Management of tracheobronchial amyloidosis: a review of the literature
Introduction Tracheobronchial amyloidosis is a rare idiopathic disorder characterised by extracellular deposition of misfolded protein fibrils in the tracheobronchial tree. It presents with nonspecific symptoms. Deciding on the best treatment approach can be challenging due to the lack of a treatmen...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
European Respiratory Society
2024-02-01
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| Series: | ERJ Open Research |
| Online Access: | http://openres.ersjournals.com/content/10/1/00540-2023.full |
| _version_ | 1797278872428871680 |
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| author | Illaa Smesseim Paul Cobussen Ricky Thakrar Hans Daniels |
| author_facet | Illaa Smesseim Paul Cobussen Ricky Thakrar Hans Daniels |
| author_sort | Illaa Smesseim |
| collection | DOAJ |
| description | Introduction
Tracheobronchial amyloidosis is a rare idiopathic disorder characterised by extracellular deposition of misfolded protein fibrils in the tracheobronchial tree. It presents with nonspecific symptoms. Deciding on the best treatment approach can be challenging due to the lack of a treatment guideline. We undertook a review to assess the therapeutic options for tracheobronchial amyloidosis and to highlight gaps within the existing evidence.
Methods
We performed a literature search from 1 January 1990 until 1 March 2022 to identify relevant literature regarding patient characteristics, symptoms, management and prognosis for patients with tracheobronchial amyloidosis.
Results
77 studies consisting of 300 patients were included. We found a great heterogeneity in the management of tracheobronchial amyloidosis 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 Sjögren syndrome (n=5, 1.7%) and tracheobronchial amyloidosis compared to the normal population (0.5–1.0%).
Conclusions
There is a great heterogeneity in the management of tracheobronchial amyloidosis 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. |
| first_indexed | 2024-03-07T16:15:23Z |
| format | Article |
| id | doaj.art-e03207f79d8e4ad4814f4252a45bfd5f |
| institution | Directory Open Access Journal |
| issn | 2312-0541 |
| language | English |
| last_indexed | 2024-03-07T16:15:23Z |
| publishDate | 2024-02-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | ERJ Open Research |
| spelling | doaj.art-e03207f79d8e4ad4814f4252a45bfd5f2024-03-04T11:30:00ZengEuropean Respiratory SocietyERJ Open Research2312-05412024-02-0110110.1183/23120541.00540-202300540-2023Management of tracheobronchial amyloidosis: a review of the literatureIllaa Smesseim0Paul Cobussen1Ricky Thakrar2Hans Daniels3 Department of Respiratory Medicine, Netherlands Cancer Institute, Amsterdam, The Netherlands Department of Radiation Oncology, Amsterdam University Medical Center, Amsterdam, The Netherlands Department of Respiratory Medicine, University College London Hospitals, London, UK Department of Respiratory Medicine, Amsterdam University Medical Center, Amsterdam, The Netherlands Introduction Tracheobronchial amyloidosis is a rare idiopathic disorder characterised by extracellular deposition of misfolded protein fibrils in the tracheobronchial tree. It presents with nonspecific symptoms. Deciding on the best treatment approach can be challenging due to the lack of a treatment guideline. We undertook a review to assess the therapeutic options for tracheobronchial amyloidosis and to highlight gaps within the existing evidence. Methods We performed a literature search from 1 January 1990 until 1 March 2022 to identify relevant literature regarding patient characteristics, symptoms, management and prognosis for patients with tracheobronchial amyloidosis. Results 77 studies consisting of 300 patients were included. We found a great heterogeneity in the management of tracheobronchial amyloidosis 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 Sjögren syndrome (n=5, 1.7%) and tracheobronchial amyloidosis compared to the normal population (0.5–1.0%). Conclusions There is a great heterogeneity in the management of tracheobronchial amyloidosis 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.http://openres.ersjournals.com/content/10/1/00540-2023.full |
| spellingShingle | Illaa Smesseim Paul Cobussen Ricky Thakrar Hans Daniels Management of tracheobronchial amyloidosis: a review of the literature ERJ Open Research |
| title | Management of tracheobronchial amyloidosis: a review of the literature |
| title_full | Management of tracheobronchial amyloidosis: a review of the literature |
| title_fullStr | Management of tracheobronchial amyloidosis: a review of the literature |
| title_full_unstemmed | Management of tracheobronchial amyloidosis: a review of the literature |
| title_short | Management of tracheobronchial amyloidosis: a review of the literature |
| title_sort | management of tracheobronchial amyloidosis a review of the literature |
| url | http://openres.ersjournals.com/content/10/1/00540-2023.full |
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