Comment on: Schnitzlers syndrome—exacerbation after anti-TNF treatment
Division of General Internal Medicine, Department of Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
Correspondence to: A. Simon, MD, PhD, Division of General Internal Medicine, 484, Department of Internal Medicine, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands. E-mail: a.simon{at}aig.umcn.nl
SIR, In the June issue of this journal, Thonhofer et al. reported on treatment of a patient with Schnitzler syndrome [1]. We would like to comment briefly on their findings.
The observation that the patient deteriorated when started on treatment with tumour necrosis factor (TNF)-inhibitors, adalimumab as well as etanercept, is intriguing, also with regard to pathophysiology. However, the use of TNF-inhibitors in Schnitzler syndrome was already described in 2005 by Lin et al. [2], whose patient ameliorated when etanercept was given at a dose of 25 mg twice weekly.
Thonhofer et al. [1] subsequently started the patient on a combination therapy of anakinra, weekly doses of methotrexate and a tapering dose of steroids. The patient responded very well, and anakinra–methotrexate combination therapy was set forth in the key message as treatment of choice. The efficacy of this treatment regimen was also reported by Martinez-Taboada et al. [3] in 2005. Based on our experience and on data from the literature, the beneficial effect of this combination therapy is most probably solely due to inhibition of interleukin-1 (IL-1) by anakinra. Since our publication of the beneficial effect of monotherapy with anakinra in Schnitzler syndrome [4], we have seen this in at least eight more patients, either in our clinic, published [5, 6] or through personal communication (V. Akhras, London, UK). We feel that co-administration of methotrexate is not indicated, especially in view of its potential side-effects.
We have recently reviewed the cases of Schnitzler syndrome in the literature and concluded that few if any therapies other than the IL-1 receptor antagonist anakinra are able to induce complete remission in Schnitzler syndrome [7]. There is no need for co-administration of methotrexate. From the point of view of efficacy and toxicity, monotherapy with anakinra should be regarded as the treatment of choice in patients with Schnitzler syndrome.
Disclosure Statement: The authors had declared no conflict of interest.
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- Thonhofer R, Uitz E, Graninger W. Schnitzler's syndrome–exacerbation after anti-TNF treatment. Rheumatology (2007) 46:1041–2.
[Free Full Text] - Lin RY, Jagannath S. Treatment of Schnitzler's syndrome with colchicine: a case report. J Asthma, Allergy Immunol (2005) 4(1).
- Martinez-Taboada VM, Fontalba A, Blanco R, Fernandez-Luna JL. Successful treatment of refractory Schnitzler syndrome with anakinra: comment on the article by Hawkins et al. Arthritis Rheum (2005) 52:2226–7.[CrossRef][Web of Science][Medline]
- de Koning HD, Bodar EJ, Simon A, van der Hilst JC, Netea MG, van der Meer JW. Beneficial response to anakinra and thalidomide in Schnitzler's syndrome. Ann Rheum Dis (2006) 65:542–4.
[Abstract/Free Full Text] - Eiling E, Moller M, Kreiselmaier I, Brasch J, Schwarz T. Schnitzler syndrome: treatment failure to rituximab but response to anakinra. J Am Acad Dermatol (2007) 57:361–4. doi:10.1016/j.jaad.2007.03.036.[CrossRef][Web of Science][Medline]
- Schneider SW, Gaubitz M, Luger TA, Bonsmann G. Prompt response of refractory Schnitzler syndrome to treatment with anakinra. J Am Acad Dermatol (2007) 56(Suppl 5):S120–2.[CrossRef][Web of Science][Medline]
- de Koning HD, Bodar EJ, van der Meer JW, Simon A, Schnitzler Syndrome Study Group. Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum (2007) doi:10.1016/j.semarthrit.2007.04.001.
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  R. Thonhofer, E. Uitz, and W. Graninger Comment on: Schnitzler's syndrome exacerbation after anti-TNF treatment: reply Rheumatology, November 1, 2007; 46(11): 1741 - 1741. [Full Text] [PDF]
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