Treatment & prognosis

Schnitzler syndrome

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Treatment & prognosis

Therapy

High-dose corticosteroids were in most cases able to decrease symptoms, but cannot be used for a long term due to the serious side effects. Cyclooxygenase inhibitors and alkylating agents were mostly ineffective, while colchicine has been reported to be highly effective in some, but ineffective in most of the cases. Plasmapheresis reduced malaise and skin symptoms only temporarily. Antihistamines, azathioprine and dapsone are ineffective.

However, the IL-1 receptor antagonist Anakinra caused complete remission in 7 out of 7 patients and so appears to be a promising treatment. Thalidomide was very effective in 3 out of 3 cases as well, but had to be stopped in 2 of them because polyneuropathies developed. Pefloxacine might also be an option, although the evidence is still scarce with a 2 out of 2 success rate.

Prognosis

Schnitzler syndrome is a chronic disease. An estimation of mortality rate does not seem to differ significantly from that of the general population. Still, the morbidity has got a profound impact on the patients’ lives. Also, long-term follow up is mandatory as a considerable percentage of patients will develop Waldenström’s macroglobulinaemia, a lymphoplasmacytic malignancy.

For references see literature page

Last update: June 30, 2007