European Journal of Radiology 44 (2002) 37 – 39 www.elsevier.com/locate/ejrad
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Primary diffuse tracheobrochial amyloidosis: case report Caner O8 zer *, Meltem Nass Duce, Altan Yildiz, F. Demir Apaydin, Hulusi Eg˘ilmez, Taner Arpaci Department of Radiology, Faculty of Medicine, Mersin Uni6ersity, Zeytinlibahc¸e Cad. Eski Otogar Yanı 33070 Mersin, Turkey Received 18 May 2001; received in revised form 28 September 2001; accepted 1 October 2001
Abstract Respiratory amyloidosis is a rare disease that occurs in three forms: tracheobronchial, nodular parenchymal and diffuse parenchymal involment. Tracheobronchial amyloidosis is characterized by focal or diffuse deposition of amyloid in the submucosa of the trachea and proximal bronchi. Herein, we report a case of diffuse tracheobronchial amyloidosis with plain radiography and thorax computed tomography findings. © 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Amyloidosis; Tracheobronchial; Computed tomography
1. Introduction Amyloidosis is a rare disease caused by extracellular deposition of amyloid, which is an inert, eosinophilic, proteinaceous material [1]. Primary pulmonary amyloidosis occurs in three forms: tracheobronchial, nodular parenchymal and diffuse parenchymal or alveolar septal [2]. Tracheobronchial amyloidosis is the most common form of primary pulmonary amyloidosis. Tracheobronchial amyloidosis characteristically demonstrates diffuse involvement of the tracheobronchial tree with submucosal deposition of amyloid plaques and nodules that may cause atrophy of the normal wall structures [3].
2. Case report A 54-year-old woman had wheezing, stridor, and hemoptysis. She presented in good health with mild resting dyspnea without worsening on exertion. On physical examination, she was obese, and had a predominant inspiratory stridor. Laryngeal examination did not reveal any finding which could explain the stridor. The lung function test showed limitation of the * Corresponding author. Tel.: +90-324-337-4300x1505; fax: +90324-337-4305. E-mail address:
[email protected] (C. O8 zer).
peak inspiratory and expiratory flow rates. Flow volume curve was consistent with central airway stenosis. Chest radiograph showed diffuse narrowing of the trachea, but the lungs were clear (Fig. 1). Coronal reformatted computed tomography (CT) images (Somatom AR Star, Siemens, Erlangen, Germany; 130 mAs, 83 kV, 3-mm slice thickness, 3-mm interval) showed diffuse thickening of the trachea and bilateral main bronchi. Mural nodulation of the tracheobronchial wall was visible (Fig. 2). On axial images, there was neither evidence of nodular or diffuse parenchymal involvement, nor postobstructive parenchymal abnormalities (Fig. 3a and b). Fiberoptic rigid bronchoscopy showed a completely irregular surface of the tracheal mucosa with prominent reddish plaques, ulcerative changes and bleeding tendency. In addition, concentric irregular narrowing of both main bronchi was detected. Biopsy specimen of the tracheal epitelium showed amyloid deposition. There was no clinical or laboratory evidence of systemic amyloidosis.
3. Discussion Amyloidosis is characterized by the deposition of a histochemically specific substance in many tissues. It is usually recognised by its staining reaction with Congo Red and subsequent demonstration of green dichroism
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when examined under polarising light [4]. Amyloid can infiltrate virtually all organ systems and may occur either idiopathically without underlying disease, thus termed primary amyloidosis, or as a part of a widespread systemic disease in association with inflammatory, hereditary (e.g. familial Mediterranean fever), or neoplastic conditions (especially of the myeloproliferative system), termed secondary amyloidosis [5– 7]. The first report of amyloid localised to the lower respiratory tract was described by Lesser in 1877 [4]. Tracheobronchial amyloidosis is the most common form of primary pulmonary amyloidosis which occurs almost exclusively in the absence of the systemic amyloidosis. The cause is unknown. Males are more often affected by the disease than females [7]. Tracheobronchial amyloidosis characteristically demonstrates diffuse or focal involvement of the tracheobronchial tree with submucosal deposition of amyloid plaques and nodules, that may cause atrophy of the normal wall structures [3,7,8]. The focal form of tracheobronchial amyloid deposition manifest as airway polyps, and diffuse form is characterized by diffuse airway calcification or ossification [8]. Progressive airway narrowing, fatal airway obstruction and obstructive pneumonia are the complications [8]. Patients with tracheobronchial amyloidosis show nonspecific clinical signs such as cough, stridor, wheezing and hemoptysis, therefore diagnosis is often delayed. Symptoms secondary to diffuse tracheobronchial amyloidosis usually begin in middle or late adult life [9]. In diffuse tracheobronchial amyloidosis, 5-year survival rates range from 30 to 50%, although developments in bronchoscopic, surgical and laser resection of airway amyloid deposits may result in improved prognosis [5,6,8]. There are only a few reports on imaging findings in tracheobronchial amyloidosis [1– 4,7,8,10,11]. On the chest radiograph, tracheobronchial wall thickening and irregular narrowing may be seen. With CT, the submucosal deposition of amyloid is of soft tissue density. The
Fig. 1. Posteroanterior X-ray of the chest shows diffuse narrowing of the trachea with irregular contours.
Fig. 2. Coronal reformat CT image in lung window setting shows irregular and circumferential narrowing of the trachea and thickened wall.
Fig. 3. Axial computed tomography examination mediastinal and lung window settings: a circumferential thickening of the tracheal wall (a) and normal lung parenchyma are seen (b).
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lumen of trachea and bronchi appears narrowed, because of the thickening of the wall [7,10,12]. Thickening of the tracheal and bronchial walls can be clearly depicted with CT. Also bronchial lumen narrowing as well as bronchiectasis, atelectasis or poststenotic pneumonia can be demonstrated with CT [7]. In our patient, the chest radiograph showed tracheal wall thickening with irregular luminal narrowing. With CT examination the submucosal deposition of amyloid was demonstrated in addition to radiographic findings. The final diagnosis was obtained by bronchoscopic biopsy. Radiologically, there are only few differential diagnosis that have to be considered. These are tracheobronchiopathia osteoplastica, chronic tracheobronchitis associated with colitis ulcerosa and relapsing polychondritis [7,10,12]. In tracheobronchiopathia osteoplastica, CT shows thickening of the tracheal wall with irregular calcifications protruding into the lumen from anterior and lateral wall which is considered as a pathognomonic finding [10,12]. Chronic tracheobronhitis associated with colitis ulcerosa shows circumferential thickening of tracheobronchial wall [10,12]. Thickening of tracheobronchial wall seen in relapsing polychondritis caused by recurrent episodes of inflamamation, seems to be another differential diagnosis [13]. As a result, in the diagnosis of tracheobronchial amyloidosis CT clearly shows the extend of the disease and its potential postobstructive pulmonary complications. CT of the central airways can be used as a guiding tool before bronchoscopic interventions, planning of operation and follow-up after laser coagulation treatment.
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