IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Hemoptysis in Primary Pulmonary Amyloidoma Treated with Intrabronchial Arterial Coiling Filiz Oezkan1, Amir Maqbul Khan1, Lutz Freitag1, Michael Montag3, Steffen Hahn2, Peter Johannes Berliner4, and Kaid Darwiche1 1 Department of Interventional Pulmonology, Ruhrlandklinik, West German Lung Center, and 2Department of Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, University Duisburg-Essen, Essen, Germany; 3Department of Radiology, Alfried Krupp Hospital, Essen, Germany; and 4Clinic of Diagnostic and Interventional Radiology, Guetersloh Hospital, Guetersloh, Germany
Figure 2. Chest CT scan showing multiple bipulmonary nodules of varying size from amyloidosis. The nodules show coarse marginal calcifications. Signs of pulmonary hemorrhage, such as ground-glass opacities, consolidations without calcifications, or Kerley B lines are not present. Figure 1. Posterior–anterior chest X-ray showing multifocal bipulmonary nodules with calcifications. Kerley B-lines and noncalcified consolidations as signs of pulmonary hemorrhage are absent.
Amyloidosis is the deposition of a variety of low-molecular-weight protein fibrils in intra- and extracellular tissue. Pulmonary manifestations of amyloidosis include tracheobronchial infiltration, persistent pleural effusions, and parenchymal nodules (amyloidomas). Primary amyloidomas of the lung with no systemic involvement are very rare. Pulmonary amyloidomas (with extensive parenchymal involvement) may remain stable. However, complications may occur (e.g., dyspnea with obstructive and/or restrictive physiology, postobstructive pneumonias, bleeding, etc.). Hemoptysis is a rare presentation. To date, surgical intervention is the treatment of choice. We present a rare case wherein extensive pulmonary amyloidomas resulted in life-threatening hemoptysis. This was treated with radiological interventional coiling of the bronchial arteries, and is the first reported amyloidoma case in English literature to date.
Am J Respir Crit Care Med Vol 190, Iss 11, pp 1311–1314, Dec 1, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201406-1009IM Internet address: www.atsjournals.org
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IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 3. Digital substraction angiogram after application of contrasting fluid via superselective coaxial microcatheter into the right bronchial artery is shown. In continuation of the right bronchial artery, an intense transfer of the contrast medium to the pulmonary arteries via systemic pulmonary arterial shunts can be detected. Coils in the left bronchial artery, placed 4 weeks earlier, are seen on the left side of the image.
Case Report A 78-year-old woman presented with complaints of hemoptysis. Her past medical history was significant for coronary artery disease, hypertension, hypercholesterolemia, chronic obstructive pulmonary disease (GOLD II), and hyperactive airway disease. She did not have any prior history of hypoxemia. Her previous negative workup included: blood count, rheumatologic workup (including antinuclear antibodies, rheumatoid factor, anti-Smith antibody, double-stranded DNA antibody, nuclear ribonucleoprotein, antineutrophil cytoplasmic antibodies, extractable nuclear antigens, anti–Ro/La etc.), C-reactive protein, liver function test, capillary blood gas analysis, echocardiography, ultrasound, and computed tomography scan of the abdomen/pelvis. Her past surgical history was significant for appendectomy, tonsillectomy, and hysterectomy. No occupational exposure or any allergies were reported. She was a former smoker (50 pack-years smoking history, abstinence for 10 yr). A prior diagnosis of amyloidosis was established 15 years ago with open lung biopsy after a failed transbronchial biopsy. Chest X-ray and chest CT scan revealed multiple bipulmonary nodules of varying size with coarse marginal calcifications (Figures 1 and 2). From a bronchoscopy,
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American Journal of Respiratory and Critical Care Medicine Volume 190 Number 11 | December 1 2014
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 4. Digital substraction angiogram after application of contrasting fluid via superselective coaxial microcatheter into the right bronchial artery is shown after coil embolization of the vessel. First coils are placed distal, the following ones more proximal. Cessation of blood flow distally is achieved after coil embolization. On the left side of the image, coils that were placed in the left bronchial artery 4 weeks earlier can be seen.
bleeding was identified from the left lower lobe of the lung and the patient underwent successful coil embolization of the left lower branch of the bronchial artery. As the patient presented with recurrent hemoptysis 4 weeks later, additional coil embolization of the right bronchial artery was performed (Figures 3 and 4). Her recovery was uneventful in the last 6 months.
Conclusions The two major forms of amyloidosis are the AL (primary) and AA (secondary) types. Biopsies are generally aimed at the dysfunctional organs (e.g., kidney, skin, etc.) or at clinically uninvolved sites such as subcutaneous fat, salivary glands, or rectal mucosa. Therapy is
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES aimed at the underlying infectious or inflammatory disorder in AA amyloidosis and at the underlying plasma cell dyscrasia in AL amyloidosis. Hemoptysis may present in pulmonary amyloidoma, and can be successfully treated with intrabronchial arterial coiling. Bronchial artery embolization (BAE) can be successfully repeated for recurrent bleeding (10). BAE, which was first reported by Remy and colleagues in 1973, has become an established procedure in the management of lifethreatening and/or recurrent hemoptysis in patients with infectious diseases or tumors affecting the lung (1). It is indicated when conservative medical treatment fails and surgery is not indicated due to poor pulmonary reserve, other comorbidities, or extensive bilateral pulmonary infiltrates, as present in this case (2, 4–6). BAE can either be performed with liquid embolic material (e.g., N-butyl cyanoacrylate), embolic particles (e.g., polyvinyl alcohol particles, gelatin sponges), or coils. Morbidity and mortality rates are better compared with surgery, and the interventional costs are lower (4, 7–9). After chest X-ray, CT scan, and flexible bronchoscopy, location of the bleeding is reached via digital substraction angiography using a coaxial superselective catheter (3, 9). In our case, the angiographic images showed prominent bronchial arteries with an intense transfer of the contrast medium to the pulmonary arteries via systemic pulmonary arterial shunts. More cases should be analyzed to determine if these findings are specific for amyloidoma. Possible complications are inguinal hematoma, lung hematoma (through perforation of the bronchial artery with the microcatheter), ischemia of central bronchi and the trachea if several central arteries are occluded at once, ischemia of other thoracic organs (heart, esophagus), and lung infarction (through migration of the coil to the pulmonary arterial periphery via systemic pulmonary arterial shunts). The most feared complication is an occlusion of the arteria radicularis magna, which is more likely when liquid embolic material or embolic particles are used. As radio-opacity is provided, coils can be placed more precisely than liquid or particle embolic material; therefore, complications are rare and less severe (10, 11). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Remy J, Voisin C, Ribet M, Dupuis C, Beguery P, Tonnel AB, Douay B, Pagniez B, Denies JL. Treatment, by embolization, of severe or repeated hemoptysis associated with systemic hypervascularization [in French]. Nouv Presse Med 1973;2: 2060–2068. 2. Yoon W, Kim JK, Kim YH, Chung TW, Kang HK. Bronchial and nonbronchial systemic artery embolization for life-threatening hemoptysis: a comprehensive review. Radiographics 2002; 22:1395–1409. 3. Yoo DH, Yoon CJ, Kang SG, Burke CT, Lee JH, Lee CT. Bronchial and nonbronchial systemic artery embolization in patients with major hemoptysis: safety and efficacy of N-butyl cyanoacrylate. AJR Am J Roentgenol 2011;196:W199-204. 4. Lee TW, Wan S, Choy DK, Chan M, Arifi A, Yim AP. Management of massive hemoptysis: a single institution experience. Ann Thorac Cardiovasc Surg 2000;6:232–235.
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5. Marshall TJ, Jackson JE. Vascular intervention in the thorax: bronchial artery embolization for haemoptysis. Eur Radiol 1997;7:1221–1227. 6. Roberts AC. Bronchial artery embolization therapy. J Thorac Imaging 1990;5:60–72. 7. Jean-Baptiste E. Clinical assessment and management of massive hemoptysis. Crit Care Med 2000;28:1642–1647. 8. Klamut M, Szczerbo-Trojanowska M, Tillmann U. Catheter embolization of the thoracic arteries in the treatment of lung hemorrhage [in German]. Rofo 1985;143:645–650. 9. Andersen PE. Imaging and interventional radiological treatment of hemoptysis. Acta Radiol 2006;47:780–792. 10. Swanson KL, Johnson CM, Prakash UBS, McKusick MA, Andrews JC, Stanson AW. Bronchial artery embolization : experience with 54 patients. Chest 2002;121:789–795. 11. Nistri M, Acquafresca M, Pratesi A, Menchi I, Villari N, Mascalchi M. Bronchial artery embolization with detachable coils for the treatment of haemoptysis. Preliminary experience. Radiol Med (Torino) 2008; 113:452–460.
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 11 | December 1 2014
Figure 2. Chest CT scan showing multiple bipulmonary nodules of varying size from amyloidosis. The nodules show coarse marginal calcifications. Signs of pulmonary hemorrhage, such as ground-glass opacities, consolidations without calcifications, or Kerley B lines are not present. Figure 1. Posterior–anterior chest X-ray showing multifocal bipulmonary nodules with calcifications. Kerley B-lines and noncalcified consolidations as signs of pulmonary hemorrhage are absent.
Amyloidosis is the deposition of a variety of low-molecular-weight protein fibrils in intra- and extracellular tissue. Pulmonary manifestations of amyloidosis include tracheobronchial infiltration, persistent pleural effusions, and parenchymal nodules (amyloidomas). Primary amyloidomas of the lung with no systemic involvement are very rare. Pulmonary amyloidomas (with extensive parenchymal involvement) may remain stable. However, complications may occur (e.g., dyspnea with obstructive and/or restrictive physiology, postobstructive pneumonias, bleeding, etc.). Hemoptysis is a rare presentation. To date, surgical intervention is the treatment of choice. We present a rare case wherein extensive pulmonary amyloidomas resulted in life-threatening hemoptysis. This was treated with radiological interventional coiling of the bronchial arteries, and is the first reported amyloidoma case in English literature to date.
Am J Respir Crit Care Med Vol 190, Iss 11, pp 1311–1314, Dec 1, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201406-1009IM Internet address: www.atsjournals.org
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
1311
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 3. Digital substraction angiogram after application of contrasting fluid via superselective coaxial microcatheter into the right bronchial artery is shown. In continuation of the right bronchial artery, an intense transfer of the contrast medium to the pulmonary arteries via systemic pulmonary arterial shunts can be detected. Coils in the left bronchial artery, placed 4 weeks earlier, are seen on the left side of the image.
Case Report A 78-year-old woman presented with complaints of hemoptysis. Her past medical history was significant for coronary artery disease, hypertension, hypercholesterolemia, chronic obstructive pulmonary disease (GOLD II), and hyperactive airway disease. She did not have any prior history of hypoxemia. Her previous negative workup included: blood count, rheumatologic workup (including antinuclear antibodies, rheumatoid factor, anti-Smith antibody, double-stranded DNA antibody, nuclear ribonucleoprotein, antineutrophil cytoplasmic antibodies, extractable nuclear antigens, anti–Ro/La etc.), C-reactive protein, liver function test, capillary blood gas analysis, echocardiography, ultrasound, and computed tomography scan of the abdomen/pelvis. Her past surgical history was significant for appendectomy, tonsillectomy, and hysterectomy. No occupational exposure or any allergies were reported. She was a former smoker (50 pack-years smoking history, abstinence for 10 yr). A prior diagnosis of amyloidosis was established 15 years ago with open lung biopsy after a failed transbronchial biopsy. Chest X-ray and chest CT scan revealed multiple bipulmonary nodules of varying size with coarse marginal calcifications (Figures 1 and 2). From a bronchoscopy,
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American Journal of Respiratory and Critical Care Medicine Volume 190 Number 11 | December 1 2014
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 4. Digital substraction angiogram after application of contrasting fluid via superselective coaxial microcatheter into the right bronchial artery is shown after coil embolization of the vessel. First coils are placed distal, the following ones more proximal. Cessation of blood flow distally is achieved after coil embolization. On the left side of the image, coils that were placed in the left bronchial artery 4 weeks earlier can be seen.
bleeding was identified from the left lower lobe of the lung and the patient underwent successful coil embolization of the left lower branch of the bronchial artery. As the patient presented with recurrent hemoptysis 4 weeks later, additional coil embolization of the right bronchial artery was performed (Figures 3 and 4). Her recovery was uneventful in the last 6 months.
Conclusions The two major forms of amyloidosis are the AL (primary) and AA (secondary) types. Biopsies are generally aimed at the dysfunctional organs (e.g., kidney, skin, etc.) or at clinically uninvolved sites such as subcutaneous fat, salivary glands, or rectal mucosa. Therapy is
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
1313
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES aimed at the underlying infectious or inflammatory disorder in AA amyloidosis and at the underlying plasma cell dyscrasia in AL amyloidosis. Hemoptysis may present in pulmonary amyloidoma, and can be successfully treated with intrabronchial arterial coiling. Bronchial artery embolization (BAE) can be successfully repeated for recurrent bleeding (10). BAE, which was first reported by Remy and colleagues in 1973, has become an established procedure in the management of lifethreatening and/or recurrent hemoptysis in patients with infectious diseases or tumors affecting the lung (1). It is indicated when conservative medical treatment fails and surgery is not indicated due to poor pulmonary reserve, other comorbidities, or extensive bilateral pulmonary infiltrates, as present in this case (2, 4–6). BAE can either be performed with liquid embolic material (e.g., N-butyl cyanoacrylate), embolic particles (e.g., polyvinyl alcohol particles, gelatin sponges), or coils. Morbidity and mortality rates are better compared with surgery, and the interventional costs are lower (4, 7–9). After chest X-ray, CT scan, and flexible bronchoscopy, location of the bleeding is reached via digital substraction angiography using a coaxial superselective catheter (3, 9). In our case, the angiographic images showed prominent bronchial arteries with an intense transfer of the contrast medium to the pulmonary arteries via systemic pulmonary arterial shunts. More cases should be analyzed to determine if these findings are specific for amyloidoma. Possible complications are inguinal hematoma, lung hematoma (through perforation of the bronchial artery with the microcatheter), ischemia of central bronchi and the trachea if several central arteries are occluded at once, ischemia of other thoracic organs (heart, esophagus), and lung infarction (through migration of the coil to the pulmonary arterial periphery via systemic pulmonary arterial shunts). The most feared complication is an occlusion of the arteria radicularis magna, which is more likely when liquid embolic material or embolic particles are used. As radio-opacity is provided, coils can be placed more precisely than liquid or particle embolic material; therefore, complications are rare and less severe (10, 11). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Remy J, Voisin C, Ribet M, Dupuis C, Beguery P, Tonnel AB, Douay B, Pagniez B, Denies JL. Treatment, by embolization, of severe or repeated hemoptysis associated with systemic hypervascularization [in French]. Nouv Presse Med 1973;2: 2060–2068. 2. Yoon W, Kim JK, Kim YH, Chung TW, Kang HK. Bronchial and nonbronchial systemic artery embolization for life-threatening hemoptysis: a comprehensive review. Radiographics 2002; 22:1395–1409. 3. Yoo DH, Yoon CJ, Kang SG, Burke CT, Lee JH, Lee CT. Bronchial and nonbronchial systemic artery embolization in patients with major hemoptysis: safety and efficacy of N-butyl cyanoacrylate. AJR Am J Roentgenol 2011;196:W199-204. 4. Lee TW, Wan S, Choy DK, Chan M, Arifi A, Yim AP. Management of massive hemoptysis: a single institution experience. Ann Thorac Cardiovasc Surg 2000;6:232–235.
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5. Marshall TJ, Jackson JE. Vascular intervention in the thorax: bronchial artery embolization for haemoptysis. Eur Radiol 1997;7:1221–1227. 6. Roberts AC. Bronchial artery embolization therapy. J Thorac Imaging 1990;5:60–72. 7. Jean-Baptiste E. Clinical assessment and management of massive hemoptysis. Crit Care Med 2000;28:1642–1647. 8. Klamut M, Szczerbo-Trojanowska M, Tillmann U. Catheter embolization of the thoracic arteries in the treatment of lung hemorrhage [in German]. Rofo 1985;143:645–650. 9. Andersen PE. Imaging and interventional radiological treatment of hemoptysis. Acta Radiol 2006;47:780–792. 10. Swanson KL, Johnson CM, Prakash UBS, McKusick MA, Andrews JC, Stanson AW. Bronchial artery embolization : experience with 54 patients. Chest 2002;121:789–795. 11. Nistri M, Acquafresca M, Pratesi A, Menchi I, Villari N, Mascalchi M. Bronchial artery embolization with detachable coils for the treatment of haemoptysis. Preliminary experience. Radiol Med (Torino) 2008; 113:452–460.
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 11 | December 1 2014
