Primary Tracheobronchial Amyloidosis Research Articles

Clinical features of primary tracheobronchial pulmonary amyloidosis

Objective: To analyze the clinical features of primary tracheobronchial pulmonary amyloidosis (PTBA). Methods: The records of 11 patients with PTBA diagnosed by pathology from January 2002 to June 2018 in Xiangya Hospital of Central South University were retrospectively reviewed, including clinical manifestations, laboratory and imaging examination, bronchoscopic manifestations and therapies. Meanwhile, PTBA was staged based on the severity and extent of lesion under bronchoscopy. Results: The most common clinical symptoms were cough and expectoration followed by shortness of breath and hemoptysis. Chest computed tomography (CT) revealed pulmonary infection (4/11), pulmonary nodules and masses (5/11), interstitial lesions (2/11), tracheal bronchial wall thickening (5/11) or airway stenosis (4/11). Bronchoscopy showed mucosal hypertrophy (8/11), nodular bulge (3/11), and luminal stenosis (6/11). According to the lesion involvement, 1 case only involved the lungs, 10 cases involved the trachea and/or bronchus, with (8/10) or without (2/10) lung lesions. According to the bronchoscopic staging, 2 cases (2/10) in the stage Ⅰ, lesions were limited; 2 cases (2/10) in the stage Ⅱ, lesions were diffuse; 6 cases (6/10) in the stage Ⅲ, diffuse lesions with stenosis. Conclusions: PTBA is a rare disease of the respiratory system, with unspecific clinical manifestations and diverse pulmonary imaging findings; airway mucosal hypertrophy and nodular bulging can be seen under bronchoscopy. Patients with advanced stage may present with airway stenosis.

A RARE CASE OF PRIMARY TRACHEOBRONCHIAL AMYLOIDOSIS PRESENTING AS LARGE UNILATERAL PLEURAL EFFUSION

SESSION TITLE: Pulmonary Manifestations of Systemic Diesase SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: The incidence of recurrent pleural effusions is 1-2% in patients with systemic amyloidosis. Tracheobronchial amyloidosis (TBA) is a rare condition that primarily involves large airways. Patients usually present with dyspnea and wheezing. Pleural effusion is not a common manifestation of TBA. Herein, we present a rare case of primary TBA presenting as a recurrent unilateral pleural effusion. CASE PRESENTATON: An 81-year-old non-smoker female with a significant history of recent hip surgery, breast cancer in remission for 10 years, hypertension and coronary artery disease presented to the hospital with a one-day history of insidious onset of dyspnea and dry cough. She appeared to be in mild distress and had decreased air entry on the left chest along with expiratory wheezing on auscultation. The rest of her exam was unremarkable. Her chest x-ray showed a new large left-sided pleural effusion. She underwent thoracentesis; drained 1500 ml of yellow serous fluid. The pleural fluid analysis was predominantly lymphocytic, exudative, with negative cultures and cytology. A post-thoracentesis chest CT scan showed left lower lobe consolidation and a segmental collapse of the left upper lobe (LUL). On Bronchoscopy, an endobronchial lesion with near total obstruction of LUL bronchus was visualized. Subsequently, an endobronchial biopsy was performed. Pathology of the lesion was consistent with amyloid deposition confirmed by Congo Red staining. Further workup to rule out systemic amyloidosis was negative. The patient’s clinical, imaging, and pathology findings were consistent with primary light chain TBA with post-obstructive atelectasis and pleural effusion. The patient was not a candidate for Melphalan secondary to her poor functional status and she declined treatment with Rituximab. Accordingly, she underwent external beam radiation therapy and was started on methylprednisolone 40 mg intravenously for five days, followed by a two-week course of a tapered regimen of oral prednisone. Subsequent follow-up a month after discharge showed complete resolution of left-sided pleural effusion. DISCUSSION: Pleural effusion is common in systemic amyloidosis, especially in the setting of amyloid cardiomyopathy, and interstitial deposition of amyloid protein. The presence of pleural effusion in systemic amyloidosis is associated with an expected survival of 1.8 months. In the absence of systemic amyloidosis, it is unknown whether pleural effusion is a manifestation of isolated TBA. In TBA, almost half of chest X-rays are normal. A common finding includes lobar collapse, but not pleural effusion. To our knowledge, this is the first case of primary TBA associated pleural effusion which is responsive to both steroids and radiotherapy. CONCLUSIONS: In the absence systemic amyloidosis, amyloid cardiomyopathy, or pulmonary parenchymal amyloidosis, pleural effusion may also be a clinical manifestation of localized TBA. Reference #1: Tada, L., Anjum, H., Linville, W.K. and Surani, S. (2015). Recurrent pleural effusions occurring in association with primary pulmonary amyloidosis. Case reports in pulmonology, 2015. Reference #2: L Berk, John & O'Regan, Anthony & Skinner, Martha. (2002). Pulmonary and Tracheobronchial Amyloidosis. Seminars in respiratory and critical care medicine. 23. 155-65. 10.1055/s-2002-25304. DISCLOSURES: No relevant relationships by Thamer Sartawi, source=Web Response No relevant relationships by Mingchen Song, source=Web Response No relevant relationships by Rajagopal Sreedhar, source=Web Response

Bronchoscopic Diagnosis and Treatment of Primary Tracheobronchial Amyloidosis: A Retrospective Analysis from China.

Objective. To assess the value of bronchoscopy in the diagnosis and treatment of primary tracheobronchial amyloidosis (TBA), in order to reduce misdiagnosis rates and improve prognosis. Methods. Clinical data of 107 patients with TBA reported from 1981 to 2015 in China were retrospectively analyzed for clinical features, bronchoscopic manifestations, pathologies, treatments, and outcomes. Results. 105 of 107 TBA patients were pathologically confirmed by bronchoscopy. Main bronchoscopic manifestations of TBA were single or multiple nodules and masses within tracheobronchial lumens; local or diffuse luminal stenosis and obstruction; luminal wall thickening and rigidity; rough or uneven inner luminal walls; congestion and edema of mucosa, which was friable and prone to bleeding upon touch; and so forth. 53 patients were treated with bronchoscopic interventions, like Nd-YAG laser, high-frequency electrotome cautery, freezing, resection, clamping, argon plasma coagulation (APC), microwaving, stent implantation, drug spraying, and other treatments. 51 patients improved, 1 patient worsened, and 1 died. Conclusion. Bronchoscopic biopsy is the primary means of diagnosing TBA. A variety of bronchoscopic interventions have good short-term effects on TBA. Bronchoscopy has important value in the diagnosis, severity assessment, treatment, efficacy evaluation, and prognosis of TBA.

原発性気管・気管支アミロイドーシスの 1 例

原発性気管・気管支アミロイドーシスの 1 例

A Case of Primary Tracheobronchial Amyloidosis with Preceding Hoarseness

A Case of Primary Tracheobronchial Amyloidosis with Preceding Hoarseness

Use of polarized light microscopy is essential in the efficient diagnosis of respiratory amyloidosis and could decrease disease prevalence

Primary tracheobronchial amyloidosis (TBA) is a rare disease of unknown etiology, with a high misdiagnosis rate. The current gold standard diagnostic criteria require double-positive results for Congo red staining and polarized light microscopy examination. The aim of the present report was to examine the diagnostic value of polarized light microscopy in TBA diagnosis in China. Thirteen cases from the Shandong University Qilu Hospital were reviewed. Polarized light microscopic examination was conducted after searching for cases with positive Congo red staining. Among the 13 patients, eight displayed yellow-green birefringence body with polarized light microscopic examination. This result indicated a false-positive rate of 38.5% with Congo red staining used as the single criteria for TBA diagnosis. After reviewing the Chinese literature and selecting 104 reported cases of TBA in China, we found that diagnosis with the gold standard was confirmed in <30% patients, which may lead to misdiagnosis and delayed treatment. The universal use of polarized light microscopy in China is currently limited, and there is a big gap from the international diagnosis standards. There is a need to include polarized light microscopy in routine TBA diagnosis to reduce the misdiagnosis rate, and achieve optimal treatment.

Diagnosis of primary tracheobronchial amyloidosis by multiplanar reconstruction of the computed tomography combined with bronchoscope

To summarize the specific CT characteristics and the endoscopic findings of primary tracheobronchial amyloidosis (PTBA) for improvement of the diagnostic accuracy. The imaging features of 6 patients with PTBA were analyzed by multiplanar reconstructed CT and the fiberoptic bronchoscope, and the pathology were summarized retrospectively. All PTBA patients received bronchoscopic examination and the definite diagnosis were confirmed by positive staining with Congo red. PTBA presented diffusethickeningofmajor airway and lumen stenosis in various degrees with scattered hemorrhage of the mucous membrane under CT and bronchoscope, which was more obvious in low part of trachea, main bronchus and lobar bronchus. The mucosa of trachea and bilateral main bronchi were irregular and bumpy with jutting nodes in 5 patients, which was called "wavy path" pattern. Widely nodular or stripy calcifications of airway were found in 4 patients, which was considered as specific imaging features in PTBA and was involved bilateral main bronchi largely.There were obstructive atelectasis in 2 patients, and calcifications of hilus of lung with longitudinal diaphragm lymph nodes in 3 patients, but they were not specific. Ignoring the extensive circumferential thickening of large airway, "wavy path sign" and rail-like calcification was mainly responsible for misdiagnosis of PTBA as endobronchial tuberculosis or other diseases. Attentions to the specific imaging features on multiplanar CT and the endoscopic findings are the fundamentals to avoid the misdiagnosis of PTBA.

Narrow-Band Imaging Bronchoscopy in Tracheobronchial Amyloidosis

Primary tracheobronchial amyloidosis (TBA) is a rare disease characterized by extracellular focal or diffuse submucosal deposits of amyloid proteins. Various types of endobronchial lesions have been described in TBA when bronchoscopy is performed using white light. Narrow-band imaging bronchoscopy has been mainly employed for detecting preneoplastic and neoplastic endobronchial lesions as it provides more detailed images of the microvasculature reflective of an altered angiogenesis process. Here, we describe bronchoscopic findings with white light and narrow-band imaging in 2 patients presenting with central airway obstructive disease later confirmed as having primary TBA.

Clinical analysis of primary tracheobronchial amyloidosis

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 function 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).Thickening of lung markings(45.5%,15/33)was the most common chest X-ray finding;airway wall thickening and stenosis(76.2%,32/42) was the most common CT manifestation;and the most common endoscopic finding was also airway wall thickening and stenosis(40.8%,20/49),followed by mucosal congestion and edema(32.7%,16/49).Obstructive ventilatory dysfunction was found to be the major problem during pulmonary function tests.The diagnoses in 47(94%) patients were confirmed by bronchoscopic biopsy.Twenty-one literatures reported complications in 3 cases and misdiagnosis in 24 cases.Forty-two of the 50 patients received drug treatment,bronchoscopic interventional therapy,external beam radiation therapy and surgical 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.

Tracheobronchial Amyloidosis Treated by Endobronchial Laser Resection and Self Expanding Y Stent

Amyloid deposition in tracheobronchial tree is a rare presentation and counts about 1% of benign tumors in this area. Herein, we describe a case of primary tracheobronchial amyloidosis which obstructed almost completely the distal trachea and the main left bronchus.The mass was successfully resected by mechanical resection and Nd-YAG laser coagulation during rigid bronchoscopy. Afterwards, a self-expanding Y-stent of new generation was inserted to restore airway patency. Five months later, radiological and bronchoscopic findings showed the stent in site, with normal patency of the tracheo-bronchial tree.

Amiloidosis traqueobronquial tratada con resección con láser endobronquial y prótesis en Y autoexpansible

Amyloid deposition in tracheobronchial tree is a rare presentation and counts about 1% of benign tumors in this area. Herein, we describe a case of primary tracheobronchial amyloidosis which obstructed almost completely the distal trachea and the main left bronchus.The mass was successfully resected by mechanical resection and Nd-YAG laser coagulation during rigid bronchoscopy. Afterwards, a self-expanding Y-stent of new generation was inserted to restore airway patency. Five months later, radiological and bronchoscopic findings showed the stent in site, with normal patency of the tracheo-bronchial tree.

Primary tracheobronchial amyloidosis

Primary tracheobronchial amyloidosis (PTBA) is a localized form of amyloidosis. The clinical signs of this condition are non-specific and diagnosis before bronchoscopic examination with multiple biopsies is uncommon. We present three cases of PTBA and compare findings between the thoracic imaging and fiberoptic bronchoscopy. Radiographic findings often had uncharacteristic features. The relatively specific coronal section CT preceded findings in transverse sections, and showed an irregular mucosal surface with diffuse prominent nodes and/or calcification of the tracheobronchus, which we called the "wavy path sign". In conclusion, coronal CT scan can provide ample clues for the early diagnosis of PTBA.

Images In Medicine: Primary Tracheobronchial Amyloidosis

We present a case of a non-smoker 23-year-old man who presented complaining of progressive shortness of breath and productive cough for 2 months. A diagnosis of tracheobronchial amyloidosis was made. CASE REPORT A non-smoker 23-year-old man has presented complaining of progressive shortness of breath and productive cough for 2 months. Physical examination revealed mild parasternal sibling, and past medical records were unremarkable. Initial chest computed tomography (CT) “hands up” scout demonstrated a tortuous trachea, without paratracheal stripes blurring (Figure 1). Figure 1 Figure 1. Chest computed tomography (CT) scouth demonstrating tracheal tortuous enlargement, without – however – blurring paratracheal stripes. A chest Computed Tomography (CT) scans corroborated such finding, and indicated the presence of diffuse circumferential tracheal thickening, with multiple foci of calcifications (Figure 2), raising the diagnostic suspicion of amyloidosis. Figure 2 Figure 2. Chest computed tomography (CT) indicating diffuse circumferential tracheal thickening, associated with multiple calcified foci, which is very useful for differential diagnosis. 3D-volume-rendering (3DVR) CT tool (Figure 3) and the Images In Medicine: Primary Tracheobronchial Amyloidosis 2 of 3 performance of virtual bronchoscopy (Figure 4) were used to confirm and better assess anatomical abnormalities. Figure 3 Figure 3. 3D-volume-rendering (3DVR) CT tool, corroborating imaging features and providing better anatomical detailing. Fiberoptical bronchoscopy samples were positively stained with Congo red at histochemical analysis, corroborating the diagnosis. Further screening investigation for other organ involvement was negative, conferring the primary character for tracheobronchial amyloidosis. Figure 4 Figure 4. Virtual bronchoscopy CT tool performance providing intraluminal appearance of tracheobronchial amyloidosis, with multiple irregular affected areas. DISCUSSION Tracheobronchial amyloidosis is a rare disease, in which CT currently plays a diagnostic role, once clinical findings are frequently non-specific. The characteristic circumferential involvement, without sparring tracheal muscular components (posterior wall) is very useful for differential diagnosis establishment with osteocondroplastic tracheobronchopathy, polychondritis recidivans and Mounier-Kuhn disease, which typically affects only cartilaginous structures. While video- guided stenotic resection is usually the therapy of choice, interventional decision should always be held on an individual basis.

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 tracheobronchial amyloidosis in China: Analysis of 64 cases and a review of literature

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 2010 were 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.

A Case of Primary Paranasal and Tracheobronchial Amyloidosis in a Young Adult

A Case of Primary Paranasal and Tracheobronchial Amyloidosis in a Young Adult

Primary tracheobronchial amyloidosis – Two case reports

Primary tracheobronchial amyloidosis is a form of respiratory amyloidosis, characterised by insoluble amyloid fibril proteins deposits along the airways wall. It is an uncommon disease, requiring a tissue sample to establish the definite diagnosis based on specific pathological features.The authors report two cases of tracheobronchial amyloidosis, describing their symptoms and the diagnostic and therapeutic procedures that were performed.

Amiloidose traqueobrônquica primária – A propósito de dois casos clínicos

Primary tracheobronchial amyloidosis is a form of respiratory amyloidosis, characterised by insoluble amyloid fibril proteins deposits along the airways wall. It is an uncommon disease, requiring a tissue sample to establish the definite diagnosis based on specific pathological features.The authors report two cases of tracheobronchial amyloidosis, describing their symptoms and the diagnostic and therapeutic procedures that were performed.Rev Port Pneumol 2008; XIV (4): 541-544

Tracheobronchopathia osteochondroplastica

A 70-year-old woman developed increasing dyspnoea and hoarseness without stridor. Bronchoscopy revealed the characteristic picture of a "rock-garden" with multiple whitish irregularly shaped nodules in the distal two third of the trachea, except the pars membranacea, involving the right-sided bronchial system to the origin of the lower-lobe bronchus, and the left main bronchus. The diagnosis of tracheobronchopathia osteochondroplastica was confirmed histologically: primary tracheobronchial amyloidosis was excluded. Tomography of the tracheobronchial tree demonstrated the findings, but a plain chest X-ray did not. The symptoms in this patient were not, however, caused by tracheobronchopathia osteochondroplastica but by concomitant pulmonary emphysema and acute laryngitis and were improved after symptomatic treatment of the laryngitis.

PRIMARY TRACHEOBRONCHIAL AMYLOIDOSIS: A SERIES OF 3 CASES

Primary tracheobronchial amyloidosis is a form of localized pulmonary amyloidosis, characterised by the deposition of AL-amyloid in trachea and bronchi. It is a rare and slowly progressive disease, usually requiring repeated endoscopic treatment. In this case series we describe symptoms, diagnostic and therapeutic procedures, radiological findings and pulmonary function testing in 3 cases of different presentation and severity. Two patients were treated by endoscopic debulking and stent placement during rigid bronchoscopy, both with excellent clinical and functional results. In one of these patients regular endoscopic and clinical control exams were performed in the 5 years following the initial treatment, showing stable disease, requiring no further therapeutic intervention until today.