IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Pulmonary Arteriovenous Malformations in the Setting of Pulmonary Amyloidosis Ujval B. Patel, Michael A. Kadoch, and Adam H. Jacobi Department of Radiology, Mount Sinai Medical Center, New York, New York
Figure 1. (A) Frontal radiograph of the chest demonstrates well-circumscribed lung masses bilaterally. (B) Follow-up radiograph obtained 5 years later demonstrates interval progression in the number and size of the bilateral lung masses.
An 80-year-old male was diagnosed with pulmonary amyloidosis. The diagnosis was made in the 1970s after he presented with recurrent episodes of pneumonia. At that time, a bronchoscopy with transbronchial biopsies was performed, which showed green birefringence on Congo red stain under polarized light. After the diagnosis, he has been followed periodically with chest X-rays. During a recent hospital admission with hypoxia, computed tomographic angiography of the chest showed several lung amyloidomas with interval development of new pulmonary arteriovenous malformations. An initial radiograph of the chest demonstrated bilateral, well-circumscribed lung masses that had increased in size and number from a study performed 5 years earlier (Figures 1A and 1B). Computed tomographic angiography demonstrated multiple bilateral pulmonary nodules and amyloidomas, with the largest measuring 14.3 cm. Pulmonary arteriovenous malformations were seen within two amyloidomas at the left lung base (Figures 2A–2F), which were likely the cause of the patient’s hypoxia via right-to-left shunting. Although amyloid deposition within vessel walls is a well-recognized feature of this disease, amyloidomas containing pulmonary arteriovenous malformations are exceedingly rare and have been described in only one other case study to date (1). n Author disclosures are available with the text of this article at www.atsjournals.org.
Reference 1. Kamei K, Kusumoto K, Suzuki T. Pulmonary amyloidosis with pulmonary arteriovenous fistula. Chest 1989;96:1435–1436.
Am J Respir Crit Care Med Vol 190, Iss 4, pp e14–e15, Aug 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201310-1883IM Internet address: www.atsjournals.org
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American Journal of Respiratory and Critical Care Medicine Volume 190 Number 4 | August 15 2014
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 2. (A–F) Computed tomographic angiography of the chest with axial images at three different levels in lung and vascular windows demonstrates multiple bilateral pulmonary nodules and amyloidomas with scattered calcifications with the largest measuring up to 14.3 cm. Images are at the level of the left pulmonary artery (A and B), right pulmonary artery (C and D), and the cardiac apex (E and F). Pulmonary arteriovenous malformations (D, arrow at feeding artery; F, circle at nidus) are seen within two amyloidomas at the left lung base.
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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Figure 1. (A) Frontal radiograph of the chest demonstrates well-circumscribed lung masses bilaterally. (B) Follow-up radiograph obtained 5 years later demonstrates interval progression in the number and size of the bilateral lung masses.
An 80-year-old male was diagnosed with pulmonary amyloidosis. The diagnosis was made in the 1970s after he presented with recurrent episodes of pneumonia. At that time, a bronchoscopy with transbronchial biopsies was performed, which showed green birefringence on Congo red stain under polarized light. After the diagnosis, he has been followed periodically with chest X-rays. During a recent hospital admission with hypoxia, computed tomographic angiography of the chest showed several lung amyloidomas with interval development of new pulmonary arteriovenous malformations. An initial radiograph of the chest demonstrated bilateral, well-circumscribed lung masses that had increased in size and number from a study performed 5 years earlier (Figures 1A and 1B). Computed tomographic angiography demonstrated multiple bilateral pulmonary nodules and amyloidomas, with the largest measuring 14.3 cm. Pulmonary arteriovenous malformations were seen within two amyloidomas at the left lung base (Figures 2A–2F), which were likely the cause of the patient’s hypoxia via right-to-left shunting. Although amyloid deposition within vessel walls is a well-recognized feature of this disease, amyloidomas containing pulmonary arteriovenous malformations are exceedingly rare and have been described in only one other case study to date (1). n Author disclosures are available with the text of this article at www.atsjournals.org.
Reference 1. Kamei K, Kusumoto K, Suzuki T. Pulmonary amyloidosis with pulmonary arteriovenous fistula. Chest 1989;96:1435–1436.
Am J Respir Crit Care Med Vol 190, Iss 4, pp e14–e15, Aug 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201310-1883IM Internet address: www.atsjournals.org
e14
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 4 | August 15 2014
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 2. (A–F) Computed tomographic angiography of the chest with axial images at three different levels in lung and vascular windows demonstrates multiple bilateral pulmonary nodules and amyloidomas with scattered calcifications with the largest measuring up to 14.3 cm. Images are at the level of the left pulmonary artery (A and B), right pulmonary artery (C and D), and the cardiac apex (E and F). Pulmonary arteriovenous malformations (D, arrow at feeding artery; F, circle at nidus) are seen within two amyloidomas at the left lung base.
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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