Need for monitoring.  As a multisystemic disease, Erdheim-Chester Disease must be closely monitored, no matter what treatment a patient might be using.  It is recommended that anyone with a positive diagnosis of ECD have the following tests performed periodically to monitor the disease:

  • Cardiac monitoring.  For patients with cardiac involvement, an additional cardiac and/or aortic evaluation should be performed every three months until the progression is stabilized with treatment, at which point studies are often extended to every six months.
  • CNS monitoring.  If CNS involvement is found or suspected, MRI evaluation of the brain with specific studies on the cerebellum should be repeated every three months until progression is stabilized with treatment.  At that point, studies are often reduced to once every six months.
  • Scans that might be considered for some patients.  PET/CT scans monitor the activity and extent of disease and response to therapy.  As advised by a physician, these tests might be considered every 3 to 6 months until the disease is stabilized.
  • Pituitary / hormone monitoring.  An evaluation of the pituitary function is suggested for some patients. A treating physician will consider whether hormone levels, including testosterone, ADH, thyroid hormones, insulin, ACTH and PTH testing by an endocrinologist (hormone specialist) is needed.
  • Skin monitoring.  For patients on BRAF- and/or MEK- inhibitors, a skin exam should be performed every three months after starting therapy, extending to every six months once the disease is stabilized.
  • Eye monitoring. For patients on MEK-inhibitors, a dedicated retinal dilated exam is recommended within 4-6 weeks of starting treatment and then can be followed up every 3-6 months, or as clinically indicated.

Physical (physio) therapy.  In addition, early initiation of physical (physio) therapy services is needed for ECD patients.  Depending upon the patient’s symptoms, this may include physical therapy, occupational therapy, and/or speech/swallowing therapy.  Patients and doctors report this is an important part of treatment management that should not be overlooked.

Psychological considerations.  Finally, any patient experiencing psychological or psychosocial issues should discuss this with their doctor.  This is not uncommon for ECD patients and treatments can help.  Patients are encouraged to continue with social interactions and leisure activities they enjoy.  Seeking counseling and getting involved with the ECDGA through offered programs have also been found to help patients.

More Information about Surviving with ECD.  See:

National Comprehensive Cancer Network (NCCN) Survivorship Issues in Adult Patients With Histiocytic Neoplasms.

Last updated: February 8, 2024

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