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Vol. 40. Issue 12.
Pages 595-598 (December 2004)
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Vol. 40. Issue 12.
Pages 595-598 (December 2004)
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Osificación pulmonar difusa asociada a fibrosis pulmonar idiopática
Diffuse Pulmonary Ossification Associated With Idiopathic Pulmonary Fibrosis
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C.A. Fernández Crisostoa,
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cafedoc007@tutopia.com

Correspondencia: Avda. Patria, 656.X5004AAP Córdoba. Argentina.
, O. Quercia Ariasb, N. Bustamantea, H. Morenoa, A. Uribe Echevarríaa
a Servicio de Cirugía de Tórax y Cardiovascular. Hospital de Córdoba. Córdoba.
b Servicio de Neumonología. Hospital de Córdoba. Córdoba. Argentina.
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La osificación pulmonar difusa es una rara entidad que consiste en la formación de hueso maduro en el parénquima pulmonar, asociada a patología pulmonar difusa y crónica, cardíaca o extracardiopulmonar. Esta entidad constituye habitualmente un hallazgo anatomopatológico post mortem. En este caso se realiza el diagnóstico mediante biopsia pulmonar a cielo abierto

Presentamos el caso de un varón de 79 años, con disnea, tos seca y pérdida de peso. Había sido fumador. En la radiografía de tórax se apreciaba un infiltrado pulmonar bilateral reticulonodulillar. La tomografía computarizada evidenció afectación intersticial con predominio septal y múltiples cavidades con tendencia a la panalización; engrosamiento pleural, retracción del parénquima y fibrosis bilateral. Se estableció el diagnóstico clínico de fibrosis intersticial idiopática, y el paciente evolucionó desfavorablemente. Se realizó una biopsia a cielo abierto. La biopsia pulmonar evidenció zonas de colapso alveolar y otras enfisematosas, algunas con secreción y extravasación eritrocitaria. Había vasocongestión intersticial; los bronquiolos presentaban infiltrado inflamatorio mononuclear y algunos polimorfonucleares. Llamaba la atención la presencia de trabéculas óseas, algunas que incluían la médula ósea, de tipo adiposo, en focos multicéntricos, predominantemente intersticiales

La osificación pulmonar difusa constituye habitualmente un hallazgo incidental en autopsias de pacientes con antecedentes de enfermedad pulmonar crónica difusa, siendo inusual el diagnóstico en un paciente vivo. La osificación pulmonar difusa no posee significación pronóstica en la fibrosis pulmonar. Constituye un signo de cronicidad y gravedad de la enfermedad

Palabras clave:
Osificación pulmonar dendriforme
Fibrosis pulmonar idiopática
Biopsia pulmonar

Diffuse pulmonary ossification is a rare entity that presents with the formation of mature bone in the pulmonary parenchyma and is associated with diffuse and chronic lung disease, heart disease, or other system disorders. Diffuse pulmonary ossification is usually a postmortem finding by the pathologist. In the case we report, the diagnosis was established by open lung biopsy. The patient was a 79- year-old man with dyspnea, dry cough, and weight loss. He had been a smoker. A chest x-ray revealed reticulonodular bilateral pulmonary infiltrates. Computed tomography revealed interstitial disease predominantly in the septum with multiple cavitations that tended to form honeycomb patterns. Pleural thickening, retraction of the parenchyma, and bilateral fibrosis were also visible. A clinical diagnosis of interstitial fibrosis was established and the patient's course was unfavorable. An open lung biopsy was performed. The lung tissue specimens revealed zones with collapsed alveoli and others with emphysema, some of which produced secretion and erythrocytic extravasation. Interstitial vascular congestion was apparent; bronchioles presented mononuclear and some polymorphonuclear inflammatory infiltrates. Noteworthy was the presence of predominantly interstitial, multicentric foci of osseous trabeculae —some of which included adipose bone marrow. Diffuse pulmonary ossification is usually an incidental finding in autopsies of patients with a history of diffuse chronic pulmonary disease, but it is an unusual diagnosis in living patients. Diffuse pulmonary ossification is of no prognostic significance in pulmonary fibrosis. It is a marker of the chronicity and/or severity of the fibrosis

Keywords:
Dendriform pulmonary ossification
Idiopathic pulmonary fibrosis
Lung biopsy
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Copyright © 2004. Sociedad Española de Neumología y Cirugía Torácica
Archivos de Bronconeumología
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