Erdheim-Chester disease

David Levin, Anne Marie Sykes

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Imaging description Erdheim-Chester disease is a very rare interstitial lung disease characterized by infiltration of non-Langerhans cell histiocytes or macrophages forming granulomatous lesions with fibrosis. The bones are the primary site of involvement (osteosclerotic lesions) (Figures 17.1 and 17.2), but pulmonary involvement can occur. The infiltration occurs most prominently along the lymphatics, and therefore affects the interlobular septa, bronchovascular bundles, and visceral pleura. On high-resolution CT chest, the major findings are smooth thickening of the fissures and interlobular septa, ground-glass opacities, and centrilobular nodules, which are slightly more prominent in the mid and upper lungs (Figures 17.1–17.3) [1–3]. Pleural effusions are present in roughly 50% of cases (Figure 17.1). Other common findings include pericardial thickening or effusion and extrathoracic soft tissue masses. The extrathoracic soft tissue is often seen surrounding the kidneys and in the retroperitoneum (Figures 17.2 and 17.3) [2].

Original languageEnglish (US)
Title of host publicationPearls and Pitfalls in Thoracic Imaging
Subtitle of host publicationVariants and Other Difficult Diagnoses
PublisherCambridge University Press
Pages44-47
Number of pages4
Volume9780521119078
ISBN (Electronic)9780511977701
ISBN (Print)9780521119078
DOIs
StatePublished - Jan 1 2011

ASJC Scopus subject areas

  • Medicine(all)

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    Levin, D., & Sykes, A. M. (2011). Erdheim-Chester disease. In Pearls and Pitfalls in Thoracic Imaging: Variants and Other Difficult Diagnoses (Vol. 9780521119078, pp. 44-47). Cambridge University Press. https://doi.org/10.1017/CBO9780511977701.018