ArticleFrontiers in immunology2025
Case Report: Relapsing pleural effusions and coated aorta revealing Erdheim-Chester disease.
Article in Frontiers in immunology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Erdheim-Chester disease (ECD) is a rare histiocytic disorder with localized presentations or multisystem disease. Clinical presentations of ECD are usually non-specific and depends on the site of involvement. ECD can involve one or several organs. Clinical manifestations range from asymptomatic lesions to severe and life-threatening organ dysfunction. Hence, accurate and timely diagnosis is challenging given the rarity and varied presentation of ECD. The most common clinical manifestations are bone pain related to osteosclerosis, usually in the lower limbs. We report here a case with no obvious clinical manifestation of ECD preceding initial recurrent pleural effusions. The diagnosis of ECD was suggested based on pleural thickening revealed by relapsing pleural effusions combined with radiological finding of a coated aorta and slight perirenal infiltrate. Pleural biopsy revealed collagen fibrosis, and immunohistochemistry with the anti-CD163 antibody showed an important infiltration by histiocytes, strong cytoplasmic phosphorylated ERK in the lesional cells, and positive factor XIIIa staining. A cell-free DNA from peripheral blood revealed negative
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