fetal bovine serum (FBS), IL-1beta or TNF-alpha with and without DL for 24 h. Culture supernatant cytokines concentration were measured by ELISA. Peripheral blood eosinophils were incubated with human epithelial cell conditioned media (HECM) and DL. Eosinophil survival was assessed by Trypan blue dye exclusion. Results are expressed as mean+/-SEM of cytokine concentration (pg/mL) or eosinophil survival index (%). RESULTS: FBS increased granulocyte-macrophage colony-stimulating factor (GM-CSF), vascular endothelial growth factor (VEGF), IL-6, IL-8, and TGF-beta(1) secretion in epithelial cell cultures from both NM and NP. Only GM-CSF secretion was significantly (P<0.05) inhibited by a dose-response of DL compared with positive controls, in both NM (10(-5) m: 125+/-36 pg/mL, 10(-6) m: 95+/-22 pg/mL vs. control: 256+/-91 pg/mL, n=6) and NP (10(-5) m: 80+/-29 pg/mL, 10(-6) m: 109+/-45 pg/mL vs. control: 333+/-212 pg/mL, n=6). DL also showed an inhibitory effect on HECM-induced eosinophil survival from both NM and NP. At 72 h, DL significantly (P<0.01) inhibited eosinophil survival induced by HECM from NM (10(-5) m: 19.9+/-5.5%, n=9; 10(-6) m: 28.7+/-7.7%, n=9) and NP (10(-5) m: 6.2+/-2.8%, n=11) compared with HECM alone (NM: 42.1+/-7.3%; NP: 45.3+/-8.1%). CONCLUSION: The inhibitory effects of DL on epithelial cell GM-CSF secretion and on eosinophil survival induced by epithelial cell secretions, suggest that this H(1) antagonist may regulate eosinophil inflammation in upper airways.

[Nonspecific interstitial pneumonia: epidemiologic and clinical characteristics]. / Neumonía intersticial no específica: Características clínicas y epidemiológicas.

BACKGROUND AND OBJECTIVES: Patients diagnosed with non-specific interstitial pneumonia (NSIP) in 2 hospitals in Barcelona, Spain, were studied to investigate the factors that could contribute to the etiology of disease. PATIENTS AND METHOD: The consensus diagnostic criteria established by the American Thoracic Society/European Respiratory Society were followed. The study included 16 patients, 10 men (65%) and 6 women (35%), all diagnosed with NSIP by open lung biopsy. Patients were questioned on pathological history, occupational or environmental exposure, medicinal drug use, contact with birds, and relationship with smoking. In addition, were recorded symptoms and physical signs, laboratory and respiratory function results, chest X-ray and computerized tomography scan features, fiberbronchoscopy findings and open lung biopsy findings. RESULTS: Eleven patients (69%) -10 men (100%) and one woman (12%) -were smokers: 4 were active and 7 former smokers; 8 (80%) patients had fibrotic NSIP and three (50%) had cellular NSIP. Nine (56%) patients had a medication history, and 5 received chronic non-steroideal anti-inflammatory drug treatment. Occupational exposure to bleach, detergents, and ammonia or chloride products was documented in 6 cases. Three patients had contact with manufactured plastic products and isocyanate inhalation. CONCLUSIONS: Smoking and medication use were prevalent in NSIP patients. Ibuprofen and celecoxib clearly provoked symptoms in one NSIP patient. Further studies are required to clarify the role of detergents, isocyanates and other occupation-related substances as triggering factors.

Neumonía intersticial no específica: Características clínicas y epidemiológicas / Nonspecific intersticial pneumonia: epidemiologic and clinical characteristics

Fundamento y objetivo: Estudiar a pacientes diagnosticados de neumonía intersticial no específica (NINE) en 2 hospitales de Barcelona prestando especial atención al papel etiológico del tabaco, los factores ambientales y/o ocupacionales y el consumo previo de medicamentos. Pacientes y método: Se han seguido los criterios diagnósticos establecidos por el consenso de la American Thoracic Society/European Respiratory Society. Se ha incluido a 16 pacientes ­10 varones (65%) y 6 mujeres (35%)­, todos diagnosticados de NINE por biopsia pulmonar quirúrgica. Se recogió información sobre antecedentes patológicos, contacto con aves y/o con tóxicos, ingesta de medicamentos y hábito tabáquico. Se estudiaron los síntomas y signos de presentación, pruebas analíticas, de función respiratoria, radiografía de tórax y tomografía computarizada de alta resolución torácica, estudio fibrobroncoscópico, que incluyó lavado broncoalveolar y biopsia transbronquial, y por último biopsia pulmonar quirúrgica. Resultados: Diez pacientes (100%) varones y una mujer (12%) eran fumadores, 4 en activo y 7 ex fumadores; 8 (80%) tenían NINE fibrótica y 3 (50%), NINE celular. Tomaban algún medicamento 9 pacientes (56%), 5 de ellos algún tipo de antiinflamatorio no estroideo. Se presenta un caso clínico de NINE relacionado con el consumo inicial de ibuprofeno y posteriormente de celecoxib. Se evidenció contacto crónico con lejías, detergentes, amoníaco y productos clorados en 6 pacientes y con productos manufacturados del plástico y, en concreto, inhalación de isocianatos, en 3 pacientes. Conclusiones: Se describe una posible relación del tabaco y/o del consumo previo de medicamentos en el desarrollo de la NINE. Se requieren estudios para aclarar el posible papel de los productos de limpieza, isocianatos y otras sustancias de carácter ocupacional

Background and objectives: Patients diagnosed with non-specific interstitial pneumonia (NSIP) in 2 hospitals in Barcelona, Spain, were studied to investigate the factors that could contribute to the etiology of disease. Patients and method: The consensus diagnostic criteria established by the American Thoracic Society/European Respiratory Society were followed. The study included 16 patients, 10 men (65%) and 6 women (35%), all diagnosed with NSIP by open lung biopsy. Patients were questioned on pathological history, occupational or environmental exposure, medicinal drug use, contact with birds, and relationship with smoking. In addition, were recorded symptoms and physical signs, laboratory and respiratory function results, chest X-ray and computerized tomography scan features, fiberbronchoscopy findings and open lung biopsy findings. Results: Eleven patients (69%) ­10 men (100%) and one woman (12%) ­were smokers: 4 were active and 7 former smokers; 8 (80%) patients had fibrotic NSIP and three (50%) had cellular NSIP. Nine (56%) patients had a medication history, and 5 received chronic non-steroideal anti-inflammatory drug treatment. Occupational exposure to bleach, detergents, and ammonia or chloride products was documented in 6 cases. Three patients had contact with manufactured plastic products and isocyanate inhalation. Conclusions: Smoking and medication use were prevalent in NSIP patients. Ibuprofen and celecoxib clearly provoked symptoms in one NSIP patient. Further studies are required to clarify the role of detergents, isocyanates and other occupation-related substances as triggering factors

Expression of glucocorticoid receptors alpha and beta in steroid sensitive and steroid insensitive interstitial lung diseases.

BACKGROUND: Sensitivity to glucocorticoids may be related to the concentration of glucocorticoid receptors alpha (GRalpha) and beta (GRbeta). A study was undertaken to assess GRalpha and GRbeta expression in steroid insensitive interstitial lung disease (idiopathic pulmonary fibrosis (IPF)) and steroid sensitive interstitial lung diseases (sarcoidosis and cryptogenic organising pneumonia (COP)). METHODS: Lung tissue was obtained from control subjects and from patients with IPF, sarcoidosis, and COP. Pulmonary function tests were carried out at the time of lung biopsy and every 3 months. GRalpha and GRbeta expression was evaluated by both competitive RT-PCR and immunohistochemistry. Data are presented as median and 25-75th percentile. RESULTS: GRalpha mRNA expression (10(5) cDNA copies/ micro g total RNA) was higher in patients with steroid sensitive interstitial lung diseases (10.0; 7.8-14.9; n = 11) than in patients with IPF (4.4; 3.2-6.6; n = 19; p<0.001). GRbeta expression was at least 1000 times lower than that of GRalpha and did not differ between the three groups. A negative correlation was found between GRalpha mRNA levels and the fibrotic pathology score of the tissue (r = -0.484, p<0.01) and a positive correlation was found between GRalpha mRNA levels and improvement in forced vital capacity (r = 0.633; p<0.01) after treatment of patients with glucocorticoids. Immunoreactivity for GR protein was also higher in patients with sarcoidosis and COP than in those with IPF. CONCLUSION: The variable response of some interstitial lung diseases to steroid treatment may be the result of differences in the expression of GRalpha.

[Bronchiolitis obliterans associated with paraneoplastic pemphigus: a paraneoplastic autoimmune multiorgan syndrome]. / Bronquiolitis obliterate y pénfigo paraneoplásico: un síndrome paraneoplásico autoimmune multiorgánico.

INTRODUCTION: Paraneoplastic pemphigus is a mucocutaneous disease characterized by well defined clinical and immunopathological features associated with neoplasia. Recent evidence of bronchial epithelium involvement has led to the suggestion that this process is a paraneoplastic autoimmune multiorgan syndrome. CLINICAL OBSERVATION: We report the case of a patient with lichenoid eruptions on the skin and mucous membranes who later developed progressive dyspnea. With a suspected diagnosis of paraneoplastic autoimmune multiorgan syndrome, the following diagnostic tests were performed: histology and immunofluorescence of the skin, oral mucosa, and bronchial epithelium; indirect immunofluorescence of serum; pulmonary function tests; and evaluation for an occult neoplasm. Findings of pathology and immunofluorescence confirmed the suspected diagnosis. The computed thoracoabdominal tomography revealed signs of bronchiolitis and the presence of a retroperitoneal tumor. CONCLUSIONS: Awareness of the mucocutaneous manifestations of paraneoplastic autoimmune multiorgan syndrome, and confirmation of this diagnosis by simple laboratory techniques can facilitate the early detection of occult neoplasia and forestall respiratory involvement.

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

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[Dendriform pulmonary ossification associated with idiopathic pulmonary fibrosis]. / Osificación pulmonar dendriforme asociada con fibrosis pulmonar idiopática.

Diffuse pulmonary ossification, a rare condition characterized by metaplastic ossification of the lung, is usually associated with diseases causing diffuse pulmonary lesions. Two types dendriform and nodular have been identified. In dendriform ossification, the less common type, osseous ramifications occur along the distal airways, with occasional islets of bone marrow. We report a case of diffuse dendriform pulmonary ossification associated with idiopathic pulmonary fibrosis. The diagnosis was based on histological examination, which demonstrated multiple nodules and ramified osseous spicules around the lung, mainly at the lower lobes, where the fibrotic lesions were also most evident.