ABSTRACT
Objective To discuss the clinical features, diagnosis, treatment and prognosis of primary tracheobronchial amyloidosis. Methods We retrospectively studied 50 primary tracheobronchial amyloidosis cases reported in Chinese literature during 1989-2012 and one in our hospital. The clinical information, symptoms, imaging findings, endoscopic manifestations and treatment were analyzed. Results The patient in our hospital was admitted due to "short breath". Chest CT, bronchoscopy and pulmonary funetion examination were done; bronchoscopy biopsy specimen confirmed the diagnosis of PTA; and the patients responded to cyclophosphamide treatment. The main clinical symptoms of the 50 patients included cough (96%, 48/ 50), short breath (70%, 35/50), sputum (66%, 33/50), fever (24%, 12/50), and hemoptysis (22%, 11/50). Thiekening of lung markings (45. 5 %, 15/33) was themost common ehest X-ray finding; airway wall thiekening and stenosis (76. 2%, 32/42) was the most common CT manifestation; and the most common endoscopic finding was also airway wall thiekening and stenosis (40. 8%, 20/49), followed by mucosal congestion and edema (32. 7%, 16/49). Obstructive ventilatory dysfunetion was found to be the major problem during pulmonary funetion tests. The diagnoses in 47(94%) patients were confirmedby bronchoscopic biopsy. Twenty-one literatures reported complications in 3 cases and misdiagnosis in 24 cases. Forty-two of the 50 patients reeeived drug treatment, bronchoscopic interventional therapy, external beam radiation therapy and surgieal treatment, and they responded well. Conclusion Primary tracheobronchial amyloidosis is rare and is prone to be misdiagnosed. Correct diagnose needs pathological examination and currently there is no specific treatment for it.
ABSTRACT
Objective To discuss the clinical features, diagnosis, treatment and prognosis of primary tracheobronchial amyloidosis. Methods We retrospectively studied 50 primary tracheobronchial amyloidosis cases reported in Chinese literature during 1989-2012 and one in our hospital. The clinical information, symptoms, imaging findings, endoscopic manifestations and treatment were analyzed. Results The patient in our hospital was admitted due to "short breath". Chest CT, bronchoscopy and pulmonary funetion examination were done; bronchoscopy biopsy specimen confirmed the diagnosis of PTA; and the patients responded to cyclophosphamide treatment. The main clinical symptoms of the 50 patients included cough (96%, 48/ 50), short breath (70%, 35/50), sputum (66%, 33/50), fever (24%, 12/50), and hemoptysis (22%, 11/50). Thiekening of lung markings (45. 5 %, 15/33) was themost common ehest X-ray finding; airway wall thiekening and stenosis (76. 2%, 32/42) was the most common CT manifestation; and the most common endoscopic finding was also airway wall thiekening and stenosis (40. 8%, 20/49), followed by mucosal congestion and edema (32. 7%, 16/49). Obstructive ventilatory dysfunetion was found to be the major problem during pulmonary funetion tests. The diagnoses in 47(94%) patients were confirmedby bronchoscopic biopsy. Twenty-one literatures reported complications in 3 cases and misdiagnosis in 24 cases. Forty-two of the 50 patients reeeived drug treatment, bronchoscopic interventional therapy, external beam radiation therapy and surgieal treatment, and they responded well. Conclusion Primary tracheobronchial amyloidosis is rare and is prone to be misdiagnosed. Correct diagnose needs pathological examination and currently there is no specific treatment for it.
ABSTRACT
Primary tracheobronchial amyloidosis (TBA) is a rare pulmonary disease. A systematic review was performed on 64 cases of primary TBA in China and progress in the diagnosis and treatment of this disease is discussed. The Chinese biological and medical databases from 1970 to 2010were searched and 75 cases of complete clinical and pathological data were identified. The clinical characteristics of the disease were summarized and longitudinal comparisons were made of diagnostic and treatment methods over time. The results showed that the morbidity associated with primary TBA has increased over recent years. The clinical manifestations were non-specific. Progressive dyspnea, cough and sputum were the most common symptoms. The percentage of patients undergoing computed tomography (CT) scan has increased over the years. The bronchoscopy and transbrochial lung biopsy (TBLB) were usually sufficient to establish the diagnosis. Treatment was reported for a total of 44 cases. Bronchoscopic Nd:YAG laser irradiation, argon plasma coagulation (APC)and drugs administration such as steroids and colchicines were reported to be effective in some patients. It is concluded that the demographic characteristics and clinical manifestations of primary TBA patients in China are largely consistent with findings reported in other countries. Dramatically more cases were reported in recent years, mainly due to the extensive application of bronchoscopy since 1990s. Chest CT scan provides important clues for the diagnosis of the disease. The definite diagnosis was confirmed by bronchoscopic findings and Congo red staining of biopsy specimen.Bronchoscopic Nd:YAG laser irradiation, argon plasma coagulation (APC) and drugs administration,such as steroids and colchicines were reported to be effective in some patients.
ABSTRACT
We report on a case of a patient with laryngo-tracheobronchial amyloidosis who complained of cough, sputum, and hoarseness. A chest X-ray showed consolidation in the right middle lobe. A chest CT scan showed diffuse, irregular narrowing of the tracheobronchial tree and atelectasis of the right middle lobe, with calcification of bronchial wall. Bronchoscopic findings were multinodular submucosal thickening of the right vocal cord, and yellowish multinodular submucosal thickening from the lower trachea through both main bronchi, as well as the lingular division of the left upper lobe, the right middle lobe, and the right lower lobe. The right middle lobe bronchus was nearly obstructed. The diagnosis of amyloidosis was made by multiple bronchoscopic biopsies on the right vocal cord and both bronchi. Pathologic findings were characteristic apple-green birefringence under polarized microscopy with Congo-red stain. The patient had no evidence of systemic amyloidosis. The patient is under conservative symptomatic treatment.
Subject(s)
Humans , Amyloidosis , Biopsy , Birefringence , Bronchi , Cough , Hoarseness , Microscopy , Pulmonary Atelectasis , Sputum , Thorax , Trachea , Vocal CordsABSTRACT
Amyloidosis limited to the tracheobronchial respiratory tract is a relatively rare condition. Fewer than 100 cases of tracheobronchial amyloidosis are reported. It is characterized by deposits of amyloid in airway walls. Bronchoscopy, although carrying a risk of bleeding, is the procedure of choice diagnostically and therapeutically. 49-year old man suffered from cough and exertional dyspnea over 20s years and recently hemoptysis, atelectasis of right middle lobe and postobstructive pneumonia. He was diagnosed as amyloidosis and treated by bronchoscopy with Nd : YAG laser. We discussed this case with review of literatures.
Subject(s)
Humans , Middle Aged , Amyloid , Amyloidosis , Bronchoscopy , Cough , Dyspnea , Hemoptysis , Hemorrhage , Laser Therapy , Lasers, Solid-State , Pneumonia , Pulmonary Atelectasis , Respiratory SystemABSTRACT
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 fib rilar 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.
Subject(s)
Adult , Humans , Male , Amyloid , Amyloidosis , Biopsy , Birefringence , Bronchi , Bronchoscopy , Constriction, Pathologic , Cough , Dyspnea , Eosinophils , Microscopy , Sputum , Tomography, X-Ray Computed , TracheaABSTRACT
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.