[Interleukin-1 receptor blockade with anakinra provided cessation of fatigue, reduction in inflammation markers and regression of retroperitoneal fibrosis in a patient with Erdheim-Chester disease - case study and a review of literature].

[erdheim-chester disease]

We describe a case of an Erdheim-Chester disease patient . First line chemotherapy treatment with 2 -chlorodeoxyadenosine did not reduce fluorodeoxyglucose accumulation in pathological lesions . The patient had continuously increased CRP values of 17 -20 mg /l . The disease continued to cause subfebrile temperatures and significant fatigue that made the patient to spend most of the daytime in bed . To manage the permanently increased inflammation markers , we decided to start treatment with anakinra , successfully used in some other autoinflammatory diseases (e .g . Schnitzler syndrome ). We have now been able to evaluate the first 6 months of treatment . Daily subcutaneous administration of anakinra (KineretTM 100 mg daily ) led to normalization of CRP values , cessation of subfebrile temperatures and , importantly , significant reduction of fatigue . Time periods the patient was able to spend out of the bed increased significantly . Consequent to the reduced fatigue , the patient was able to perform basic household tasks he was unable to undertake without treatment . After 3 months of treatment , fatigue of the same intensity returned following a short interruption of therapy . The CRP values went up again to 12 mg /l . CRP value returned back to norm and fatigue ceased after re -initiation of daily Kineret injections . Objective treatment response was assessed by measuring the degree of fluorodeoxyglucose accumulation in pathological bone lesions . PET-CT was performed before and 3 and 6 months after anakinra initiation . Intensity of accumulation did not change significantly after the first 3 months of therapy but decreased after 6 month therapy . Follow up CT of abdominal cavity was performed at the end of the 6 th month of treatment . Presented CT images from before and 6 months after the treatment evidence an obvious reduction in fibroid changes in the retroperitoneum . Daily administration of anakinra to a patient with active Erdheim-Chester disease significantly reduced intensity of fatigue and improved quality of life , led to a reduction in inflammatory markers and regression in retroperitoneal fibrotization .