How is Erdheim-Chester Disease Monitored?

As a multisystemic disease, Erdheim-Chester Disease must be closely monitored, no matter what treatment a patient might be using. It is recommended that those with ECD have the following tests performed to understand the areas of involvement:

  • A systematic cardiac and aortic evaluation. For patients with cardiac involvement, an additional study should be performed every 3 months until the progression is stabilized with treatment, at which point studies are often extended to every 6 months.
  • An MRI evaluation of the brain with specific studies on the cerebellum. If CNS involvement is found or suspected, studies should be repeated every 3 months until progression is stabilized with treatment. At that point studies are often reduced to once ever 6 months.
  • Renal and renovascular evaluation, with additional studies every 3 months after starting therapy, extending to every 6 months once the disease is stabilized.
  • PET/CT scans to access and monitor the activity and extent of disease and response to therapy; this is typically done every 3 to 6 months until the disease is stabilized
  • A check of Vitamin B12 levels (400pg/ml is the recommended level)
  • A check of vitamin E levels that could be involved in neurological manifestations
  • An evaluation of the pituitary function. Hormone levels, including testosterone, ADH, thyroid hormones, insulin, ACTH and PTH should all be considered.

In addition, early initiation of rehab services is needed for ECD patients. Depending upon the patient’s symptoms, this may include physical therapy, occupational therapy and/or speech/swallowing therapy.

Other treatments that some patients have found helpful in managing symptoms of ECD include:

  • Bisphosphonates
    Bisphosphonates are a class of drugs that prevent the loss of bone mass, used to treat osteoporosis and similar diseases. High-potency intravenous bisphosphonates have been shown to modify progression of skeletal metastasis in several forms of cancer. Bisphosphonates may contribute to the reduction of bone pain and to the management of the skeletal involvement in ECD.
  • Modafinil (trade name Provigil)
    Some patients have been treated with provigil to help relieve some of the fatigue/sleepiness symptoms they experience with ECD. Patients report varying levels of success regarding the relief provided. This medication will not treat the disease, but has simply been tried as a way to help patients cope with severe fatigue. For more information about modafinil, see
  • Long-Term Antibiotics
    Many ECD patients report an increased tendency to suffer from infections and have a more difficult time recovering from them. Some treatments can reduce a patient’s immune system, putting them at a higher risk for infection. Most patients deal with common infections such as sinus, upper respiratory, urinary tract, etc., but others may have to deal with more opportunistic infections that occur when the immune system is compromised. The following are various methods used by some doctors and patients to help keep infections under control.
    – Visit your local doctor as soon as you suspect an infection.
    – Some doctors arrange a standing order for antibiotics in the event their patient cannot see him/her in a timely manner. This is done on a case by case basis. Even when using this method, it is very important to see your doctor as soon as possible when you suspect an infection. Choosing the best antibiotic for a particular infection can be very important and this can only be done with proper testing. Many times an infection may be the result of a virus in which case an antibiotic will NOT help in fighting the infection at all.
    – Still other doctors place patients on long term courses of certain antibiotics as a prophylactic treatment to prevent an infection. This is done on a case-by-case basis and requires close monitoring by a doctor. There are differing opinions on the benefits versus risks of this approach to treating those with chronic infections.
    For more information regarding antibiotics see”

Additional Resources:

Last updated: May 30, 2017

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