Successful Treatment of Schnitzler Syndrome with Tocilizumab

Abstract

Introduction: Schnitzler syndrome (SchS) is an uncommon, autoinflammatory disorder, which is hard to be controlled by conventional therapy. Recently, anti-interleukin (IL)-1 agents has been reported as the most effective therapeutic regimen. Here, we reported that a patient with SchS has achieved a rapid remission after using tocilizumab, an anti-IL-6 receptor antagonist. Case presentation: A 56-year-old man presented with recurrent generalized urticarial rash and knee soreness, and had irregular febrile episode during these two years, accompanying with or without the skin eruptions. Main laboratory evaluations were notable for inflammatory indicators. Skin biopsy revealed that numerous neutrophils in the superficial dermis and around the vascular vessels. Myeloid inverse conversion of his knee joints was discovered. As soon as the diagnosis of SchS was made, tocilizumab intravenously 400 mg was given, and the patient showed no relapse in 30 weeks follow-up. Discussion: Cytokine activation and elevated inflammasome is involved in the disorder of Schs, in which IL-1 and IL-6 have been considered as important factors. To diagnose Schs, it needs to be distinguished from some autoimmune diseases associated with neutrophilic urticarial dermatosis such as cryopyrin-associated periodic syndromes and adult-onset still disease. Although IL-1 blockers are widely used and get curative effect recent years, patients failed to response to IL-1 blockers may benefit from anti–IL-6 treatment. Conclusion: Anti-IL-6 therapy may be a reliable candidate for Schs from our experience.