Schnitzler syndrome responding to interleukin-1 antagonist anakinra

Since 2005, an 82-year-old female patient suffered from relapsing, generalized urticarial rash with relapsing fever episodes, arthralgias and burning tibial pain sensations. She unintentionally lost about 10 kg of body weight within 9 months. Systemic treatment with desloratadine, ranitidine, prednisolone and later, ciclosporin A was started. All these efforts could not control the symptoms but caused adverse effects including glaucoma and hypertension. Several years later a therapy with interleukin-1 inhibitor anakinra 100 mg s.c. once daily was started. The patient became symptom-free within 24 h after the first injection. With a follow up of three years the patient remained completely symptom-free with no fever or burning sensations. Later on, the dosage was reduced to 100 mg s.c. every other day. Anakinra reduces pro-inflammatory cytokine release due to lipopolysaccharide stimulation. In a long-term use the drug does not lose effectiveness.