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 6th 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.