Erdheim‐Chester disease progression from miliary pulmonary nodules to large tumours

Abstract

Erdheim‐Chester disease (ECD), a rare form of non‐Langerhans cell histiocytosis, affects long bones, the retroperitoneal region, and the central nervous, cardiovascular, and pulmonary systems. Most patients with ECD show interlobular septal thickening, centrilobular micronodules, and ground glass opacities on chest computed tomography (CT). We encountered a 66‐year‐old man with ECD who presented at first visit with randomly distributed multiple pulmonary nodules and who then developed large tumour shadows, observed on chest CT. To our knowledge, this random distribution pattern of multiple pulmonary nodules has not previously been reported.

Cardiac and pleuropulmonary involvement in Erdheim-Chester disease without bone lesions: A case report

“…Knowledge of the clinical context helps diagnosis if biopsy results are not available. We must emphasize that radiologic pleuropulmonary and cardiac manifestations of ECD may precede clinical manifestations [ 4 , 8 ], as observed in the present case.…”

“…Pleuropulmonary involvement in ECD is also common and is observed in approximately half of the cases. Radiologic evidence of pleuropulmonary disease is also non-specific but often presents as inter-lobular septal, pleural, or fissural thickening, pulmonary nodules, ground-glass opacities, and effusion [ 1 , 8 ]. The differential diagnosis is wide, including lymphangitic carcinomatosis, tuberculosis, lymphoma sarcoidosis, and some interstitial pneumonias.…”

“…Meanwhile, extra-skeletal manifestations are reported in more than half of the cases, mainly affecting the central nervous, respiratory, cardiovascular, and renal systems, including the retroperitoneum. Pleuropulmonary and cardiovascular involvement have also been reported [8] , [9] , [10] . Although frequently asymptomatic, cardiac involvement is present in about half the cases and is associated with a poor prognosis [ 7 , 9 ].…”

Examining Miliary Disease Etiology in a Coccidioides-Endemic Center: A Retrospective Cohort Study

“…Various metastatic cancers and other neoplastic processes are known causes of miliary disease [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. Several interstitial lung diseases can present atypically with a miliary pattern, such as sarcoidosis [21], pulmonary alveolar microlithiasis [22], various pneumoconioses [23], hypersensitivity pneumonitis [24], lipoid pneumonia [25], vaping-associated lung injury [26], drug-induced pneumonitis [27], and organizing pneumonia [28].…”