Tocilizumab in Patients With Schnitzler's Syndrome

Inclusion Criteria: * Adults (18 years or older) * SchS diagnosis based on Strasbourg clinical criteria * Active SchS, refractory to treatment with antihistamines, NSAIDS or colchicine, hydroxychloroquine or dapsone * Patients who have a symptom score (PGA) of at least 8 (0-20) at baseline * If necessary, concurrent/ongoing treatment with a stable dose of systemic corticosteroids not greater than 10mg/d for 14 days prior to screening * If necessary, concurrent/ongoing treatment with a stable dose of antihistamines and NSAIDs for 7 days prior to screening * Able to read, understand and willing to sign the informed consent form and abide with study procedures * Willing, committed and able to return for all clinic visits and complete all study-related procedures, including willingness to have SC injections administered by a qualified person * In females of childbearing potential: Negative pregnancy test within 28 days of randomization; males and females willing to use highly effective contraception (Pearl-Index \< 1) during study treatment and for a minimum of 3 months after last dose of TCZ. Pregnancies occurring up to 90 days after the completion of the study medication must be reported to the investigator. A woman will be considered not of childbearing potential if she is post-menopausal for greater than two years or surgically sterile (bilateral tubal ligation, bilateral oophorectomy or hysterectomy) * Subjects are considered eligible, if they meet the following tuberculosis (TB) screening criteria: no history of latent or active TB prior to screening, no signs or symptoms suggestive of active TB, no recent close contacts with a person with active TB, and negative QuantiFERON-TB test at screening (if QuantiFERON-TB test is positive, the patient can only be included if active TB is ruled out with appropriate measurements according to standard of care, e.g. the patient is pre-treated with isoniazide for 4 weeks). * No participation in other clinical trials 4 weeks before and after participation in this study Exclusion criteria: * Major surgery (including joint surgery) within 8 weeks prior to screening or planned major surgery within 6 months following randomization. * Concurrent/ongoing treatment with biologics or recent treatment (less than 5 half lives) * With Anakinra within 7 days prior to screening, with canakinumab within 100 days prior to screening * with oral/parental corticosteriods greater than 10 mg/d within 2 weeks prior to screening * with Cyclosporin A Methotrexate, Dapsone, Chloroquine, Hydroxychloroquine, Azathioprine, Cyclophosphamide within 4 weeks prior to screening * other immunosuppressives within 4 weeks or 5 half lives prior to screening, whichever is longer * Previous treatment within six months of randomization with any cell-depleting therapies, including investigational agents or approved therapies, some examples include: CAMPATH, anti-CD4, anti-CD5, anti-CD3, anti-CD19 and anti-CD20. * Treatment with intravenous gamma globulin, plasmapheresis or Prosorba column within 6 months of baseline. * Treatment with a live (attenuated) virus vaccine within 4 weeks prior to Baseline visit * Significant medical condition rendering the patient immunocompromised or not suitable for a clinical trial * Any previous treatment with alkylating agents such as chlorambucil, or with total lymphoid irradiation. * History of severe allergic or anaphylactic reactions to human, humanized or murine monoclonal antibodies. * An abnormal chest radiograph consistent with clinical signs of prior or present tuberculosis infection whether or not previously treated with anti-tuberculosis agents * Significant concomitant illness such as, but not limited to, cardiac, renal, neurological, endocrinological, metabolic, or lymphatic disease that would adversely affect the subject's participation or evaluation in this study * Evidence of current HIV, Hepatitis B, or Hepatitis C infection by clinical or serological history * Presence of any of the following laboratory abnormalities at enrollment visit: serum creatinine \> 1.6 mg/dL (141 µmol/L) in female patients and \> 1.9 mg/dL (168 µmol/L) in male patients, WBC \<3,000/µl; platelet count \<100000/µl ; ALT or AST \>2 x ULN or total bilirubin \>ULN, Hemoglobin \<8.0 g/dL, neutrophil count \<2,000 cells/µl or lymphocyte count \<500/ µl * Evidence of active, recurrent or latent systemic infection * Active systemic inflammatory condition other than SchS including, but not limited to, rheumatoid arthritis * History of malignancies within five years prior to screening other than a successfully treated non-metastatic cutaneous, basal, or squamous cell carcinoma and/or in situ cervical cancer * Lactating females or pregnant females * Enrollment in another investigational treatment or device study or use of an investigational agent, or less than 4 weeks or 5 half-lives, whichever is longer, since end of another investigational device or drug trial * Subjects for whom there is concern about compliance with the protocol procedures * Any medical condition which, in the opinion of the Investigator, would interfere with participation in the study or place the subject at risk * History of substance abuse (drug or alcohol) or any other factor (e.g., serious psychiatric condition) that could limit the subject's ability to comply with study procedures. * Patients with known hypersensitivity to tocilizumab