Anakinra to Treat Patients With Neonatal Onset Multisystem Inflammatory Disease

-INCLUSION CRITERIA: There is no age limitation. Patients fulfill at least 2 of the following 3 clinical manifestations: Typical NOMID rash CNS involvement (papilledema, CSF pleocytosis, sensorineural hearing loss) Typical arthropathic changes on radiograph (epiphyseal and/or patellar overgrowth. Onset of manifestations of NOMID/CINCA at less than or equal to 6 months of age. Stable dose of steroids, NSAIDs, DMARDs for 4 weeks prior to enrollment visit. Washout period for biologics: 6 half-lives before anakinra administration for all drugs with anti TNF properties. For etanercept (6 half-lives=24 days) this calculates to drug discontinuation 3 days before enrollment into the observation period, for infliximab and adalimumab (6 half-lives=48 days) drug will be discontinued 27 before the observation period, and for thalidomide (6 half-lives=3 days) drug will be discontinued for 3 days prior to anakinra administration. Patient's or legal guardian's ability and willingness to give informed consent. 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 baseline prior to performance of any radiologic procedure or administration of study medication. Women of childbearing age and men able to father a child, who are sexually active, will be asked to use a form of effective birth control, including abstinence. Negative PPD test using 5 T.U. intradermal testing per CDC guidelines with exception of inclusion criteria #9 below. Patients with latent TB (positive PPD test) must have adequate therapy for TB initiated prior to first dose of study medication as recommended in published guidelines. EXCLUSION CRITERIA: Having received live virus vaccine during 3 months prior to baseline visit (1st visit to NIH). Patients with active infections or a history of pulmonary TB infection with or without documented adequate therapy, Patients with current active TB, or recent close exposure to an individual with active TB are excluded from the study. Positive testing for HIV, Hepatitis B or C known or documented at screening, enrollment or baseline visit. Have a history of or concomitant diagnosis of congestive heart failure. History of malignancy. Recent use of IL-1 antagonist within the last three months or prior use of anti CD4 antibody. Known hypersensitivity to E. coli derived products or any components of anakinra. Presence of any other rheumatic disease or major chronic infectious/inflammatory/immunologic disease (e.g. inflammatory bowel disease, psoriatic arthritis, spondyloarthropathy, SLE in addition to NOMID/CINCA). Presence of the following at enrollment visit: ALT or AST greater than 2.0 x upper limit of normal (ULN) of the local laboratories values, creatinine greater than 1.5 xULN, WBC less than 3.6x10(9)/l; platelet count less than 150,000 mm(3). Enrollment in any other investigational clinical study or receiving an investigational agent, or has not yet completed at least 4 weeks since ending another investigational device or drug trial. Subjects for whom there is concern about compliance with the protocol procedures by subject and/or parent/s and legally acceptable representative/s. Lactating females or pregnant females. Patients with asthma will only be included after evaluation by a pulmonary and infectious disease consultation.