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Objective: Interstitial lung disease (ILD) is a common feature of connective tissue disease (CTD). The diagnosis of CTD-ILD can be challenging and is important for therapeutic decisions. In this study, we aimed to determine whether a systematic rheumatological assessment could help pulmonologists in the diagnosis and care of ILD patients.Method: We conducted an observational single-centre study of patients with ILD. All patients underwent standardized pulmonary and rheumatological evaluations, including clinical evaluation (pulmonary symptoms and musculoskeletal signs), immunological screening, chest high-resolution computed tomography, pulmonary function tests, and ultrasonography (US) of joints and major salivary glands.Results: We included 100 consecutive ILD patients (47% women, mean ± sd age 67 ± 14 years); 15 patients already had CTD. The main extrapulmonary symptoms were joint pain (n = 52), joint swelling (n = 26), and sicca syndrome (n = 33). US of joints revealed synovitis, bone erosion, and tenosynovitis in 37, 17, and 13 patients, respectively. US of major salivary glands detected features associated with Sjögren's syndrome in 13 patients. After rheumatological evaluation, CTD-ILD was confidently diagnosed in 39 patients; diseases were mainly rheumatoid arthritis (n = 20), primary Sjögren's syndrome (n = 17), and inflammatory myopathies (n = 7). The diagnosis of CTD-ILD was associated with the presence of musculoskeletal symptoms and immunological and US abnormalities. The CTD diagnosis led to a therapeutic change in 21 patients.Conclusion: Our findings suggest that musculoskeletal symptoms are frequent in ILD patients, which supports multidisciplinary management, involving the rheumatologist, for evaluating patients with ILD.
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Enfermedades del Tejido Conjuntivo , Enfermedades Pulmonares Intersticiales , Síndrome de Sjögren , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Pulmón , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/etiología , Masculino , Persona de Mediana Edad , Pruebas de Función Respiratoria , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnósticoRESUMEN
AIM: To determine whether findings from lung ultrasound and chest high-resolution computed tomography (HRCT) correlate when evaluating COVID-19 pulmonary involvement. MATERIALS AND METHODS: The present prospective single-centre study included consecutive symptomatic patients with reverse transcription polymerase chain reaction (RT-PCR)-proven COVID-19 who were not in the intensive care unit. All patients were assessed using HRCT and ultrasound of the lungs by distinct operators blinded to each other's findings. The number of areas (0-12) with B-lines and/or consolidations was evaluated using ultrasound and compared to the percentage and classification (absent or limited, <10%; moderate, 10-25%; extensive, 25-50%; severe, 50-75%; critical, >75%) of lung involvement on chest HRCT. RESULTS: Data were analysed for 21 patients with COVID-19 (median [range] age 65 [37-90] years, 76% male) and excellent correlation was found between the ultrasound score for B-lines and the classification (p<0.01) and percentage of lung involvement on chest HRCT (r=0.935, p<0.001). In addition, the ultrasound score correlated positively with supplemental oxygen therapy (r=0.45, p=0.041) and negatively with minimal oxygen saturation at ambient air (r=-0.652, p<0.01). CONCLUSION: The present study suggests that among COVID-19 patients, lung ultrasound and HRCT findings agree in quantifying lung involvement and oxygen parameters. In the context of the COVID-19 pandemic, lung ultrasound could be a relevant alternative to chest HRCT.
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Infecciones por Coronavirus/diagnóstico por imagen , Infecciones por Coronavirus/epidemiología , Neumonía Viral/diagnóstico por imagen , Neumonía Viral/epidemiología , Síndrome Respiratorio Agudo Grave/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Ultrasonografía Doppler/métodos , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , COVID-19 , Distribución de Chi-Cuadrado , Estudios de Cohortes , Infecciones por Coronavirus/fisiopatología , ADN Viral/análisis , Femenino , Humanos , Italia , Masculino , Persona de Mediana Edad , Variaciones Dependientes del Observador , Pandemias , Neumonía Viral/fisiopatología , Estudios Prospectivos , Reacción en Cadena en Tiempo Real de la Polimerasa/métodos , Medición de Riesgo , Síndrome Respiratorio Agudo Grave/epidemiología , Síndrome Respiratorio Agudo Grave/fisiopatología , Índice de Severidad de la Enfermedad , Factores Sexuales , Estadísticas no ParamétricasAsunto(s)
Metales Pesados , Micorrizas , Simbiosis , Micorrizas/fisiología , Metales Pesados/análisisRESUMEN
INTRODUCTION: Methotrexate (MTX) is a folate antagonist used as an immunosuppressant in a number of conditions, including rheumatoid arthritis (RA). Low-dose MTX (MTX-LD) is associated with a risk of haematological, hepatic, gastrointestinal and pulmonary toxicity, which may up until now have limited its use. STATE OF THE ART: In RA, data from retrospective cohorts have reported a possible excess risk of methotrexate toxicity in cases of underlying interstitial lung disease (ILD). However, recent prospective and retrospective multicentre studies have found no such increased risk, and have reassuringly concluded that MTX-LD can be prescribed in cases of RA-associated ILD (RA-ILD). PERSPECTIVES AND CONCLUSIONS: Current recommendations are not to delay the introduction of MTX in patients with RA at risk of developing ILD or in the presence of RA-ILD with mild to moderate respiratory impairment.
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INTRODUCTION: Asthma is a common disease whose diagnosis does not typically rely on the results of imaging. However, chest CT has gained a key place over the last decade to support the management of patients with difficult to treat and severe asthma. STATE OF THE ART: Bronchial wall thickening and mild dilatation or narrowing of bronchial lumen are frequently observed on chest CT in people with asthma. Bronchial wall thickening is correlated to the degree of obstruction and to bronchial wall remodeling and inflammation. Diverse conditions which can mimic asthma should be recognized on CT, including endobronchial tumours, interstitial pneumonias, bronchiectasis and bronchiolitis. Ground-glass opacities and consolidation may be related to transient eosinophilic infiltrates, infection or an associated disease (vasculitis, chronic eosinophilic pneumonia). Hyperdense mucous plugging is highly specific for allergic bronchopulmonary aspergillosis. PERSPECTIVES: Airway morphometry, air trapping and quantitative analysis of ventilatory defects, with CT or MRI, can help to identify different morphological subgroups of patients with different functional or inflammatory characteristics. These imaging tools could emerge as new biomarkers for the evaluation of treatment response. CONCLUSION: Chest CT is indicated in people with severe asthma to search for additional or alternative diagnoses. Quantitative imaging may contribute to phenotyping this patient group.
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Aspergilosis Broncopulmonar Alérgica , Asma , Bronquiectasia , Asma/diagnóstico por imagen , Humanos , Pulmón , Tomografía Computarizada por Rayos XRESUMEN
INTRODUCTION: Thoracic endometriosis (TE) is a rare disorder affecting women during their reproductive years. Manifestations of TE include pneumothorax and haemothorax. Treatment is based on surgical and hormonal therapy that aims at eradicating existing endometrial thoracic plaques and to prevent reseeding from pelvic endometriosis. CASE REPORT: We report the case of a 36 year-old young woman presenting thoracic endometriosis revealed by a recurring spontaneous, large and isolated right haemothorax. Diagnosis, pathogeny and treatment are discussed. CONCLUSION: Thoracic endometriosis needs to be considered as a cause of haemothorax in women of childbearing age.
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Endometriosis/diagnóstico , Hemotórax/diagnóstico , Enfermedades Torácicas/diagnóstico , Adulto , Endometriosis/complicaciones , Endometriosis/cirugía , Femenino , Hemotórax/etiología , Hemotórax/cirugía , Humanos , Enfermedades Pleurales/complicaciones , Enfermedades Pleurales/diagnóstico , Enfermedades Pleurales/cirugía , Neumotórax/diagnóstico , Neumotórax/etiología , Neumotórax/cirugía , Recurrencia , Enfermedades Torácicas/complicaciones , Enfermedades Torácicas/cirugíaRESUMEN
Idiopathic pulmonary alveolar proteinosis is presumed to be an autoimmune disorder that may lead to pulmonary insufficiency. However, steroids do not appear to be effective and the standard of therapy is whole-lung lavage. We report the first case of successful therapy with rituximab, which addresses the pathogenic mechanism of pulmonary alveolar proteinosis.
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Anticuerpos Monoclonales/uso terapéutico , Enfermedades Autoinmunes/tratamiento farmacológico , Factores Inmunológicos/uso terapéutico , Proteinosis Alveolar Pulmonar/tratamiento farmacológico , Adulto , Anticuerpos Monoclonales de Origen Murino , Enfermedades Autoinmunes/diagnóstico , Biopsia , Lavado Broncoalveolar , Diagnóstico Diferencial , Ensayo de Inmunoadsorción Enzimática , Humanos , Proteinosis Alveolar Pulmonar/diagnóstico , Pruebas de Función Respiratoria , Rituximab , Tomografía Computarizada por Rayos XRESUMEN
The present authors report the case of an adult with chronic granulomatous disease who developed an unusual lung fibrosis associated with severe pulmonary hypertension. Histological analysis of a lung biopsy showed a diffuse infiltration with pigmented macrophages without granulomas, which particularly involved the pulmonary arterial and venular walls. Clinical and histological findings were suggestive of pulmonary veno-occlusive disease. Such a clinical association has not been previously described in the literature and might be due to the persistent expression of gp91phox at a very low level. In conclusion, the present case report illustrates a novel manifestation of chronic granulomatous disease.
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Enfermedad Granulomatosa Crónica/complicaciones , Hipertensión Pulmonar/etiología , Enfermedades Pulmonares Intersticiales/etiología , Adulto , Biopsia , Lavado Broncoalveolar , Broncoscopía , Diagnóstico Diferencial , Diuréticos/uso terapéutico , Furosemida/uso terapéutico , Hemodinámica , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/fisiopatología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Masculino , Inhibidores de Fosfodiesterasa/uso terapéutico , Piperazinas/uso terapéutico , Purinas/uso terapéutico , Pruebas de Función Respiratoria , Citrato de Sildenafil , Fumar/efectos adversos , Sulfonas/uso terapéutico , Tomografía Computarizada por Rayos XRESUMEN
Lung hyperinflation which is a hallmark of advanced emphysema plays a major role in the exertional dyspnoea experienced by patients. This has led to the development of surgical lung volume reduction which, though effective, is also associated with significant morbidity and mortality. The goal of endoscopic lung volume reduction which has developed over several years is to decrease hyperinflation without exposing patients to the risks of surgery. Several endoscopic techniques have been assessed by high quality controlled studies: airway by-pass, instillation of glue, insertion of coils or unidirectional valves, vapour ablation. The aim of this review is to present the results of these studies in terms of functional benefit and side effects. Based on these studies, an algorithm for the endoscopic management of advanced forms of emphysema is proposed.
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Broncoscopía/métodos , Neumonectomía/métodos , Enfisema Pulmonar/cirugía , Algoritmos , Broncoscopía/efectos adversos , Broncoscopía/mortalidad , Humanos , Selección de Paciente , Neumonectomía/efectos adversos , Neumonectomía/mortalidad , Enfisema Pulmonar/mortalidadRESUMEN
Niemann Pick disease type B (NPD type B) is a rare autosomal recessive lipid storage disorder, characterized by a partial deficiency of sphingomyelinase. We report the case of an adult male patient affected by NPD type B and diagnosed at 39-years-of age. Pulmonary CT scan revealed a cranio-caudal gradient with nodular centrilobular ground glass opacities and thickening of the interlobular septa. Pathological examination of the bronchoalveolar lavage showed foamy alveolar macrophages and vacuolated bronchial epithelial cells on bronchial biopsy. Diagnostic confirmation was achieved by a decrease in cell lysosomal enzyme activity and by the presence of the homozygous DeltaR608 mutation in the acid sphingomyelinase gene (SMPD1).
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Bronconeumonía , Enfermedad de Niemann-Pick Tipo B , Adulto , Biopsia , Médula Ósea/patología , Lavado Broncoalveolar , Broncoscopía , Humanos , Masculino , Enfermedad de Niemann-Pick Tipo B/diagnóstico , Enfermedad de Niemann-Pick Tipo B/diagnóstico por imagen , Enfermedad de Niemann-Pick Tipo B/genética , Radiografía Torácica , Tomografía Computarizada por Rayos XAsunto(s)
Fármacos Gastrointestinales/uso terapéutico , Fibrosis Pulmonar Idiopática/tratamiento farmacológico , Octreótido/uso terapéutico , Anciano , Anciano de 80 o más Años , Biomarcadores/sangre , Femenino , Francia , Fármacos Gastrointestinales/efectos adversos , Humanos , Fibrosis Pulmonar Idiopática/fisiopatología , Masculino , Persona de Mediana Edad , Octreótido/efectos adversos , Octreótido/sangre , Pruebas de Función Respiratoria , Índice de Severidad de la Enfermedad , Insuficiencia del TratamientoRESUMEN
INTRODUCTION: Sjogren's syndrome is a common auto-immune disease. BACKGROUND: Clinically significant pulmonary involvement affects approximately 10% of patients and may be the first manifestation of the disease, putting the respiratory physician in a position to suspect and confirm the diagnosis. Besides interstitial lung disease and bronchial disorders, cough is a common symptom of the disease and particularly difficult to treat. Lung cysts and amyloid deposits, sometimes associated with lymphoma, have recently been described. The development of a primary pulmonary lymphoma, usually from MALT, is a major complication of the disease. VIEWPOINT: Characterisation of the pathophysiology of pulmonary involvement in Sjogren's syndrome and the institution of specific treatment merits the interest of the respiratory physician. CONCLUSION: The respiratory physician should consider the diagnosis of Sjogren's syndrome in many different clinico-pathological situations.
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Enfermedades Pulmonares/etiología , Síndrome de Sjögren/complicaciones , Humanos , Pulmón/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Interferon alpha is approved for the treatment of Kaposi's sarcoma in HIV infected patients. Hemolytic and uremic syndrome (HUS) is a rare side-effect of interferon alpha and has been reported primarily in chronic myelogenous leukemia. CASE REPORT: A 44-year-old HIV-infected woman from Cameroon was admitted for treatment of cutaneous Kaposi's sarcoma. Two days later, she presented severe HUS. Abdominal pains subsequently revealed non-occlusive mesenteric ischemia. The patient rapidly improved after interferon withdrawal. DISCUSSION: To our knowledge this is the first case of HUS induced by interferon alpha given for Kaposi's sarcoma. Further, no cases of acute mesenteric ischemia have been reported with interferon alpha. It is possible that the condition may have been induced or aggravated by HUS or a low infusion rate. Interferon can exert vascular toxicity on both the mesenteric vessels and the renal vessels in a setting of microangiopathy.
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Síndrome de Inmunodeficiencia Adquirida/complicaciones , Antineoplásicos/efectos adversos , Síndrome Hemolítico-Urémico/inducido químicamente , Interferón-alfa/efectos adversos , Isquemia/inducido químicamente , Mesenterio/irrigación sanguínea , Sarcoma de Kaposi/tratamiento farmacológico , Sarcoma de Kaposi/etiología , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/etiología , Enfermedad Aguda , Adulto , Femenino , Humanos , Interferón alfa-2 , Proteínas RecombinantesRESUMEN
Idiopathic interstitial pneumonias comprise 8 clinicopathological entities, most of them with a chronic course and various prognosis. Idiopathic pulmonary fibrosis is the most frequent and most severe of these. Computed tomography has an important role for its diagnosis. It can identify the corresponding pathological pattern of usual interstitial pneumonia in about 50 percent of cases. It can suggest differential diagnosis in other cases, most frequently fibrosing nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis. Imaging features should be integrated to clinical and available pathologic data during multidisciplinary team meetings involving physicians with a good knowledge of interstitial diseases. Some cases may be unclassifiable, but these could later be reclassified as new data may occur or imaging features may change. Surgical lung biopsy is being less frequently performed and an emerging less invasive technique, lung cryobiopsy, is under evaluation. Pleuroparenchymal fibroelastosis is a distinct entity only recently described, with uncertain prevalence and prognosis that seems being quite often associated to another pattern of interstitial pneumonia.
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Neumonías Intersticiales Idiopáticas/diagnóstico , Alveolitis Alérgica Extrínseca/clasificación , Alveolitis Alérgica Extrínseca/diagnóstico , Alveolitis Alérgica Extrínseca/epidemiología , Alveolitis Alérgica Extrínseca/patología , Biopsia , Diagnóstico Diferencial , Humanos , Neumonías Intersticiales Idiopáticas/clasificación , Neumonías Intersticiales Idiopáticas/epidemiología , Neumonías Intersticiales Idiopáticas/patología , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/epidemiología , Fibrosis Pulmonar Idiopática/patología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/patología , Valor Predictivo de las Pruebas , PronósticoRESUMEN
Lung cancer is the leading cause of cancer death worldwide. Prognosis and treatment outcomes are known to be related to the disease stage at the time of diagnosis. Therefore, an accurate assessment of the extent of disease is critical to determine the most appropriate therapy. Currently available imaging modalities for diagnosis and follow-up consist of morphological and functional imaging. Morphological investigations are mainly performed with CT-scan and in some cases with MRI. In this review, we describe the contribution of MRI in lung cancer staging focusing on solid pulmonary nodule characterization and TNM staging assessment using chest and whole-body MRI examinations, detailing in each chapter current recommendations and future developments.
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Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/patología , Imagen por Resonancia Magnética/métodos , Imagen de Difusión por Resonancia Magnética/métodos , Estudios de Seguimiento , Humanos , Interpretación de Imagen Asistida por Computador/métodos , Pulmón/diagnóstico por imagen , Pulmón/patología , Neoplasias Pulmonares/terapia , Estadificación de Neoplasias/métodos , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Pronóstico , Sensibilidad y Especificidad , Tomografía Computarizada por Rayos X/métodosRESUMEN
The influence of dietary fatty acids on Na+ sensitivity and ouabain affinity of Na+/K(+)-ATPase isoenzymes of whole brain membranes were studied in weaned rats fed for two generations with diets either devoid of alpha-linolenic acid (sunflower oil diet) or rich in alpha-linolenic acid (soya oil diet). The (n--3) deficiency induced by the sunflower oil diet led to an increase in the (n--6)/(n--3) molar ratio in whole brain membranes. Na+/K(+)-ATPase isoenzymes were discriminated on the basis of their differential affinities for ouabain. In rats fed sunflower oil diet, the ouabain titration displayed three inhibitory processes with markedly different affinities: low affinity (alpha 1); high affinity (alpha 2); and very high affinity (alpha 3). Membranes of rats fed soya oil diet exhibited only two inhibitory processes, i.e., low affinity (likely alpha 1+ alpha 2) and high affinity (likely alpha 2+ alpha 3) with the low affinity form intermediate between the sunflower alpha 1 and alpha 2 forms, and the high affinity form intermediate between the sunflower alpha 2 and alpha 3 forms. In fact, the Na+ response shows that the three isoenzymes have different Na+ sensitivities. Regardless of the diet, alpha 1 has a similar Na+ sensitivity (less than 1 mM), whilst alpha 2 and alpha 3 are more sensitive in soya oil membranes compared to sunflower oil membranes (5.1 vs. 7.2 mM and about 11 vs. 22.5 mM, respectively). Thus, sodium appears to be a better criterion of heterogeneity than ouabain.
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Encéfalo/efectos de los fármacos , Grasas Insaturadas en la Dieta/farmacología , Isoenzimas/metabolismo , Ácidos Linolénicos/farmacología , ATPasa Intercambiadora de Sodio-Potasio/metabolismo , Animales , Encéfalo/enzimología , Relación Dosis-Respuesta a Droga , Ácidos Grasos/análisis , Ácidos Linolénicos/deficiencia , Membranas/efectos de los fármacos , Membranas/enzimología , Ouabaína/farmacología , Aceites de Plantas/administración & dosificación , Ratas , Ratas Wistar , Sodio/farmacología , ATPasa Intercambiadora de Sodio-Potasio/antagonistas & inhibidores , Aceite de Soja/administración & dosificación , Aceite de GirasolRESUMEN
Epicardial fat necrosis is a rare cause of benign chest pain. Its physiopathological mechanism is unknown. Diagnosis is easily performed through radiological investigations that show a round opacity of fat density limited by a dense pseudo-capsule in the anterior mediastinum, close to the heart.