Ann Thorac Surg 2004;77:1832-1834
© 2004 The Society of Thoracic Surgeons
Case report
A case of primary diffuse tracheobronchial amyloidosis
Hayssam Gibbaoui, MDa*,
S. Abouchacra, MD, FRCS(C)a,
M. Yaman, MD, FRCS(C)a
a Tawam Hospital, Abu Dhabi, United Arab Emirates
Accepted for publication June 4, 2003.
* Address reprint requests to Dr Gibbaoui, Tawam Hospital, PO Box 15258, Al ain, Abu Dhabi, United Arab Emirates.
e-mail: h.gibbaoui{at}voila.fr
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Abstract
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Tracheobronchial amyloidosis (TBA) is an uncommon localized form of amyloidosis with fewer than 150 reported cases in the literature. We report a case of primary diffuse tracheobronchial amyloidosis who presented with cough, wheezing, recurrent and progressive dyspnea as well as hemoptysis. Though there is no universally accepted treatment for the localized form, this patient was successfully treated with three sessions of bronchoscopic resection and adjuvant steroids. This report will review the various types of amyloidosis of the respiratory tract and their clinical features, in addition to discussing the different available treatment modalities for TBA.
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Introduction
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Tracheobronchial amyloidosis is a rare potentially fatal condition characterized by deposition of amyloid within the airway walls. These manifest as submucosal plaques or polypoid tumors. Though the symptoms are caused by bronchial obstruction, routine chest radiograph is usually normal. Consequently, the diagnosis is delayed and made through bronchoscopy and biopsy of infiltrated mucosa. With fewer than 150 reported cases in the literature, this condition has no universally accepted treatment and late cases with major airway obstruction are difficult to manage. We present a patient with the diffuse variety of primary TBA that was successfully treated with repeated bronchoscopic resection and adjuvant steroids.
A 55-year-old woman presented with chronic cough, wheezing, intermittent hemoptysis, and recurrent progressive dyspnea. She was diagnosed with asthma 10 years prior and was managed with inhaled glucocorticoids and ß-adrenergic agonists. Despite treatment, her symptoms progressively worsened, with physical examination revealing only mild stridor and minimal wheezing. Chest radiograph revealed areas of atelectasis in both lung bases although chest computed tomographic (CT) scan demonstrated extensive thickening of the walls of the trachea and main bronchi with luminal narrowing (Fig 1).
Flexible bronchoscopy revealed diffuse nodular thickening of the airway walls, mucosal friability, and narrowing of the right main bronchus as well as near total occlusion of the left main bronchus. Biopsy specimen revealed eosinophilic deposits in the submucosa, with apple-green birefringence on Congo red stain; a finding characteristic of amyloid. There was no evidence of extrapulmonary organ involvement with amyloidosis. Moreover, blood and urine chemistry including serum immunoelectrophoresis for light chains were negative, hence consistent with the diagnosis of primary diffuse TBA. Due to airway obstruction, the patient underwent rigid bronchoscopy under general anesthesia and resection of most of the nodularities in the trachea as well as the left and right main bronchi. Airway control was difficult during the initial procedure as the amyloid flaps quickly migrated to the lumen once the mucosa was opened. The patient was subsequently started on 60 mg of prednisolone per day. She had a smooth postoperative course except for mild residual stridor. However, 5 days later, she suddenly deteriorated with increasing shortness of breath, requiring transfer to the intensive care unit and intubation. Her airway pressure was high, necessitating an urgent rigid bronschoscopy, which indicated that the external flaps of amyloidosis migrated into the carina. These were resected once again and both her left upper and lower lobes in addition to the right lower lobe were opened. However, the right upper lobe was still closed with amyloid flap. She was stabilized and extubated 5 days later with improvement in her exercise tolerance and no residual stridor. The patient was discharged home and readmitted 2 weeks later electively for repeat bronchoscopy to relieve the remaining obstruction of the right upper lobe. She underwent flexible bronchoscopy on this occasion, with endobronchial resection of right upper lobe occlusion. This resulted in successful reexpansion of the right upper lobe and the patient was discharged home in good condition. The dose of prednisolone was progressively decreased over 2 months. At 1-year follow-up the patient was doing well off steroids and had no respiratory complaints.
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Comment
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Amyloidosis is a spectrum of diseases associated with abnormal extracellular deposition of amyloid, and autologus fibrillar protein material. Histochemically this protein binds with Congo red, revealing green birefringence under polarized light [1]. Although amyloidosis is usually a multiorgan disease, localized amyloid deposits may affect single organs, such as the kidney, bladder, or respiratory tract. Respiratory amyloidosis, first described in 1877 by Lesser [2], has three characteristic forms: focal or diffuse TBA, nodular parenchymal, and diffuse parenchymal amyloidosis [1, 3]. Although the tracheobronchial variety is the most common, the nodular form is a rare condition that is usually asymptomatic and is often discovered as an incidental finding on chest radiography. The diffuse parenchymal form is the least common, and is generally fatal within 2 years as a result of respiratory failure [1, 4].
Tracheobronchial amyloidosis has an intermediate prognosis and occurs almost exclusively in the absence of systemic amyloidosis. It is usually diffuse in nature and manifests as submucosal plaques, but in 14% of patients the deposition is solitary and can mimic an endobronchial neoplasm. TBA affects males more often than females, and typically manifests in the fifth or sixth decade of life. However, the condition may be seen in patients from 16 to 85 years of age [5]. Common presenting symptoms include dyspnea, cough, and wheezing; features that can be confused with asthma, as well as hemoptysis and recurrent pneumonia [4]. The constellation of symptoms in TBA may vary according to the anatomic distribution of amyloid deposition with three identified patterns of involvement: proximal, mid, and distal airways types. Patients with "proximal" or upper tracheal disease develop varying degrees of upper airway obstruction. Those with "mid" or distal tracheal and main bronchial disease suffer from lobar collapse or recurrent airway and parenchymal infections. Progression to severe obstruction of the main bronchi may occur with consequent respiratory failure; fortunately, however, this complication is quite rare. Patients with the "distal" airway variety have sparing of the trachea, but significant involvement of the main bronchi and peripheral airways and hence present with repeated pneumonia, cough, and bronchiectasis [3].
Bronchoscopy is the cornerstone in the diagnosis of TBA and characteristically manifests widely dispersed lesions that are irregular in size and shape with diverse colors. They may vary from small, flat elevations to broad plaques and longitudinal folds. Chest radiograph may be normal (50%), or may reveal areas of atelectasis due to obstructing lesions [3]. These appear on CT as circumferential wall thickening with calcifications as well as luminal narrowing at various levels of the tracheobronchial tree, as was seen in our patient. Although the CT findings of TBA are specific of this condition, other diseases should be considered, such as tracheobronchial tuberculosis, tracheobronchopathia osteochondroplastica, and relapsing polychondritis [1, 3, 5]. Tissue biopsy is necessary for definitive diagnosis with the classic appearance of green birefringence under polarized microscopy.
Treatment options for TBA include conservative measures, aggressive local and systemic therapy, and radiation (Table 1).
. The local measures that have been utilized have varied include laser resection, bronchoscopic dilatation and stenting, and cryosurgery. Flemming and colleagues [6] demonstrated improvement in symptoms and pulmonary function following intermittent bronchoscopic resection of tracheobronchial amyloid lesions, which was performed in our patient. Cazalets and colleagues [7] reported 2 patients with TBA that were treated successfully with bronchoscopic dilatation, and Yang and colleagues [8] described 1 patient managed with bronchoscopic resection and stenting with good outcome. However, attempts at resection of airway amyloid deposition are commonly limited by significant bleeding during the procedure given the friable nature of the amyloid lesion. Bleeding may also occur during bronchoscopy, likely related to the fragility of the amyloid infiltrated blood vessels [5]. Alternatively, successful laser resection using CO2 and Nd:YAG has been described by O'Regan and coworkers [3] with disease control lasting up to 7 years, and repeated sessions of cryosurgery have resulted in good response in a case reported by Maiwand and associates [4]. Moreover, various medical treatment modalities have been used with agents that include corticosteroids, melphalan, and colchicine, but have had limited success. However, in patients who progressively worsen, and are not amenable to the temporizing benefits of debridment or laser resection, radiation treatment should be considered. The resultant improvement can last for several months based on the radiosensitivity of the amyloid secreting plasma cells within the bronchial walls. Kurrus and colleagues [9], and Kalra and coworkers [10] have reported excellent response to radiotherapy in 2 patients with TBA.
At present, broncoscopic resection remains the standard therapeutic approach to TBA. An experienced anesthetist and bronchoscopist are needed to ventilate the patient and maintain adequate oxygenation. In bilateral disease the trachea and one main bronchus should be opened first to secure single lung ventilation, followed by endobronchial opening of the controlateral lung. The right upper lobe is usually the most difficult to open due to the angulation with the right main bronchus. Five-year survival rates range from 30% to 50%. Patients with proximal airways disease have worse prognosis than those with mid or distal airways involvement [3]. Although spontaneous resolution has been reported, patients with TBA will most likely undergo repeated attempts of tumor resection to control progressive respiratory symptoms [5]. Despite significant airway disease, our patient has fortunately responded well to the bronchoscopic resections and has remained symptom free.
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References
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- Kim H.Y., Im J.G., Song K.S., et al. Localized amyloidosis of the respiratory system: CT features. J Comput Assist Tomogr 1999;23:627-631.[Medline]
- Lesser A. Ein Fall von Enchondroma osteiodes mixtum der Lunge mit partieller amyloid Entortung. Virchows Arch (Path Anat) 1877;69:404–8
- O'Regan A., Fenlon H.M., Beamis J.F., Jr, et al. Tracheobronchial amyloidosis. The Boston University experience from 1984 to 1999. Medicine (Baltimore) 2000;79:69-79.[Medline]
- Maiwand M Omar, Nath Abdul R, Kamath BSK. Cryosurgery in the treatment of tracheobronchial amyloidosis. J Bronchol 2001;8:95–7
- Capizzi S.A., Betancourt E., Prakash U.B. Tracheobronchial amyloidosis. Mayo Clin Proc 2000;75:1148-1152.[Medline]
- Flemming AF, Fairfax AJ, Arnold AG, Lane DJ. Treatment of endobronchial amyloidosis by intermittent bronchoscopic resection. Br J Dis Chest 1980;74:183–8
- Cazalets C, Belleguic C, Sost G, et al. Tracheobronchial amyloidosis: a propos of 2 cases. Rev Med Interne 2002;23:317–21
- Yang S, Chia SY, Chuah KL, Eng P. Tracheobronchial amyloidosis treated with rigid bronchoscopy and stenting. Surg Endosc 2003;17:658–9
- Kurrus J.A., Hayes J.K., Hoidal J.R., et al. Radiation therapy for tracheobronchial amyloidosis. Chest 1998;114:1489-1492.[Abstract/Free Full Text]
- Kalra S., Utz J.P., Edell E.S., et al. External-beam radiation therapy in the treatment of diffuse tracheobronchial amyloidosis. Mayo Clin Proc 2001;76:853-856.[Medline]
- Herman DP, Colchen A, Milleron B, Bentata-Pessayre M, Personne C, Akoun G. The treatment of tracheobronchial amyloidosis using a bronchial laser. A propos of a series of 13 cases. Rev Mal Respir 1985;2:19–23
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Abstract
Introduction
