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Updated: Aug 1, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
A recurrent pleuropneumonia revealing Erdheim-Chester Disease
Yousra Kherabi1, Thibaud Chazal2, Jean-François Emile3
1Service de Médecine Interne, AP-HP.Nord, Hôpital Beaujon, Université Paris Cité, Clichy, France.
Abstract:
Erdheim-Chester disease (ECD) is a rare form of L group histiocytosis, accounting for up to 1500 cases to date worldwide, which mainly affects men between their 5th and 7th decade of life. The most frequent manifestations are bone involvement, perirenal infiltration with an evocating appearance of "hairy kidneys", and a "coated aorta" aspect. Lung involvement in ECD is less common and includes pleural infiltration and interstitial lung disease. Herein, we report the case of a 76-year-old woman with recurrent pleuropneumonia revealing ECD.
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