Pulmonary manifestations of Erdheim–Chester disease: clinical characteristics, outcomes and comparison with Langerhans cell histiocytosis

Abstract

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis that typically affects many organs, including the lung and pleura. However, there are few studies concerning pulmonary involvement in ECD patients, as well as the difference of pulmonary involvement between ECD and Langerhans cell histiocytosis (LCH). We performed a retrospective study of 54 ECD patients, and compared the pulmonary manifestations with those of adult LCH patients in our centre. The median age of diagnosis of the 54 ECD patients was 48 years (range 9-66 years). Chest computed tomography (CT) scans revealed lung involvement in 49 (91%) patients and pleural involvement in 34 (63%). Thirty-three (61%) patients had interstitial lung disease (ILD) with varying degrees of interlobular septal thickening, micronodules, and ground-glass opacities. ECD and LCH patients with pulmonary involvement showed significant differences in smoking status (P < 0Á001), respiratory symptoms (P = 0Á001) such as cough and pneumothorax (P < 0Á001), and radiological findings, including cysts (P < 0Á001), opacities (P < 0Á001), and pleural thickening (P < 0Á001). With a median follow-up duration of 24 months (range, 1-84 months), the estimated three-year overall survival (OS) of this entire ECD cohort was 90Á2%. Patients with ILD tended to have worse progression-free survival (PFS) than those with no ILD (P = 0Á29).

Erdheim‐Chester disease: A multisystem disease case illustration with rare manifestations and treatment challenges

“… 13 Mixed ventilation disorders have been reported in 13% of patients in Wang et al study such in our patient's case. 13 …”

“…Pulmonary disease includes parenchymal and pleural involvement. When systematically screened by chest computed tomography parenchymal abnormalities reach up to 90% of patients and pleural involvement 63% 13 in some studies and 53% and 41%, respectively, in others. 14 Interstitial lung disease including frequently septal thickening, solitary or multiple nodules, ground glass opacities and less commonly cysts.…”

“…14 Interstitial lung disease including frequently septal thickening, solitary or multiple nodules, ground glass opacities and less commonly cysts. 13 When systematically performed, pulmonary function tests are usually abnormal, with mostly diffusing‐capacity defects. 13 Mixed ventilation disorders have been reported in 13% of patients in Wang et al study such in our patient's case.…”

“… 13 When systematically performed, pulmonary function tests are usually abnormal, with mostly diffusing‐capacity defects. 13 Mixed ventilation disorders have been reported in 13% of patients in Wang et al study such in our patient's case. 13 …”

Pancreatic involvement in Erdheim-Chester disease: a case report and review of the literature

“…Theoretically, ECD can affect every tissue and organ, while so far pancreatic involvement has been reported only in one case. The main sites of involvement in ECD patients include bone (95%), lung (91%) [ 2 ], cardiovascular region (50%), retroperitoneum (40–50%), central nervous system (40%), and skin (25%) [ 3 ]. Iconic radiographic signs of ECD include the ‘hairy kidney’, sheath around the aorta, long-bone sclerosis, and right atrial pseudo tumors.…”