Rohan Crouch, MB BS. Victoria Akhras, MB BS. Robert Sarkany, FRCP.
Schnitzler's syndrome: Successful treatment with anakinra
Article first published online: 31 JUL 2007
Australasian Journal of Dermatology
Volume 48, Issue 3, pages 178–181, August 2007
How to Cite
Crouch, R., Akhras, V. and Sarkany, R. (2007), Schnitzler's syndrome: Successful treatment with anakinra. Australasian Journal of Dermatology, 48: 178–181. doi: 10.1111/j.1440-0960.2007.00375.x
- Issue published online: 31 JUL 2007
- Article first published online: 31 JUL 2007
- Submitted 19 January 2007; accepted 29 March 2007.
- IgM paraproteinaemia;
- interleukin-1 antagonist;
- monoclonal IgM gammopathy;
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.