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Amyloidosis and the Respiratory Tract

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Abstract

Amyloidosis can both complicate long standing respiratory conditions and be deposited within the respiratory system itself. Its presentation is extremely heterogeneous with either a benign or life threatening course. The manifestations, significance and prognosis of amyloid deposits depend on the underlying aetiology and anatomical distribution. Each patient requires thorough evaluation to determine the extent and severity of amyloid deposition and to ensure the optimal treatment. In most cases of localised amyloidosis disease management is resection or ablation of symptomatic deposits. In contrast, systemic anti-inflammatory treatment and chemotherapy can be extremely effective in patients with the systemic AA and AL forms of the disease. Encouragingly, specific drug therapies designed to stabilize amyloid precursor proteins, interfere with amyloid fibrillogenesis and accelerate the clearance of tissue amyloid deposits are all on the horizon.

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Acknowledgements

We would like to thank Dorothea Gopal and David Hutt for their help in acquiring and processing the radiographic images. We would also like to thank Janet Gilbertson for aiding in acquiring and processing the pathology images.

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Authors and Affiliations

  1. Division of Medicine, Department of Medical Oncology, University of Alberta, Cross Cancer Institute, 11560 University Avenue, Edmonton, Alberta, Canada, T6G 1Z2

    Christopher P. Venner MD

  2. Division of Medicine, UK National Amyloidosis Centre and UCL Centre for Nephrology, Center for Amyloidosis and Acute Phase Proteins, University College London Medical School, Royal Free Campus, Rowland Hill Street, London, NW3 2PF, UK

    Jennifer H. Pinney BMedSci, MRCP & Helen J. Lachmann MA, MB, BChir, MD, FRCP

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  1. Christopher P. Venner MD
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  2. Jennifer H. Pinney BMedSci, MRCP
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  3. Helen J. Lachmann MA, MB, BChir, MD, FRCP
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Correspondence to Christopher P. Venner MD .

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Editors and Affiliations

  1. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Vincent Cottin

  2. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Jean-Francois Cordier

  3. Respiratory Medicine, University of Southampton, Southampton, United Kingdom

    Luca Richeldi

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Venner, C.P., Pinney, J.H., Lachmann, H.J. (2015). Amyloidosis and the Respiratory Tract. In: Cottin, V., Cordier, JF., Richeldi, L. (eds) Orphan Lung Diseases. Springer, London. https://doi.org/10.1007/978-1-4471-2401-6_7

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