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Sclerosing mediastinitis is a rare and benign disease, which can cause obstruction of mediastinal structures, this determines the clinical presentation and prognosis. The obstruction of pulmonary venous structures secondary to mediastinal fibrosis has been rarely reported, clinically it mimics mitral stenosis. We describe the clinical case of a young patient with idiopathic sclerosing mediastinitis. The radiologic exams found stenosis of the right pulmonary artery, the right bronchus and pulmonary veins. Through this observation, we discuss this rare entity that although benign, can also be deadly. We study the different etiologies, clinical and therapeutic possibilities.
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