Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Silverstone L, Kearns C, et al. Light chain deposition disease (pulmonary manifestations). Reference article, Radiopaedia.org (Accessed on 07 Oct 2024) https://doi.org/10.53347/rID-24849
Pulmonary light chain deposition disease is a rare manifestation of systemic light chain deposition disease (LCDD).
Pathology
Fragments of immunoglobulin light chains secreted by a plasma clone are deposited as amorphous eosinophilic material in the alveolar walls, small airways, and vessels 2 and may involve the interlobular septa 4. LCDD is Congo Red negative (in contrast to the Congo Red positive amyloid fibrils present in amyloidosis) 5. The pathogenesis of the cysts is thought to be due to multifocal lung destruction by macrophage metalloproteinases. The macrophages are attracted to sites of protein deposition. This mechanism may explain the presence of residual tissue strands within some of the cysts 6.
Radiographic features
CT
HRCT manifestations include cysts, nodules, and lymphadenopathy 4. Cysts are usually thin-walled, spherical and perivascular with vessels in the wall or traversing the cyst 4. Zonal distribution is diffuse. Cysts may slowly increase in size and number, coalescing into irregular shapes 4.
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1; Seaman DM, Meyer CA, Gilman MD et-al. Diffuse cystic lung disease at high-resolution CT. AJR Am J Roentgenol. 2011;196 (6): 1305-11. doi:10.2214/AJR.10.4420 - Pubmed citation
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2. Colombat M, Stern M, Groussard O et-al. Pulmonary cystic disorder related to light chain deposition disease. Am. J. Respir. Crit. Care Med. 2006;173 (7): 777-80. doi:10.1164/rccm.200510-1620CR - Pubmed citation
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3. Colombat M, Gounant V, Mal H et-al. Light chain deposition disease involving the airways: diagnosis by fibreoptic bronchoscopy. Eur. Respir. J. 2007;29 (5): 1057-60. doi:10.1183/09031936.00134406 - Pubmed citation
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4. Sheard S, Nicholson A, Edmunds L, Wotherspoon A, Hansell D. Pulmonary Light-Chain Deposition Disease: CT and Pathology Findings in Nine Patients. Clin Radiol. 2015;70(5):515-22. doi:10.1016/j.crad.2015.01.002 - Pubmed
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5. Fogo A, Lusco M, Najafian B, Alpers C. AJKD Atlas of Renal Pathology: Light Chain Deposition Disease. Am J Kidney Dis. 2015;66(6):e47-8. doi:10.1053/j.ajkd.2015.10.007 - Pubmed
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6. Jensen L & Mann H. Cystic Lung Disease from Protein Deposition: Pathogenesis and Associated Conditions. Curr Radiol Rep. 2018;6(3):1-10. doi:10.1007/s40134-018-0271-y
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