Tracheobronchial amyloidosis

Jivraj, Khalil; Bromley, Amy; MacEachern, Paul; Elliot, T.

doi:10.1155/2014/624615

Cited by 3 publications

(2 citation statements)

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“…Tracheobronchial amyloidosis, manifested by amyloid deposits limited specifically to tracheal and bronchial tissue, is a rare disorder with only a few hundred published cases [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] . Patients classically present with non-specific symptoms related to fixed upper airway obstruction caused by tracheal stenosis [9] .…”

Section: Discussionmentioning

confidence: 99%

Tracheobronchial amyloidosis: A case report and review of literature

Crain

Lakhani

Balar

et al. 2021

Radiology Case Reports

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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.

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Section: Discussionmentioning

confidence: 99%

Tracheobronchial amyloidosis: A case report and review of literature

Crain

Lakhani

Balar

et al. 2021

Radiology Case Reports

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“…In the current issue of the Canadian Respiratory Journal, three different cases of primary tracheobronchial amyloidosis are reported (2)(3)(4).…”

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confidence: 99%

Primary Tracheobronchial Amyloidosis: Commonly Uncommon?

Dumoulin

2014

Canadian Respiratory Journal

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Management of tracheobronchial amyloidosis: a review of the literature

Smesseim,

Cobussen,

Thakrar

et al. 2023

ERJ Open Res

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IntroductionTracheobronchial amyloidosis (TBA) is a rare idiopathic disorder characterized by extracellular deposition of misfolded protein fibrils in the tracheobronchial tree. It presents with non-specific symptoms. Deciding on the best treatment approach can be challenging due to the lack of a treatment guideline. We undertook a review to review the therapeutic options for tracheobronchial amyloidosis and to highlight gaps within the existing evidence.MethodsWe performed a literature search from 1st January 1990 until 1st March 2022 to identify relevant literature regarding patient characteristics, symptoms, management and prognosis for patients with TBA.ResultsA total of 77 studies consisting of 300 patients were included. We found a great heterogeneity in the management of TBA patients. Although a fifth of the reported patients were managed with a wait and see approach many different treatments were used as a single intervention or multiple treatments were combined. An interesting finding is the slightly higher percentage of patients with Morbus Sjögren (n=5, 1.7%) and TBA compared to the normal population (0.5% to 1.0%).ConclusionsThere is a great heterogeneity in the management of TBA patients. The treatment is still based on expert opinion due to the lack of a treatment guideline. Various treatment approaches include a wait and see approach, external beam radiotherapy, therapeutic bronchoscopy, immunosuppressive treatment and surgery.

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Tracheobronchial amyloidosis

Cited by 3 publications

References 0 publications

Tracheobronchial amyloidosis: A case report and review of literature

Tracheobronchial amyloidosis: A case report and review of literature

Primary Tracheobronchial Amyloidosis: Commonly Uncommon?

Management of tracheobronchial amyloidosis: a review of the literature

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