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Deposition of amyloid proteins in extracellular space can occur due to uncontrolled inflammatory processes. Tracheobronchial amyloidosis (TBA) is a rare form of such disease. A 68‐year‐old woman was evaluated for chronic cough following a COVID‐19 infection. Although post‐viral sequelae were suspected, further investigation revealed TBA. This condition involves amyloid deposits within the airways. The temporal association with COVID‐19 raises a potential etiological link, suggesting viral‐induced inflammatory or immune‐mediated processes may play a role. This case underlines the need to consider rare etiologies like TBA in patients with unresolved respiratory symptoms post‐COVID‐19. [ABSTRACT FROM AUTHOR]

Abstract Background Pulmonary amyloidosis often occurs as a part of systemic AL (amyloid light chain) amyloidosis. Localized tracheobronchial amyloidosis is a very rare entity. Most often, it is asymptomatic, or if symptomatic, it usually presents with vague symptoms such as cough, dyspnoea, hoarseness, wheezing, haemoptysis, and rarely respiratory failure. It is commonly misdiagnosed as asthma, tuberculosis, COPD, malignancy, or pneumonia. Case presentation We are presenting a case of an elderly male, aged 64 years, who presented with symptoms of productive cough, dyspnoea, wheezing, and significant weight loss over a period of 6 months. Investigations such as bronchoscopy, histopathology, and imaging studies showed an endobronchial mass and left-sided pleural effusion. Conclusions Endobronchial amyloidosis generally has a nodular appearance which can imitate an endobronchial tumour. The quality of life is decreased in these patients, and the life expectancy is approximately 8–10 years. Therefore, the early diagnosis of this disease is essential.

Background: Pulmonary amyloidosis often occurs as a part of systemic AL (amyloid light chain) amyloidosis. Localized tracheobronchial amyloidosis is a very rare entity. Most often, it is asymptomatic, or if symptomatic, it usually presents with vague symptoms such as cough, dyspnoea, hoarseness, wheezing, haemoptysis, and rarely respiratory failure. It is commonly misdiagnosed as asthma, tuberculosis, COPD, malignancy, or pneumonia. Case presentation: We are presenting a case of an elderly male, aged 64 years, who presented with symptoms of productive cough, dyspnoea, wheezing, and significant weight loss over a period of 6 months. Investigations such as bronchoscopy, histopathology, and imaging studies showed an endobronchial mass and left-sided pleural effusion. Conclusions: Endobronchial amyloidosis generally has a nodular appearance which can imitate an endobronchial tumour. The quality of life is decreased in these patients, and the life expectancy is approximately 8–10 years. Therefore, the early diagnosis of this disease is essential. [ABSTRACT FROM AUTHOR]

Zekewos Demissie,1 Nahom Zemedkun,1 Abraham Demile,1 Berhanu Nega,2 Yonas Bekuretsion,3 Bisrat Getachew,4 Bethelhem Berhanu5 1Department of Internal Medicine, Lancet General Hospital, Addis Ababa, Ethiopia; 2Department of Surgery, Division of Cardiothoracic Surgery, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia; 3Department of Pathology, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia; 4Department of Otolaryngology, Head and Neck Surgery, St. Paul Millennium Medical College, Addis Ababa, Ethiopia; 5Department of Radiology, St. Paul Millennium Medical College, Addis Ababa, EthiopiaCorrespondence: Zekewos Demissie, Email zakdem61@gmail.comAbstract: This is a case of localized primary tracheobronchial amyloidosis in a patient who presented with upper airway obstruction. Tracheobronchial amyloidosis is a type of localized bronchopulmonary amyloidosis that is frequently overlooked. It involves the deposition of amyloid protein on the tracheal and bronchial tissue, leading to progressive tracheal stenosis and airway obstruction that can be seen on imaging as a tracheal mass. Because of its significant diagnostic difficulty and therapeutic conundrum, it ought to be considered in the differential diagnosis of upper airway symptoms with an unknown etiology.Keywords: tracheobronchial amyloidosis, tracheal mass, upper airway obstruction

Localized tracheobronchial amyloidosis (TBA) is a rare form of pulmonary amyloidosis that is characterized by amyloidosis accumulation in the trachea and main bronchus submucosa. TBA is usually localized in the lung and is not associated with systemic amyloidosis. Although patients may be asymptomatic at first, they may develop dyspnea, recurrent cough, and hemoptysis attacks as the lesions narrow the tracheobronchial tree. Histochemical examination of biopsies taken with flexible bronchoscopy after thorax CT findings is usually used to make the diagnosis. There has never been a case reported in which TBA and acute myeloid leukemia (AML) coexisted. We report the first case of TBA in a patient diagnosed with AML. [ABSTRACT FROM AUTHOR]

Tracheobronchial amyloidosis, manifested by amyloid deposits limited specifically to tracheal and bronchial tissue, is a rare manifestation with only a few hundred published cases. Patients classically present with symptoms related to fixed upper airway obstruction caused by tracheal stenosis. Clinical symptoms are non-specific and include hoarseness, dyspnea, cough, stridor, hemoptysis, and dysphagia, which are similar to those caused by more common airway disorders, often leading to incorrect, missed, and delayed diagnosis. The wide-spread use of computerized tomography (CT) imaging has the potential of dramatically advancing the early diagnosis of tracheobronchial amyloidosis. We present a case of a patient with chronic and progressive hoarseness, diagnosed with tracheobronchial amyloidosis, with a focus on unusually clear and precise CT soft tissue neck imaging. CT imaging demonstrated nodular circumferential raised mass-like thickening involving the long-segment posterior wall of the distal trachea. The wall thickening also extended into the proximal left main stem bronchi, but spared the distal bronchial tree. This resulted in moderate (approximately 50%) narrowing of the tracheal lumen, which explained the patient's hoarseness. Routine CT imaging of patients with chronic and progressive respiratory symptoms, including cough, hoarseness, and dyspnea, is recommended. Tracheobronchial amyloidosis is an uncommon disease, but it may become more commonly recognized with broader use of more effective CT imaging protocols.

Primary tracheobronchial light chain (AL) amyloidosis is a rare and heterogeneous disease characterized by the buildup of amyloid deposits in the airway mucosa. Although its treatment remains challenging, the current view is that the localized form can be treated conservatively due to its slow progression. While radiotherapy has proven effective in treating localized form of the disease, some patients do not respond to local treatment and continue to experience poor quality of life, highlighting the need to explore additional treatment strategies. In this report, we discuss a case of primary tracheobronchial AL amyloidosis with biclonal gammopathy (IgA κ and IgG κ) in a 46-year-old man who was transferred to our hospital due to dyspnea progression over the preceding 3 years. Chest computed tomography revealed irregular tracheobronchial stenosis with wall thickening, and histological examination of the bronchial biopsies confirmed the diagnosis of endobronchial AL amyloidosis. Owing to the poor effect of radiation therapy and treatments for improving airway patency, he was treated with a systemic chemotherapy regimen [cyclophosphamide-bortezomib-dexamethasone (CyBorD)]. We observed substantial improvements in his dyspnea, highlighting the potential of systemic therapy to improve quality of life of patients with tracheobronchial AL amyloidosis. However, the long-term pathological changes associated with local bronchial lesions require further investigation.

Abstract Amyloidosis is an uncommon heterogeneous and multi‐systemic disease characterized by extracellular amyloid deposition. The size of proteins varies and forms a part of local disease or systemic process. Light chain amyloidosis (AL) is the most prevalent form of systemic amyloidosis which may also be seen in localized disease. Isolated tracheobronchial amyloidosis (TBA) is rather unusual with local amyloid deposition which may pose a diagnostic dilemma with subsequent therapeutic challenge. Awareness of such a presentation is crucial in the diagnosis of this rare disease. We describe three cases who presented with haemoptysis, which on further evaluation were diagnosed as isolated TBA, and a review of literature.

Amyloidosis is a spectrum of diseases, in which various proteins which are usual components of plasma are deposited as insoluble beta-pleated sheets extracellularly, disrupting function of various organs. Amyloid light-chain amyloidosis occurs due to the deposition of proteins, derived from immunoglobulin light chains, routinely manifesting with multisystem involvement. Pulmonary involvement is seen in about 50% of cases. Three common patterns of pulmonary amyloidosis on computed tomography (CT) chest are tracheobronchial, nodular parenchymal, and diffuse alveolar septal variety. We hereby report two cases of pulmonary amyloidosis, one being a case of diffuse alveolar septal pulmonary amyloidosis, which is an extremely rare pattern of involvement, with a very poor prognosis, and the other one being tracheobronchial pattern of involvement, which usually results due to the localized deposition of amyloid in the tracheobronchial tree. Knowledge about pulmonary amyloidosis is important due to its poor prognosis and nonspecific findings in CT chest.

Amyloidosis is an uncommon heterogeneous and multi-systemic disease characterized by extracellular amyloid deposition. The size of proteins varies and forms a part of local disease or systemic process. Light chain amyloidosis (AL) is the most prevalent form of systemic amyloidosis which may also be seen in localized disease. Isolated tracheobronchial amyloidosis (TBA) is rather unusual with local amyloid deposition which may pose a diagnostic dilemma with subsequent therapeutic challenge. Awareness of such a presentation is crucial in the diagnosis of this rare disease. We describe three cases who presented with haemoptysis, which on further evaluation were diagnosed as isolated TBA, and a review of literature. [ABSTRACT FROM AUTHOR]

Tracheobronchopathia osteochondroplastica (TPO) is an idiopathic disease involving the cartilage rings of the large airway, characterized by submucosal calcified nodules. Localized tracheobronchial amyloidosis (TBA) is another rare disease with localized amyloid deposits in the tracheobronchial tree. The two diseases rarely coincide, and only a few case reports and series have been reported.A patient with dyspnea was referred to our clinic for suspicion of TBA. Chest computed tomography (CT) scan showed marked thickening of the tracheobronchial wall with calcified endobronchial submucosal nodules. The nodules were resected with a Diode Laser under rigid bronchoscopy, and results from the biopsy showed both osteochondroid metaplasia on microscopy in Hematoxylin and Eosin staining and apple-green birefringence on polarized microscopy in Congo red staining. This is a rare case in which microscopic findings of both TPO and TBA were observed on one slide. These findings suggest that localized TBA could be a cause of TPO.

Amyloidosis is a spectrum of diseases, in which various proteins which are usual components of plasma are deposited as insoluble beta-pleated sheets extracellularly, disrupting function of various organs. Amyloid light-chain amyloidosis occurs due to the deposition of proteins, derived from immunoglobulin light chains, routinely manifesting with multisystem involvement. Pulmonary involvement is seen in about 50% of cases. Three common patterns of pulmonary amyloidosis on computed tomography (CT) chest are tracheobronchial, nodular parenchymal, and diffuse alveolar septal variety. We hereby report two cases of pulmonary amyloidosis, one being a case of diffuse alveolar septal pulmonary amyloidosis, which is an extremely rare pattern of involvement, with a very poor prognosis, and the other one being tracheobronchial pattern of involvement, which usually results due to the localized deposition of amyloid in the tracheobronchial tree. Knowledge about pulmonary amyloidosis is important due to its poor prognosis and nonspecific findings in CT chest. [ABSTRACT FROM AUTHOR]

Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease. Typically lung parenchymal and tracheobronchial amyloidosis do not present together in localized forms of pulmonary amyloidosis. Here we report a unique case of localized pulmonary immunoglobulin light-chain amyloidosis, manifested as both parenchymal nodules and tracheobronchial amyloid deposition. [ABSTRACT FROM AUTHOR]

Amyloidosis is a disease caused by misfolded proteins that deposit in the extracellular matrix as fibrils, resulting in the dysfunction of the involved organ. The lung is a common target of Amyloidosis, but pulmonary amyloidosis is uncommonly diagnosed since it is rarely symptomatic. Diagnosis of pulmonary amyloidosis is usually made in the setting of systemic amyloidosis, however in cases of localized pulmonary disease, surgical or transbronchial tissue biopsy might be indicated. Pulmonary amyloidosis can be present in a variety of discrete entities. Diffuse Alveolar septal amyloidosis is the most common type and is usually associated with systemic AL amyloidosis. Depending on the degree of the interstitial involvement, it may affect alveolar gas exchange and cause respiratory symptoms. Localized pulmonary Amyloidosis can present as Nodular, Cystic or Tracheobronchial Amyloidosis which may cause symptoms of airway obstruction and large airway stenosis. Pleural effusions, mediastinal lymphadenopathy and pulmonary hypertension has also been reported. Treatment of all types of pulmonary amyloidosis depends on the type of precursor protein, organ involvement and distribution of the disease. Most of the cases are asymptomatic and require only close monitoring. Diffuse alveolar septal amyloidosis treatment follows the treatment of underlying systemic amyloidosis. Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions including debulking and stenting or with external beam radiation. Long-term prognosis of pulmonary amyloidosis usually depends on the type of lung involvement and other organ function., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Primary localized amyloidosis of the airways is an uncommon disorder characterized by amyloid deposits in the airway mucosa. In contrast to systemic amyloidosis other organs are not involved. Among the entities of airway amyloidosis, tracheobronchial amyloidosis is comparatively the most common subtype in the lower respiratory tract and laryngeal amyloidosis in the upper respiratory tract. The pathophysiology of localized airway amyloidosis is poorly understood. The clinical presentation is variable and often non-specific. No general consensus exists with regard to optimal treatment resulting in a variety of modalities used in clinical practice to manage this disorder. We report the case of a 50 year old woman with multifocal localized amyloidosis of the tracheobronchial tree and the upper airways. Tracheobronchial amyloidosis was treated with endoscopic debulking and external beam radiation, sinunasal amyloid deposits were surgically excised and are currently under surveillance. The importance of this extremely rare case lies in the multifocal presentation of an uncommon disorder requiring a multidisciplinary approach to offer optimal treatment including external beam radiation.

Primary localized tracheobronchial amyloidosis ( TBA) is a rare respiratory tract dysfunction, which is a heterogeneous group of diseases involving abnormal extracellular deposition of amyloid and autologous fibrillar protein material in β-pleated sheets. A 64-year-old man was referred to our hospital because of hemoptysis. Physical examination showed decreased breath sounds in the right lung on auscultation. Chest computed tomography scan displayed multiple nodules with varied size in main bronchia around bilateral hilus of the lung. After admission, bronchoscopy was performed for this patient, and roughness of mucosa in trachea and multiple nodules in respiratory tract were observed. Through further tissue biopsy, the diagnosis of primary TBA was confirmed. [ABSTRACT FROM AUTHOR]

Tracheobronchial amyloidosis is an infrequent disease characterized by the deposition of proteinaceous material in the tracheobronchial tree. The disease generally has a high morbidity and variable mortality in the years following diagnosis. There is no consensus on the optimal treatment. We report a case of a 63-year-old woman who presented with a diffuse tracheobronchial amyloidosis associated with laryngeal involvement, which required a percutaneous tracheostomy due to high-grade subglottic stenosis, with no evidence of systemic amyloidosis. After treatment exclusively with colchicine, she had a complete resolution of the stenotic area, with a very good response from the tracheobronchial amyloidosis disease, with only minor yellow plaques persisting. The patient has remained asymptomatic in the next 4 years of follow-up, with no evidence of endoscopic progression. This is the first documented case of this kind of response of tracheobronchial amyloidosis to colchicine treatment alone. A review of the available literature is presented. [ABSTRACT FROM AUTHOR]

Amyloid is a heterogeneous family of extracellular proteinaceous deposits characterized by apple-green birefringence on polarized light microscopy. There are rare case reports of these extracellular deposits accumulating in the upper and central airways. Progressive infiltration may impair glottic and airway function with some cases requiring intervention to improve flow. Bronchoscopy and lung function testing provide dynamic information to monitor for disease progression; however, the recent development of 320 multislice computed tomography (320 CT) enables dynamic, four-dimensional (4-D) evaluation of laryngeal and tracheal structure and function and presents as a noninvasive, low-radiation dose surveillance tool. We reviewed a 43-year-old man with primary amyloidosis of the larynx and central airways who presented with an 18-year history of progressive dysphonia without breathlessness and preserved lung function. 4-D CT demonstrated marked thickening of supraglottic folds and trachea with marked tracheal dilatation. Despite gross structural abnormalities, dynamic function assessed throughout inspiration and expiration was normal, demonstrating neither rigidity nor dynamic collapse. This combination of structural and functional assessment of the proximal airway by 4-D CT is a novel application to surveillance for laryngeal and tracheal amyloid. [ABSTRACT FROM AUTHOR]

Amyloidosis is a systemic disease characterized by extensive accumulation of poorly soluble autologous fibrous proteins in the extracellular space of various organs. Respiratory tract involvement is rarely seen in primary amyloidosis. Respiratory primary amyloidosis is named as tracheobronchial amyloidosis. We want to offer our patients that we have in mind the purpose of this preliminary diagnosis. repeatedly in the last 10 years with the same complaints. She was performed nonspecific therapies and followed up. Progressive dyspnea and coughing forced us to see a computerized tomography of thorax. Images revealed parenchymal changes. In Positron Emission Tomography peribroncial lesions with high FDG uptake were detected. We determined an obliterative lesion in bronchoscopic examination which is almost fully obstructing the tracheobronchial tree. Pathologic evaluation was reported as amyloidosis. We wanted to share a tracheobronchial amyloidosis case while this is a rare but curable entity with satisfactory results by multimodal treatment strategies. [ABSTRACT FROM AUTHOR]

Amyloidosis is a systemic disease involving abnormal extracellular deposition of amyloid and autologous fibrillar protein material in β-pleated sheets. Accumulation of this abnormal protein leads to organ dysfunction, although respiratory tract involvement is rare. We present two cases of tracheobronchial amyloidosis successfully treated with surgery and radiation. [ABSTRACT FROM PUBLISHER]

Primary isolated tracheobronchial amyloidosis is an uncommon disease. It manifests in symptoms such as progressive dyspnea, cough, and haemoptysis. Airway obstruction can cause atelectasis and recurrent bronchopulmonary infections. A 47-year old man was admitted with the complaint of dyspnea upon exertion of 15 years duration. It is learned that he had a productive cough in winter, and received treatment for bronchial asthma, but had no recovery. Hemoptysis added to his complaints for 10 days, and he had a smoking history of 30 packet/year. Upon physical examination, he had prolonged expirium, and other system signs were normal. Laboratory findings; WBC: 7.100, Hb: 14, Hct: 42.6%, Plt: 479.000, ESR: 80 mm/sec, biochemical parameters were within the normal range. The posteroanterior radiogram of the chest revealed bilateral hilar fullness, and double contour on the right diaphragm. Results of pulmonary function tests were as follows; FEV1/FVC: 44%, FEV1: 47%, FVC: 84%. A computed tomographys can of the chest showed clear emphysemal areas in lung parenchyma, and diffuse thickening on the wall of the trachea, bilateral main bronchii, and segmental bronchii. Fiberoptic bronchoscopy revealed multiple endobronchial lesions on the walls of trachea,and subsegmental bronchii, and mucosal infiltration. A biopsy from these lesions revealed pink-purple amyloid accumulation on mucosal connective tissue, mucosal gland wall, and mucosal vascules after painting with crystal violet and Kongo red. The patient was diagnosed as tracheobronchial amyloidosis, and argon plasma coagulation applied under general anesthesia first to the left main bronchus, and then the right main bronchus and trachea during 2 sessions. After coagulation, mechanical resection was applied.Cryotherapy was applied to the remaining tissue. After treatment, his symptoms were significantly improved. Although spontaneousresolution has been reported in the tracheobronchial amyloidosis, most cases require multiple therapeutic interventions in order to control progressive respiratory symptoms. [ABSTRACT FROM AUTHOR]

Summary: The aim of the prospective study was to explore the safety and effectiveness of external beam radiation therapy (EBRT) in three patients with biopsy proven primary pulmonary amyloidosis, including two tracheobronchial amyloidosis patients and one primary parenchymal amyloidosis patient. All three patients were treated to 24Gy in 12 fractions utilizing CT simulation and 3-D planning. All three patients had significant improvement in clinical symptoms, radiological imaging as well as pulmonary function tests. The improvement in the clinical symptoms was evident in 2 days. Toxicities related to EBRT were not observed during the follow-up range from 42 to 54 months. EBRT to 24Gy was safe and effective in the three patients with primary pulmonary amyloidosis, and resulted in rapid relief of pulmonary symptoms. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the efficacy of conformal external beam radiotherapy (RT) for local control of progressive airway amyloidosis. Methods and Materials: We conducted a retrospective review of patients with biopsy-proven progressive airway amyloidosis treated with conformal RT between 2000 and 2006 at Boston Medical Center. The patients were evaluated for performance status and pulmonary function, with computed tomography and endoscopy after RT compared with the pretreatment studies. Local control was defined as the lack of progression of airway wall thickening on computed tomography imaging and stable endobronchial deposits by endoscopy. Results: A total of 10 symptomatic airway amyloidosis patients (3 laryngeal and 7 tracheobronchial) received RT to a median total dose of 20 Gy in 10 fractions within 2 weeks. At a median follow-up of 6.7 years (range, 1.5–10.3), 8 of the 10 patients had local control. The remaining 2 patients underwent repeat RT 6 and 8.4 months after initial RT, 1 for persistent bronchial obstruction and 1 for progression of subglottic amyloid disease with subsequent disease control. The Eastern Cooperative Oncology Group performance status improved at a median of 18 months after RT compared with the baseline values, from a median score of 2 to a median of 1 (p = .035). Airflow (forced expiratory volume in 1 second) measurements increased compared with the baseline values at each follow-up evaluation, reaching a 10.7% increase (p = .087) at the last testing (median duration, 64.8 months). Acute toxicity was limited to Grade 1-2 esophagitis, occurring in 40% of patients. No late toxicity was observed. Conclusions: RT prevented progressive amyloid deposition in 8 of 10 patients, resulting in a marginally increased forced expiratory volume in 1 second, and improved functional capacity, without late morbidity. [ABSTRACT FROM AUTHOR]

The article presents a study on the significance of external beam radiation therapy (EBRT) in treating tracheobronchial amyloidosis. A study involving individuals with tracheobronchial amyloidosis in Rochester, Minnesota manifested the safe therapeutic use of ERBT as well as its potential in giving symptomatic and objective improvement to patients. It is noted that the restrictions of treatment, recurrence, and complications are major concerns in the management of the disease.

Between June 1983 and December 2002, 32 patients were treated for primary localized laryngo-tracheobronchial amyloidosis (LTBA) at our institution. For enrollment in this retrospective study, at least one positive biopsy with Congo red stain and a diagnostic test battery excluding systemic or secondary amyloidosis were mandatory. The most common presenting symptom was dyspnea, which was observed in 75% of cases. Endoscopic appearance was described as submucosal plaques and nodules with a cobblestone appearance in 44% of patients, tumor-like in 28% and circumferential wall thickening in 28% of cases. Tracheobronchopatia osteochondroplastica was associated with LTBA in 22% of patients, both at the first treatment (four cases) or during follow-up examinations (three). Synchronous or metachronous disease in the larynx and tracheobronchial tree was observed in 47% of cases, while only one patient showed lung involvement with extensive amyloidosis of the trachea and bronchi. All but two patients were endoscopically treated. One of these was affected by a tracheal amyloidoma cured by endoscopic debulking and further tracheal resection-anastomosis. The other patient presented massive laryngo-pharyngeal involvement and was therefore treated by total laryngectomy. A total of 58 endoscopic procedures (range: 1–7; mean: two per patient) were carried out, 11 with CO2 laser (for supraglottic, glottic and selected subglottic lesions) and 47 with Nd:YAG laser (for the most part of subglottic lesions and amyloid deposits located in trachea and bronchi). Four patients required a tracheotomy and three necessitated a T-tube stent during management, which was subsequently removed in all cases. One patient was lost to follow-up, nine were endoscopically controlled elsewhere (Group A) and 22 were followed at our institution (Group B). In Group A, the patient with tracheal amyloidoma is asymptomatic and free of additional LTBA localizations 8 years after surgery. The remaining eight are asymptomatic, but with persistent endoscopic signs of amyloidosis. In Group B, five patients died: one from heart failure and another one from pneumonia 3 and 4 days after surgery, respectively. In one case death was from unrelated causes, and in the two remaining patients it was from respiratory failure due to uncontrolled bilateral bronchial amyloidosis in the subsequent 5 to 6 years. One patient is completely cured 5 years after surgery, and 16 are asymptomatic with persistence of LTBA. [ABSTRACT FROM AUTHOR]

Primary localized amyloidosis of the airways is an uncommon disorder characterized by amyloid deposits in the airway mucosa. In contrast to systemic amyloidosis other organs are not involved. Among the entities of airway amyloidosis, tracheobronchial amyloidosis is comparatively the most common subtype in the lower respiratory tract and laryngeal amyloidosis in the upper respiratory tract. The pathophysiology of localized airway amyloidosis is poorly understood. The clinical presentation is variable and often non-specific. No general consensus exists with regard to optimal treatment resulting in a variety of modalities used in clinical practice to manage this disorder. We report the case of a 50 year old woman with multifocal localized amyloidosis of the tracheobronchial tree and the upper airways. Tracheobronchial amyloidosis was treated with endoscopic debulking and external beam radiation, sinunasal amyloid deposits were surgically excised and are currently under surveillance. The importance of this extremely rare case lies in the multifocal presentation of an uncommon disorder requiring a multidisciplinary approach to offer optimal treatment including external beam radiation. [ABSTRACT FROM AUTHOR]

The author reports on the electron-microscopic examination of the diffuse tracheobronchial amyloidosis of a 51-year-old patient. The amyloid deposits were located in the lamina propria of the tracheal and bronchial mucosa. At the edge of the nodular deposits, condensation and radial arrangement of amyloid fibrils could be seen. Closely connected with the amyloid, active fibroblasts were present and their cytoplasm contained amyloid fibrils. The cell membrane of the fibroblasts was missing in part. Intracellular amyloid fibrils mingled with extracellular deposits. It can be assumed that active fibroblasts play an important role in local amyloid formation. [ABSTRACT FROM AUTHOR]

Amyloidosis is a rare disorder caused by amyloid deposits in various organs and tissues resulting in vital organ dysfunction, eventually death. There are two forms of amyloidosis - systemic, characterized by multiple organs affected, and localized (focal). Localized forms of amyloidosis usually affect urinary bladder, skin and lungs. Pulmonary amyloidosis may be localized or systemic such as diffuse alveolo-septal pulmonary amyloidosis which usually accompanies systemic AL amyloidosis. Other two forms of pulmonary amyloidosis are tracheobronchial and nodular. All three forms are usually detected by accident when patients undergo chest examination for different reasons as most cases of pulmonary amyloidosis are asymptomatic. The prognosis of localized amyloidosis is good with 5-year overall survival being 90,6 %. In our case report we present three patients diagnosed with localized pulmonary amyloidosis at our center. In all cases the diagnoses were made following the resection of affected lung segments with no further treatment needed.

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