ABSTRACT
Sarcoidosis is a disease of unknown etiology affecting most frequently young adults. Diagnosis is based on clinical, radiological and histopathological features and presentation is frequently of constitutional and respiratory symptoms, hilar and mediastinal adenopathies, with or without parenquimal involvement. Confirmation is histopathological with demonstration of non-caseificating granulomas in affected organs, with negative cultures for mycobacteria and fungi. We describe the clinical, radiological and histopathological findings in 8 patients. Mean age was 44 years (28-59) and half the patients were male. Two patients were symptom-free at diagnosis, thoracic findings were present in five (cough, retroesternal pain and arrhythmias) and one had extrathoracic symptoms (ankle arthritis). All showed mediastinal adenopathies in radiological and tomographic images (six were in stage 2 and two in stage 1). Two patients had spontaneous remission, four received oral steroids, one required oral steroids and azathioprine and there was no treatment data on one patient. In one patient, recidiva of severe and bilateral erithema nodosum required long term treatment with steroids and immunosupressors. Although sarcoidosis is a low prevalent disease in our country, a high index of suspicion is advisable when facing patients with respiratory symptoms, parenquimal involvement and mediastinal adenopathies, with or without extrathoracic manifestations.
Subject(s)
Sarcoidosis, Pulmonary , Adult , Diagnosis, Differential , Female , Glucocorticoids/therapeutic use , Granuloma/diagnostic imaging , Granuloma/pathology , Humans , Lymphatic Diseases/diagnostic imaging , Lymphatic Diseases/pathology , Male , Mediastinal Diseases/diagnostic imaging , Mediastinal Diseases/pathology , Middle Aged , Prednisone/therapeutic use , Prognosis , Pulmonary Fibrosis/diagnostic imaging , Pulmonary Fibrosis/pathology , Radiography , Sarcoidosis, Pulmonary/diagnostic imaging , Sarcoidosis, Pulmonary/drug therapy , Sarcoidosis, Pulmonary/pathologyABSTRACT
La sarcoidosis es una enfermedad de etiología desconocida, que afecta con mayor frecuencia a adultos jóvenes. El diagnóstico es clínico, radiológico e histopatológico y la presentación frecuente es la de síntomas constitucionales y respiratorios, acompañados de linfoadenopatías hiliares y mediastinales, con o sin compromiso del parénquima pulmonar. La confirmación es anatomopatológica con la demostración de granulomas no caseificantes en los tejidos afectados, con cultivos negativos para micobacterias y hongos. Presentamos los hallazgos clínicos, radiológicos e histopatológicos en 8 pacientes. Cuatro eran de sexo masculino y la edad promedio fue de 44 años (28-59). Dos de los pacientes se encontraban asintomáticos al momento del diagnóstico, cinco de ellos se presentaron con hallazgos toráxicos (tos, dolor retroesternal y arritmias) y uno con síntomas extratoráxicos (artritis de ambos tobillos). Todos presentaron adenopatías mediastinales en los estudios radiológicos y tomográficos y seis de ellos se encontraban en el estadio 2. En cuanto al tratamiento, dos pacientes remitieron espontáneamente, cuatro pacientes recibieron prednisona, uno requirió prednisona y azatioprina y de un paciente no contamos con registros de tratamiento. Uno de nuestros pacientes evolucionó desfavorablemente con aparición y recidiva de lesiones de eritema nodoso severas y bilaterales que motivaron la necesidad de tratamiento permanente con esteroides e inmunosupresores. Si bien se trata de una enfermedad de baja prevalencia en nuestro país, deberíamos mantener un alto índice de sospecha ante la presentación de síntomas respiratorios con lesiones parenquimatosas y adenopatías mediastinales, con o sin manifestaciones extratorácicas.
Sarcoidosis is a disease of unknown etiology affecting most frequently young adults. Diagnosis is based on clinical. Radiological and histopathological features and presentation is frequently of constitutional and respiratory symptoms, hilar and mediastinal adenopathies, with or without parenquimal involvement. Confirmation is histopathological with demonstration of non-caseificating granulomas in affected organs, with negative cultures for mycobacterium and fungi. We describe the clinical, radiological and histopatbological findings in 8 patients. Mean age was 44 years (28-59) and half the patients were male. Two patients were symptom-free at diagnosis, thoracic findings were present in five (cough, retroesternal pain and arrhytbmias) and one had extratboracic symptoms (ankle arthritis). All showed mediastinal adenopathies in radiological and tomographic images (six were in stage 2 and two in stage 1). 1/vo patients had spontaneous remission, four received oral steroids, one required oral steroids and azatbioprine and tbere was no treatment data on one patient. In one patient, recidiva of severe and bilateral erithema nodosum required long term treatment with steroids and immunosupressors. Although sarcoidosis is a low prevalent disease in our country, a high index of suspicion is advisable when facing patients with respiratory symptoms, parenquimal involvement and mediastinal adenopathies, with or without extrathoracic manifestations.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Sarcoidosis, Pulmonary/pathology , Sarcoidosis, Pulmonary , Glucocorticoids/therapeutic use , Granuloma/pathology , Granuloma , Mediastinal Diseases/pathology , Mediastinal Diseases , Prognosis , Prednisone/therapeutic use , Pulmonary Fibrosis/pathology , Pulmonary Fibrosis , Sarcoidosis, Pulmonary/drug therapyABSTRACT
La sarcoidosis es una enfermedad de etiología desconocida, que afecta con mayor frecuencia a adultos jóvenes. El diagnóstico es clínico, radiológico e histopatológico y la presentación frecuente es la de síntomas constitucionales y respiratorios, acompañados de linfoadenopatías hiliares y mediastinales, con o sin compromiso del parénquima pulmonar. La confirmación es anatomopatológica con la demostración de granulomas no caseificantes en los tejidos afectados, con cultivos negativos para micobacterias y hongos. Presentamos los hallazgos clínicos, radiológicos e histopatológicos en 8 pacientes. Cuatro eran de sexo masculino y la edad promedio fue de 44 años (28-59). Dos de los pacientes se encontraban asintomáticos al momento del diagnóstico, cinco de ellos se presentaron con hallazgos toráxicos (tos, dolor retroesternal y arritmias) y uno con síntomas extratoráxicos (artritis de ambos tobillos). Todos presentaron adenopatías mediastinales en los estudios radiológicos y tomográficos y seis de ellos se encontraban en el estadio 2. En cuanto al tratamiento, dos pacientes remitieron espontáneamente, cuatro pacientes recibieron prednisona, uno requirió prednisona y azatioprina y de un paciente no contamos con registros de tratamiento. Uno de nuestros pacientes evolucionó desfavorablemente con aparición y recidiva de lesiones de eritema nodoso severas y bilaterales que motivaron la necesidad de tratamiento permanente con esteroides e inmunosupresores. Si bien se trata de una enfermedad de baja prevalencia en nuestro país, deberíamos mantener un alto índice de sospecha ante la presentación de síntomas respiratorios con lesiones parenquimatosas y adenopatías mediastinales, con o sin manifestaciones extratorácicas.(AU)
Sarcoidosis is a disease of unknown etiology affecting most frequently young adults. Diagnosis is based on clinical. Radiological and histopathological features and presentation is frequently of constitutional and respiratory symptoms, hilar and mediastinal adenopathies, with or without parenquimal involvement. Confirmation is histopathological with demonstration of non-caseificating granulomas in affected organs, with negative cultures for mycobacterium and fungi. We describe the clinical, radiological and histopatbological findings in 8 patients. Mean age was 44 years (28-59) and half the patients were male. Two patients were symptom-free at diagnosis, thoracic findings were present in five (cough, retroesternal pain and arrhytbmias) and one had extratboracic symptoms (ankle arthritis). All showed mediastinal adenopathies in radiological and tomographic images (six were in stage 2 and two in stage 1). 1\vo patients had spontaneous remission, four received oral steroids, one required oral steroids and azatbioprine and tbere was no treatment data on one patient. In one patient, recidiva of severe and bilateral erithema nodosum required long term treatment with steroids and immunosupressors. Although sarcoidosis is a low prevalent disease in our country, a high index of suspicion is advisable when facing patients with respiratory symptoms, parenquimal involvement and mediastinal adenopathies, with or without extrathoracic manifestations.(AU)
