Presentation
Dyspnea.
Patient Data
Circumferential soft tissue infiltration of the right atrial wall and septum with intense late gadolinium enhancement (LGE), extending into the SVC and the aortic root, also visible on the short-axis view.
Annotated images showing the circumferential soft tissue infiltration of the right atrium with intense late gadolinium enhancement demonstrated on the motion-corrected free-breathing PSIR image.
Image credit: Dr Azza Elgendy
PET CT shows non FDG avid infiltration surrounding both kidneys.
Case Discussion
Erdheim-Chester disease and immunoglobulin G4-related diseases are rare diseases with potential cardiac involvement. Both diseases can affect the heart and kidneys resulting in dark T2 signal intensity on SSFP and intense late gadolinium enhancement (LGE). FDG avidity is variable, usually low. Both diseases progress slowly.
In this case, there are bilateral perirenal masses and right atrial mass-like infiltration.
Biopsy of the right atrial mass showed histiocytes, xanthogranulomatous and mixed inflammatory infiltrate on a background of fibrosis suggesting Erdheim-Chester disease.
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