Diffuse Tracheobronchial Amyloidosis Research Articles

Tracheobronchial Amyloidosis, as an Uncommon Form of Amyloidosis: Evaluation with MDCT

Tracheobronchial amyloidosis is a localised form of amyloidosis characterised by the accumulation of amyloid deposit in the trachea, main bronchi and segmentary bronchi. Although diffuse tracheobronchial amyloidosis is the most frequently seen form of primary pulmonary amyloidosis, this is a rare disease. It is demonstrated with symptoms such as progressive dyspnea, cough and haemoptysis. It may cause airway obstruction, atelectasis and recurrent bronchopulmonary infections. Although the proximal section of the tracheobronchial tree can be visualised with chest radiography and fluoroscopy, the basis for examination is computed tomography (CT). The tracheobronchial tree can be effectively evaluated with high resolution images provided by multidetector CT (MDCT) and the post-processing images of them (multiplanar reformations [MPR], minimum intensity projections [MinIP], 3D volume-rendered images (3D VR), virtual bronchoscopic images) (4). The clinical and radiological findings are here presented of a rarely encountered case of tracheal amyloidosis initially diagnosed with MDCT. Keywords: Tracheobronchial amyloidosis, MDCT, progressive dyspnea, airway obstruction, progressive dyspnea, haemoptysis.

Outcomes after Bronchoscopic Procedures for Primary Tracheobronchial Amyloidosis: Retrospective Study of 6 Cases

Respiratory amyloidosis is a rare disease which refers to localized aberrant extracellular protein deposits within the airways. Tracheobronchial amyloidosis (TBA) refers to the deposition of localized amyloid deposits within the upper airways. Treatments have historically focused on bronchoscopic techniques including debridement, laser ablation, balloon dilation, and stent placement. We present the outcomes after rigid bronchoscopy to remove the amyloid protein causing the airway obstruction in 6 cases of tracheobronchial amyloidosis. This is the first report of primary diffuse tracheobronchial amyloidosis in our department; clinical features, in addition to therapy in the treatment of TBA, are reviewed. This paper shows that, in patients with TBA causing airway obstruction, excellent results can be obtained with rigid bronchoscopy and stenting of the obstructing lesion.

Primary Diffuse Tracheobronchial Amyloidosis in a Dog

Primary diffuse tracheobronchial amyloidosis was diagnosed at necropsy in an intact male Akita dog aged 11 years, a non-productive chronic cough having been the only related clinical sign. Histologically, eosinophilic hyalinized deposits were found as a band in the lamina propria underneath the epithelium of the trachea and bronchi. When stained with Congo red, apple-green birefringence was observed in the deposits viewed with polarized light. The amyloid did not lose sensitivity to Congo red staining after incubation with potassium permanganate, indicating that it was of the AL (amyloid light chain) type. Ultrastructural features of the amyloid included a typical fibrillar meshwork with individual fibrils measuring 9.5 to 10.5 nm in diameter. This is the first report of primary diffuse tracheobronchial amyloidosis in the dog.

A case of primary diffuse tracheobronchial amyloidosis

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.

Primary diffuse tracheobrochial amyloidosis: case report

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.

A Case of Primary Localized Tracheobronchial Amyloidosis

Primary pulmonary amyloidosis is a rare condition that can be classified into the tracheobronchial, diffuse alveolar septal, and nodular parenchymal type. Tracheobronchial amyloidosis is characterized by deposition of fibrilar proteins in the tracheobronchial tree, and it can be subdivided into diffuse and focal varieties. In this report, a case of diffuse tracheobronchial amyloidosis confirmed by flexible fiberoptic bronchoscopic biopsy is presented. The patient was a 43-year old male with a chief complaint of cough and sputum for 20 days and dyspnea for one day. The chest CT scan showed diffusely thickened walls of both the main and lobar bronchi with calcification. The bronchoscopic findings showed nodular lesions of the trachea, a diffuse bronchial stenosis of both the main bronchi and a pinpoint narrowing of the left upper and right middle lobar bronchus. The biopsy showed submucosal deposits of homogenous eosinophilic amyloid materials and an apple-green birefringence under polarizing microscopy following the Congo-red stain.

Amiloidosis traqueobronquial. Una forma infrecuente de amiloidosis restringida al tracto respiratorio inferior

We describe an 83-year-old man with diffuse tracheobronchial amyloidosis presenting with pneumonia, against a background of chronic obstructive pulmonary disease. The condition continued with intermittent episodes of obstruction for 2 years after diagnosis and the patient died after upper digestive tract hemorrhage. Monoclonally was never demonstrated.

Treatment of Diffuse Tracheobronchial Amyloidosis by Repeated Electrocautry Under Fiberoptic Bronchoscopy

Diffuse tracheobronchial amyloidosis is an uncommon form of pulmonary involvement, and causes prolonged cough, dyspnea, wheezing and repeated development of pneumonia. We report a case of diffuse tracheobronchial amyloidosis in 58-year-old woman that was traeted by repeated electrocautry under flexible fiberoptic bronchoscopy. The patient had a long-standing history of dyspnea and was admitted due to resting dyspnea, which was aggravated to impending respiratory failure after diagnostic procedures. We applied repeated electrocautry to the endobronchial amyloid tumors and successfully reduced bronchial stenosis and the pateint didn't feel dyspnea. We suppose that, in certain cases of tracheobronchial amyloidosis patients, endobronchial electrocautry would be a helpful procedure.

Primary diffuse tracheobronchial amyloidosis treated by bronchoscopic Nd-YAG laser irradiation.

A case of primary diffuse tracheobronchial amyloidosis in a 45-year-old woman is reported. Because of tracheal stenosis immediately beneath the vocal cords, due to amyloid deposits, she was treated with Nd-YAG laser irradiation. Immediately after treatment, symptoms such as cough, wheezing, and shortness of breath on exertion improved. Bronchoscopic Nd-YAG laser irradiation proved useful for the removal of amyloid deposits from the trachea in this patient.

PRIMARY TRACHEOBRONCHIAL AMYLOIDOSIS

A case of diffuse tracheobronchial amyloidosis in a 70-year-old woman thought clinically at first to have lung carcinoma is reported. Bronchoscopic biopsy was necessary to make a diagnosis. Amyloid was deposited markedly in the subepithelial layer. After Congo-red staining amyloid showed the usual apple-green birefringence under polarizing microscope. Electron microscopic study disclosed typical amyloid fibrillar structure. Amyloid protein of this patient was confirmed to be AL protein by immunoperoxidase study which showed specific staining by the anti-A lambda antiserum. When potassium permanganate reaction was used amyloid protein of this patient resisted to the reaction. We reviewed 19 cases from the previously reported cases of primary amyloidosis of the lower respiratory tract, and gave some description of pathologic and clinical aspects of this disease.

Spontaneous Regression of Diffuse Tracheobronchial Amyloidosis

Diffuse tracheobronchial amyloidosis which underwent an extensive spontaneous regression is described in a 39-year-old man. This regression was documented by serial roentgenograms of the chest, studies of pulmonary function, and bronchoscopic procedures with mucosal biopsies. The clinical implications of this spontaneous change are discussed.

Primary diffuse tracheobronchial amyloidosis.

The case is reported of a woman who died at the age of 36 years from obstructive respiratory failure due to diffuse tracheobronchial amyloidosis which had caused symptoms for six years. When first seen her symptoms of wheezing cough and mucopurulent sputum sometimes streaked with blood were of recent onset, but on bronchogram and bronchoscopy her disease was already widespread. Appearances at bronchography were interpreted as malformation and at bronchoscopy as tracheobronchitis, possibly tuberculous. Bronchoscopic biopsy was necessary to make the diagnosis of amyloidosis. The amyloid gave the usual staining reactions with an apple-green birefringence in polarized light following staining with Congo red. At necropsy an extensive histological survey proved that the amyloid was confined to the trachea and main stem bronchi. There was no associated disease, no family history, and no upset in plasma proteins.

Primary diffuse tracheo-bronchial amyloidosis.

A case of diffuse tracheo-bronchial amyloidosis in a 62-year-old woman thought to have chronic bronchitis is reported. The immunological mechanism in the pathogenesis of this condition is considered and the need for bronchological investigation in chronic bronchial disorders is stressed.