Citation, DOI, disclosures and article data
At the time the article was created Yuranga Weerakkody had no recorded disclosures.View Yuranga Weerakkody's current disclosures
At the time the article was last revised Mostafa El-Feky had no recorded disclosures.View Mostafa El-Feky's current disclosures
Pulmonary amyloidosis is a rare manifestation of amyloidosis characterized by involvement of lung parenchyma. Two patterns are described:
- nodular parenchymal amyloidosis (more common)
- diffuse parenchymal amyloidosis (least common type)
Sometimes tracheobronchial amyloidosis is included under this entity, although the term thoracic amyloidosis is more accurate.
On this page:
Amyloidosis is of unknown incidence but is estimated to affect approximately 8 in 100,000 people from Western Europe and the United States 10.
The disease is characterized by extracellular deposition of fibrillary protein in the lungs. With lung parenchymal involvement there are two distinct forms:
- nodular parenchymal amyloidosis / nodular pulmonary amyloidosis (more common)
- diffuse parenchymal amyloidosis / diffuse pulmonary amyloidosis / alveolar septal amyloidosis / diffuse interstitial amyloidosis (least common type) 4
The disease can be primary or secondary where the term primary pulmonary amyloidosis is given when the disease is confined to the lungs 3. With pulmonary amyloidosis, the primary form tends to be more common 3. 30% of patients with primary pulmonary amyloidosis are ultimately diagnosed with multiple myeloma or B cell lymphoma, and 10-15% of patients with myeloma or lymphoma will develop pulmonary amyloidosis 9.
The most common pulmonary manifestation is amyloid deposition in small pulmonary artery branches 11.
There are many imaging patterns depending on the form and it may be better to refer to individual subtypes.
- 1. Kirchner J, Jacobi V, Kardos P et-al. CT findings in extensive tracheobronchial amyloidosis. Eur Radiol. 1998;8 (3): 352-4. Eur Radiol (link) - Pubmed citation
- 2. Georgiades CS, Neyman EG, Barish MA et-al. Amyloidosis: review and CT manifestations. Radiographics. 2004;24 (2): 405-16. Radiographics (full text) - doi:10.1148/rg.242035114 - Pubmed citation
- 3. Vieira IG, Marchiori E, Zanetti G et-al. Pulmonary amyloidosis with calcified nodules and masses - a six-year computed tomography follow-up: a case report. Cases J. 2009;2 (1): 6540. doi:10.4076/1757-1626-2-6540 - Free text at pubmed - Pubmed citation
- 4. Lee AY, Godwin JD, Pipavath SN. Case 182: pulmonary amyloidosis. Radiology. 2012;263 (3): 929-32. Radiology (full text) - doi:10.1148/radiol.12103540 - Pubmed citation
- 5. GiméNez A, Franquet T, Prats R et-al. Unusual primary lung tumors: a radiologic-pathologic overview. Radiographics. 2002;22 (3): 601-19. Radiographics (full text) - Pubmed citation
- 6. Urban BA, Fishman EK, Goldman SM et-al. CT evaluation of amyloidosis: spectrum of disease. Radiographics. 1993;13 (6): 1295-308. Radiographics (abstract) - Pubmed citation
- 7. Lantuejoul S, Moulai N, Quetant S et-al. Unusual cystic presentation of pulmonary nodular amyloidosis associated with MALT-type lymphoma. Eur. Respir. J. 2007;30 (3): 589-92. Eur. Respir. J. (full text) - doi:10.1183/09031936.00136605 - Pubmed citation
- 8. Rajagopala S, Singh N, Gupta K et-al. Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature. Respirology. 2010;15 (5): 860-6. Respirology (full text) - doi:10.1111/j.1440-1843.2010.01772.x - Pubmed citation
- 9. Webb WR, Muller NL, Naidich DP. High-resolution CT of the lung. Lippincott Williams & Wilkins. ISBN:0781769094. Read it at Google Books - Find it at Amazon
- 10. Czeyda-Pommersheim F, Hwang M, Chen SS, Strollo D, Fuhrman C, Bhalla S. Amyloidosis: Modern Cross-sectional Imaging. (2015) Radiographics : a review publication of the Radiological Society of North America, Inc. 35 (5): 1381-92. doi:10.1148/rg.2015140179 - Pubmed
- 11. Coolbear F, Bilawich AM, Tongson J, Adamo J, Churg A. Pleural amyloidosis imitating pleural malignancy. (2017) Respiratory medicine case reports. 20: 195-197. doi:10.1016/j.rmcr.2017.03.004 - Pubmed
- 12. Milani P, Basset M, Russo F, Foli A, Palladini G, Merlini G. The Lung in Amyloidosis. Eur Respir Rev. 2017;26(145):170046. doi:10.1183/16000617.0046-2017 - Pubmed