ABSTRACT
BACKGROUND: Acute exacerbation of interstitial lung disease (AE-ILD) is a severe complication with a poor prognosis. No clinical trials have supported the use of rituximab in AE-ILD associated with connective tissue disease. METHODS: We present a series of four cases in which administration of rituximab was associated with appropriate clinical, radiological and functional progress. RESULTS: The four patients were alive 30 days after discharge following their exacerbation. CONCLUSIONS: Given the speed of action, safety and efficacy profile observed for rituximab, we believe that this agent should be further investigated in clinical trials so that it could be included in the daily clinical management of this severe condition.
Subject(s)
Connective Tissue Diseases , Lung Diseases, Interstitial , Humans , Rituximab/therapeutic use , Connective Tissue Diseases/complications , Connective Tissue Diseases/diagnosis , Connective Tissue Diseases/drug therapy , Lung Diseases, Interstitial/complications , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/drug therapy , Patient DischargeABSTRACT
La fibrosis pulmonar idiopática se define como una neumonía intersticial fibrosante crónica progresiva de etiología desconocida. Existen factores de riesgo, tanto intrínsecos como extrínsecos, que pueden fomentar el desarrollo de la enfermedad en individuos con predisposición genética. El diagnóstico se realiza mediante hallazgos radiológicos y/o histológicos característicos en la tomografía axial computarizada de alta resolución y la biopsia pulmonar, respectivamente, en ausencia de causa específica identificable. La supervivencia media de la enfermedad sin tratamiento es de 3-5años desde el inicio de los síntomas, aunque su historia natural es variable e impredecible. Actualmente existen dos fármacos antifibróticos que enlentecen la progresión de la enfermedad. El abordaje multidisciplinar considerará el estado nutricional, el estado emocional, el acondicionamiento físico y el tratamiento de comorbilidades, así como el trasplante pulmonar y los cuidados paliativos en fases avanzadas. En este artículo se revisan los aspectos fundamentales para el diagnóstico y el tratamiento de la fibrosis pulmonar idiopática. (AU)
Idiopathic pulmonary fibrosis is defined as a chronic progressive fibrosing interstitial pneumonia of unknown etiology. There are intrinsic and extrinsic risk factors that could favor the development of the disease in individuals with a genetic predisposition. The diagnosis is made by characteristic radiological and/or histological findings on high-resolution computed tomography and lung biopsy, respectively, in the absence of a specific identifiable cause. The median survival of the disease for patients without treatment is 3-5years from the onset of symptoms, although its natural history is variable and unpredictable. Currently, there are two antifibrotic drugs that reduce disease progression. The multidisciplinary approach will consider the nutritional and emotional status, physical conditioning, and treatment of comorbidities, as well as lung transplantation and palliative care in advanced stages. The following article reviews the fundamental aspects for the diagnosis and treatment of idiopathic pulmonary fibrosis. (AU)
Subject(s)
Humans , Biopsy/adverse effects , Idiopathic Pulmonary Fibrosis/diagnosis , Idiopathic Pulmonary Fibrosis/etiology , Idiopathic Pulmonary Fibrosis/therapy , Lung Transplantation , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/etiology , Lung Diseases, Interstitial/therapy , Diagnosis , Therapeutics , Lung/pathologyABSTRACT
Idiopathic pulmonary fibrosis is defined as a chronic progressive fibrosing interstitial pneumonia of unknown etiology. There are intrinsic and extrinsic risk factors that could favor the development of the disease in individuals with a genetic predisposition. The diagnosis is made by characteristic radiological and/or histological findings on high-resolution computed tomography and lung biopsy, respectively, in the absence of a specific identifiable cause. The median survival of the disease for patients without treatment is 3-5years from the onset of symptoms, although its natural history is variable and unpredictable. Currently, there are two antifibrotic drugs that reduce disease progression. The multidisciplinary approach will consider the nutritional and emotional status, physical conditioning, and treatment of comorbidities, as well as lung transplantation and palliative care in advanced stages. The following article reviews the fundamental aspects for the diagnosis and treatment of idiopathic pulmonary fibrosis.
Subject(s)
Idiopathic Pulmonary Fibrosis , Lung Diseases, Interstitial , Lung Transplantation , Biopsy/adverse effects , Humans , Idiopathic Pulmonary Fibrosis/diagnosis , Idiopathic Pulmonary Fibrosis/etiology , Idiopathic Pulmonary Fibrosis/therapy , Lung/pathology , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/etiology , Lung Diseases, Interstitial/therapy , Tomography, X-Ray Computed/methodsABSTRACT
No disponible
