PMID: 22559807May 9, 2012Paper

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

Vnitr̆ní lékar̆ství
Z AdamJ Mayer

Abstract

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 nor...Continue Reading

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