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Schnitzler's syndrome: Successful treatment with anakinra
Author(s) -
Crouch Rohan,
Akhras Victoria,
Sarkany Robert
Publication year - 2007
Publication title -
australasian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.67
H-Index - 53
eISSN - 1440-0960
pISSN - 0004-8380
DOI - 10.1111/j.1440-0960.2007.00375.x
Subject(s) - medicine , anakinra , prednisone , erythrocyte sedimentation rate , gastroenterology , malaise , fibrinoid necrosis , rash , surgery , dermatology , pathology , vasculitis , disease
SUMMARY A 44‐year‐old man presented with a 2‐year history of an intermittent urticarial rash, malaise, weight loss, night sweats, headaches and bone pains. Initial investigations indicated an elevated erythrocyte sedimentation rate, white cell count and a monoclonal immunoglobulin‐M paraprotein. Histological examination revealed a perivascular mixed inflammatory infiltrate with leukocytoclasis, nuclear dust without fibrinoid necrosis and extravasated red blood cells. A diagnosis of Schnitzler's syndrome was made. Over an 8‐year period, the patient was treated with continuous oral prednisone (minimum dose 20 mg/day) combined with multiple systemic agents. He was commenced on anakinra, a recombinant form of human interleukin‐1 receptor antagonist, at a dose of 100 mg injected subcutaneously daily. On review 1 week later, the patient's systemic symptoms had resolved, and his previously elevated white cell count and inflammatory markers had normalized. The use of anakinra in our patient resulted in resolution of symptoms and has enabled cessation of oral prednisone. Our patient remains symptom free on anakinra after 14 months of follow up.