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Sarcoidosis

Cytoxan

06/25/2010

Question:

I have been diagnosed with bone marrow sarcoidosis via biopsy to three different marrow sites. I have tried Prednisone at 1.5mg/kg, plaquenil, remicaide, methotrexate oral and SQ. Now, I have multiple biopsy proving sarcoid lesions to skin. My liver biopsy was positive for sarcoidosis and fibrosis. Is cytoxan a reasonable next step? If so, what dosage?

Answer:

Dear Sir/Madam- There are a number of options available for treating sarcoidosis that is refractory to conventional therapies.
 
In many cases, treatment-resistant disease does not respond to a single agent, and combination therapy should be tried. The medications you have previously received (hydroxychloroquine, prednisone, methotrexate, infliximab) are generally less toxic and are shown to be effective for the treatment of sarcoidosis. 

If these drugs fail, a limited course of cytoxan (cyclophosphamide) is a reasonable next step. Cytoxan is reported to reduce the dependence on corticosteroids (e.g., prednisone) in patients with neurological manifestations.

The study I am referring to (Doty et al. Chest 2003) started at a dose of 500 mg every 3 weeks and slowly increased to 1000 mg every 3 weeks for a total duration of anywhere between 6 months and 15 months, depending on the response. Others have reported good clinical responses to cytoxan in otherwise unresponsive sarcoidosis. 

The long-term risk of cytoxan, which is related to the duration of therapy, is bladder cancer. The short-term risks relate to suppression of the immune system (i.e., severe infections). Thus, it is important to monitor for these potential complications. 

Naturally, a very experienced clinician should be involved in decisions relating to the management of treatment-resistant sarcoidosis, such as yours. 

For more information:

Go to the Sarcoidosis health topic, where you can:

Response by:

Elliott D Crouser, MD Elliott D Crouser, MD
Associate Professor of Pulmonary, Allergy, Critical Care & Sleep Medicine
College of Medicine
The Ohio State University