Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Elfeky M, Murphy A, et al. Erdheim-Chester disease (pulmonary manifestations). Reference article, Radiopaedia.org (Accessed on 30 Mar 2024) https://doi.org/10.53347/rID-29370
Pulmonary manifestations of Erdheim-Chester disease are uncommon.
The lungs are affected in ~25% (range 20-35%) of cases 5.
HRCT chest
Described findings include 1
Look also extra-pulmonary features such as
- peri-aortic infiltration: may extend to the ascending aorta, coronary arteries, and aortic branches
- infiltration of the pulmonary arteries, superior vena cava, pericardium, and myocardium
Treatment and prognosis
Pulmonary involvement is generally an indicator or poor prognosis 4.
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1. Wittenberg KH, Swensen SJ, Myers JL. Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings. AJR Am J Roentgenol. 2000;174 (5): 1327-31. doi:10.2214/ajr.174.5.1741327 - Pubmed citation
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2. Chung JH, Park MS, Shin DH et-al. Pulmonary involvement in Erdheim-Chester disease. Respirology. 2005;10 (3): 389-92. doi:10.1111/j.1440-1843.2005.00671.x - Pubmed citation
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3. Kong PM, Pinheiro L, Kaw G et-al. Erdheim-Chester disease: a rare cause of interstitial lung disease. Singapore Med J. 2007;48 (2): e57-9. Pubmed citation
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4. Protopapadakis C, Antoniou KM, Nicholson AG et-al. Erdheim-Chester disease: pulmonary presentation in a case with advanced systemic involvement. Respiration. 2009;77 (3): 337-40. doi:10.1159/000129758 - Pubmed citation
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5. Ahuja J, Kanne JP, Meyer CA et-al. Histiocytic disorders of the chest: imaging findings. Radiographics. 2015;35 (2): 357-70. doi:10.1148/rg.352140197 - Pubmed citation
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