Schnitzler's syndrome presenting as fever of unknown origin (FUO) The role of cytokines in its systemic features

Abstract

A patient with Schnitzler's syndrome is described presenting with fever of unknown origin. Although he had all characteristic features of the syndrome (urticarial vasculitis, hyperostosis, lymphadenopathy, fever and serum IgM monoclonal component), it was recognized very late in the diagnostic process. Cytokines were measured to get more insight into the role of cytokines in this syndrome, but only interleukin-6 was elevated. It is important for internists and rheumatologist to recognize this entity in order to prevent unnecessary diagnostic procedures. © 1997 Elsevier Science B.Y.

Complete remission in 3 of 3 anti-IL-6–treated patients with Schnitzler syndrome

“…This is supported by previous reports demonstrating increased IL-6 serum levels in SchS. [8][9][10] An increase in IL-6 levels after anti-IL-6 treatment has been described before. It was suggested that tocilizumab competes with IL-6 for binding to IL-6 receptors, thus leading to elevated IL-6 levels in the serum.…”

Beneficial response to anakinra and thalidomide in Schnitzler's syndrome

“…However, these anti-IL1a antibodies could not be detected in other patients, nor were the serum concentrations of tumour necrosis factor a, IL1b or IL1Ra increased. [13][14][15] In conclusion, anakinra (IL1Ra) proved to be very effective in our three patients with Schnitzler's syndrome. This treatment is preferable to thalidomide, which induced a complete remission in one of our patients, as it has fewer side effects.…”

Schnitzler?s syndrome with neurological findings

“…Bone marrow biopsy, white blood cell counts, skeletal radiography, abdominal ultrasound and draining lymph node ultrasound excluded the presence of a lymphoma. For an observation period of more than ten years complement factors 3 and 4 were within normal limits, excluding a hypocomplemental urticarial vasculitis [5]. The consistently normal blood IgD level effectively ruled out hyper-IgD syndrome.…”