Ilaris® in Urticarial Vasculitis - Investigation of Treatment Responses

Inclusion Criteria:

• Adults (18 years or older)

• UV diagnosis based on diagnostic criteria defined in Appendix 3

• Active UV, refractory to treatment with antihistamines, NSAIDS or colchicine,hydroxychloroquine or dapsone

• Patients who have a mean symptom score (UVAS) of at least 5 during baseline

• If necessary, concurrent/ongoing treatment with a stable dose of systemiccorticosteroids not greater than 10mg/d for 14 days prior to screening

• If necessary, concurrent/ongoing treatment with a stable dose of antihistamines andNSAIDs for 7 days prior to screening

• Informed consent signed and dated

• Able to read, understand and willing to sign the informed consent form and abide withstudy procedures

• Willing, committed and able to return for all clinic visits and complete allstudy-related procedures, including willingness to have SC injections administered bya qualified person

• In females of childbearing potential: Negative pregnancy test; males and femaleswilling to use highly effective contraception (Pearl-Index < 1). A woman will beconsidered not of childbearing potential if she is post-menopausal for greater thantwo years or surgically sterile (bilateral tubal ligation, bilateral oophorectomy orhysterectomy)

• In men: Willingness to utilize highly effective contraception and to not have theirpartner become pregnant during the full course of the study. Adequate contraceptivemeasures include oral contraceptives (stable use for two or more cycles prior toscreening); bilateral tubal ligation; vasectomy; condom or diaphragm plus eithercontraceptive sponge, foam or jelly

• 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 orsymptoms suggestive of active TB, no recent close contacts with a person with activeTB, 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 appropriatemeasurements according to standard of care).

• No participation in other clinical trials 4 weeks before and after participation inthis study

Exclusion Criteria:

• Concurrent/ongoing treatment with Anakinra (Kineret®) or recent treatment within 3days prior to screening

• Concurrent/ongoing treatment with other biologics or recent treatment (less than 5half lives)

• Concurrent/ongoing treatment with

• oral corticosteroids greater than 10 mg/d within 2 weeks prior to screening

• parenteral corticosteroids within 4 weeks prior to screening

• 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

• Evidence of recurrent or latent systemic infection such as tuberculosis

• Significant medical condition rendering the patient immunocompromised or not suitablefor a clinical trial

• Treatment with a live (attenuated) virus vaccine during three months prior to Baselinevisit (Visit 2)

• An abnormal chest radiograph consistent with clinical signs of prior or presenttuberculosis infection whether or not previously treated with anti-tuberculosis agents

• A history of listeriosis, active tuberculosis, persistent chronic or activeinfection(s) requiring treatment with parenteral antibiotics, parenteral antivirals,or parenteral antifungals within four weeks prior to screening

• Significant concomitant illness such as, but not limited to, cardiac, renal,neurological, endocrinological, metabolic, or lymphatic disease that would adverselyaffect the subject's participation or evaluation in this study

• Active systemic inflammatory condition other than UV including, but not limited to,rheumatoid arthritis, systemic lupus erythematosus or Sjögren's Syndrome

• History of fibromyalgia or chronic fatigue syndrome

• Evidence of current HIV, active hepatitis B, or hepatitis C infection by clinical orserological history

• History of malignancies within five years prior to screening other than a successfullytreated non-metastatic cutaneous, basal, or squamous cell carcinoma and/or in situcervical cancer

• History of a demyelinating disease or multiple sclerosis

• Severe respiratory disease, including, but not limited to severe bronchiectasis,chronic obstructive pulmonary disease, bullous lung disease, uncontrolled asthma, orpulmonary fibrosis

• Presence of any of the following laboratory abnormalities at enrollment visit:creatinine >1.5 x Upper Limit of Normal (ULN), WBC <3000/µl; platelet count <100000/µl ; ALT or AST >3.0 x ULN

• Lactating females or pregnant females

• Males not willing to use highly effective contraception

• Enrollment in another investigational treatment or device study or use of aninvestigational 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 withparticipation in the study or place the subject at risk

• History of substance abuse (drug or alcohol) or any other factor (e.g., seriouspsychiatric condition) that could limit the subject's ability to comply with studyprocedures

• Subjects who are detained officially or legally to an official institute