A Pilot Study of XOMA 052 in Familial Cold Autoinflammatory Syndrome / Muckle-Wells Syndrome and Behcet's Disease

* INCLUSION CRITERIA: FOR FCAS / MWS: 1. Male or female subjects with inflammatory disease greater than or equal to 18 years of age. 2. Participation in NIH study #03-AR-0173 ( Studies of the Natural History, Pathogenesis, and Outcome of Autoinflammatory Diseases ) 3. CIAS1 mutation positivity or FCAS / MWS based on clinical grounds with a history of a complete response to IL-1 blocking medications. 4. Subjects presenting with active FCAS / MWS based on clinical signs/symptoms in addition to elevated acute phase reactants (CRP, SAA or ESR). To meet established clinical criteria for active disease, patients must have recurrent intermittent episodes of fever and rash, an age of onset \< 6 months of age, duration of most attacks \< 24 hours, and the presence of conjunctivitis with attacks. Active disease will be defined as either the presence of classical features or a history of such features that became quiescent in the setting of therapy with anakinra or rilonacept. However, before a patient who has quiescent disease and is currently taking anakinra or rilonacept can receive study drug, he/she must fulfill criteria for active disease after anakinra or rilonacept has been discontinued. Subjects must be greater than 48 hours from their last dose of anakinra (half-life 4-6 hours) and 14 days from their last dose of rilonacept (half-life 7.5 days) before beginning XOMA 052 therapy, and will not take anakinra or rilonacept for the remainder of their enrollment in the study. 5. Stable dose of steroids, NSAIDs, DMARDs, or colchicine for four weeks prior to enrollment visit. 6. Females of childbearing potential (young women who have had at least one menstrual period regardless of age) must have a negative urine pregnancy test at screening and a negative serum pregnancy test at baseline prior to performance of any radiologic procedure or administration of study medication. 7. Women of childbearing age and men able to father a child, who are sexually active, who agree to use a form of effective birth control, including abstinence. 8. Either (1) a negative PPD test using 5 T.U. intradermal testing per CDC guidelines and no evidence of active TB by history on chest X-ray at the time of enrollment or (2) a positive PPD with no evidence of active TB on chest X-ray at the time of enrollment and either past or present treatment with adequate therapy for at least one month prior to first dose of study medication. Full prophylaxis regimens will be completed. Subjects who have been BCG-vaccinated will also be skin-tested. 9. Able to understand, and complete study-related questionnaires. 10. Able and willing to give informed consent and abide with the study procedures. FOR BD: 1. Male or female subjects with BD associated inflammatory disease greater than or equal to 18 years of age. 2. Participation in NIH study #03-AR-0173 ( Studies of the Natural History, Pathogenesis, and Outcome of Autoinflammatory Diseases ) 3. Diagnosis of Behcet's disease as determined by the International Study Group Criteria or by complete Japanese Criteria 4. Active mucocutaneous disease as defined by at least one oral or genital ulcer within the past month. 5. Stable dose of steroids, NSAIDs, DMARDs, or colchicine for four weeks prior to enrollment visit. 6. For patients with ocular disease, no active intermediate or posterior disease at enrolment but history of an ocular flare (greater than or equal to 3 in the last 6 months) in the presence of any systemic anti-inflammatory therapy such as prednisone, azathioprine, Mycophenolate, methotrexate, cyclosporine, a TNF inhibitor, or a combination of these medications. Patients must have developed active disease in the presence of at treatment with at least one of the following medications for at least six months: azathioprine, cyclosporine, or a TNF inhibitor. 7. Females of childbearing potential (young women who have had at least one menstrual period regardless of age) must have a negative urine pregnancy test at screening and a negative serum pregnancy test at baseline prior to performance of any radiologic procedure or administration of study medication. Female patients will be screened for pregnancy at all NIH visits. 8. Women of childbearing age and men able to father a child, who are sexually active, who agree to use a form of effective birth control, including abstinence. 9. Either (1) a negative PPD test using 5 T.U. intradermal testing per CDC guidelines and no evidence of active TB on chest X-ray at the time of enrollment or (2) a positive PPD with no evidence of active TB by history or on chest X-ray at the time of enrollment and either past or present treatment with adequate therapy for at least one month prior to first dose of study medication. Full prophylaxis regimens will be completed. Subjects who have been BCG-vaccinated will also be skin-tested. 10. Able to understand, and complete study-related questionnaires. 11. Able and willing to give informed consent and abide with the study procedures. EXCLUSION CRITERIA: For both study populations: 1. Treatment with a live virus vaccine during 3 months prior to baseline visit. No live vaccines will be allowed throughout the course of this study. 2. Patients with ocular disease who received local treatments other than eye drops (i.e. periocular or intraocular steroids, implants or other antinflammatory agents within 4 weeks prior to enrollment) 3. Current treatment with TNF inhibitors or discontinuation of TNF inhibitors within 8 weeks. 4. Presence of active infections or a history of pulmonary TB infection with or without documented adequate therapy. 5. Chest x-ray read by a radiologist with pleural scarring and/or calcified granuloma consistent with prior TB. 6. Positive test for or prior history of HIV, Hepatitis B or C. 7. History or concomitant diagnosis of congestive heart failure. 8. History of malignancy. Subjects deemed cured of superficial malignancies such as cutaneous basal or squamous cell carcinomas, or in situ cervical cancer may be enrolled. 9. Known hypersensitivity to CHO cell derived biologicals or any components of XOMA 052. 10. Presence of any additional rheumatic disease or significant systemic disease. For example, major chronic infectious/ inflammatory/ immunologic disease (such as inflammatory bowel disease, psoriatic arthritis, spondyloarthropathy, SLE in addition to autoinflammatory disease). 11. Presence of any of the following laboratory abnormalities at enrollment visit: creatinine\> 1.5 times the ULN, WBC\< 3.6x10(9)/mm(3); platelet count \< 75,000 mm(3); ALT or AST \> 2.0 times the ULN 12. Lactating females or pregnant females. 13. Subjects with asthma not adequately controlled on current inhaled therapy for at least four weeks. 14. Enrollment in any other investigational treatment study or use of an investigational agent, or has not yet completed at least 4 weeks or 5 half-lives, whichever is longer, since ending another investigational device or drug trial. 15. Subjects for whom there is concern about compliance with the protocol procedures. 16. Presence of other severe acute or chronic medical or psychiatric condition, or significant laboratory abnormality requiring further investigation that may cause undue risk for the subject's safety, inhibit protocol participation, or interfere with interpretation of study results, and in the judgment of the investigator would make the subject inappropriate for entry into this study. 17. Treatment within the past 12 months with canakinumab 18. Active neurologic disease which would require cyclophosphamide treatment. Active neurologic disease is defined as either new evidence of parenchymal (meningoencephalitis) or non-parenchymal (vascular complications including thrombosis) disease.