Organ responses. (A-C) Chest high-resolution CT scans in patient 12 show progressive regression of ECD-related lung disease (the scans were taken respectively before treatment and at months 6 and 12 after the beginning of therapy with everolimus) (axial view). (D-E) Contrast-enhanced abdominal CT scans in patient 8 show marked regression of perirenal infiltration with consequent improvement of hydronephrosis and calycectasia (axial view). (F-I) Cardiac magnetic resonance (cine frame from steady-state free precession sequence) in a patient (#19) with cardiac involvement; the scans were taken at baseline (F,H) and after 1 year of treatment (G,I). (F) Circumferential pericardial effusion (black star) and pathologic soft tissue in the right atrioventricular groove and the right side of the interatrial septum (white stars). Arrows (H) indicate nodular thickening of the visceral pericardium. Pericardial effusion and infiltrative lesions almost completely disappeared (G,I; scans after treatment).