{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T23:50:32Z","timestamp":1788306632989,"version":"build-2803163510"},"reference-count":142,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2012,1,30]],"date-time":"2012-01-30T00:00:00Z","timestamp":1327881600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,1,30]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>OTHER THEMES PUBLISHED IN THIS IMMUNOLOGY IN THE CLINIC REVIEW SERIES<\/jats:p>\n               <jats:p>Allergy, Host Responses, Cancer, Type 1 diabetes and viruses, Metabolic diseases.<\/jats:p>\n               <jats:p>The disease-based discovery of the molecular basis for autoinflammatory diseases has led not only to a rapidly growing number of clinically and genetically identifiable disorders, but has unmantled key inflammatory pathways such as the potent role of the alarm cytokine interleukin (IL)-1 in human disease. Following its initial failures in the treatment of sepsis and the moderate success in the treatment of rheumatoid arthritis, IL-1 blocking therapies had a renaissance in the treatment of a number of autoinflammatory conditions, and IL-1 blocking therapies have been Food and Drug Administration (FDA)-approved for the treatment of the autoinflammatory conditions: cryopyrin-associated periodic syndromes (CAPS). CAPS and deficiency of the IL-1 receptor antagonist (DIRA), both genetic conditions with molecular defects in the IL-1 pathway, have provided a pathogenic rationale to IL-1 blocking therapies, and the impressive clinical results confirmed the pivotal role of IL-1 in human disease. Furthermore, IL-1 blocking strategies have shown clinical benefit in a number of other genetically defined autoinflammatory conditions, and diseases with clinical similarities to the monogenic disorders and not yet identified genetic causes. The discovery that IL-1 is not only triggered by infectious danger signals but also by danger signals released from metabolically \u2018stressed\u2019 or even dying cells has extended the concept of autoinflammation to disorders such as gout, and those that were previously not considered inflammatory, such as type 2 diabetes, coronary artery disease, obesity and some degenerative diseases, and provided the conceptual framework to target IL-1 in these diseases. Despite the tremendous success of IL-1 blocking therapy, the use of these agents in a wider spectrum of autoinflammatory conditions has uncovered disease subsets that are not responsive to IL-1 blockade, including the recently discovered proteasome-associated autoinflammatory syndromes such as chronic atypical neutrophilic dermatitis with lipodystrophy and elevated temperatures (CANDLE), Japanese autoinflammatory syndrome with lipodystrophy (JASL), Nakajo\u2013Nishimura syndrome (NNS) and joint contractures, muscle atrophy, panniculitis induced lipodystrophy (JMP), and urge the continued quest to characterize additional dysregulated innate immune pathways that cause autoinflammatory conditions.<\/jats:p>","DOI":"10.1111\/j.1365-2249.2011.04533.x","type":"journal-article","created":{"date-parts":[[2011,11,26]],"date-time":"2011-11-26T00:09:51Z","timestamp":1322266191000},"page":"391-404","source":"Crossref","is-referenced-by-count":122,"title":["Immunology in clinic review series; focus on autoinflammatory diseases: update on monogenic autoinflammatory diseases: the role of interleukin (IL)-1 and an emerging role for cytokines beyond IL-1"],"prefix":"10.1093","volume":"167","author":[{"given":"R","family":"Goldbach-Mansky","sequence":"first","affiliation":[{"name":"Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2012,1,30]]},"reference":[{"key":"2021122708460802300_b1","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1016\/S0092-8674(00)80539-5","article-title":"Ancient missense mutations in a new member of the RoRet gene family are likely to cause familial Mediterranean fever","volume":"90","author":"International FMF Consortium","year":"1997","journal-title":"Cell"},{"key":"2021122708460802300_b2","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1038\/ng0997-25","article-title":"A candidate gene for familial Mediterranean fever","volume":"17","author":"French FMF Consortium","year":"1997","journal-title":"Nat Genet"},{"key":"2021122708460802300_b3","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/S0092-8674(00)80721-7","article-title":"Germline mutations in the extracellular domains of the 55 kDa TNF receptor, TNFR1, define a family of dominantly inherited autoinflammatory syndromes","volume":"97","author":"McDermott","year":"1999","journal-title":"Cell"},{"key":"2021122708460802300_b4","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1038\/ng756","article-title":"Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle\u2013Wells syndrome","volume":"29","author":"Hoffman","year":"2001","journal-title":"Nat Genet"},{"key":"2021122708460802300_b5","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1086\/341357","article-title":"Chronic infantile neurological cutaneous and articular syndrome is caused by mutations in CIAS1, a gene highly expressed in polymorphonuclear cells and chondrocytes","volume":"71","author":"Feldmann","year":"2002","journal-title":"Am J Hum Genet"},{"key":"2021122708460802300_b6","doi-asserted-by":"crossref","first-page":"3340","DOI":"10.1002\/art.10688","article-title":"De novo CIAS1 mutations, cytokine activation, and evidence for genetic heterogeneity in patients with neonatal-onset multisystem inflammatory disease (NOMID): a new member of the expanding family of pyrin-associated autoinflammatory diseases","volume":"46","author":"Aksentijevich","year":"2002","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b7","doi-asserted-by":"crossref","first-page":"29874","DOI":"10.1074\/jbc.M203915200","article-title":"PYPAF7, a novel PYRIN-containing Apaf1-like protein that regulates activation of NF-kappa B and caspase-1-dependent cytokine processing","volume":"277","author":"Wang","year":"2002","journal-title":"J Biol Chem"},{"key":"2021122708460802300_b8","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1016\/S1097-2765(02)00599-3","article-title":"The inflammasome: a molecular platform triggering activation of inflammatory caspases and processing of proIL-beta","volume":"10","author":"Martinon","year":"2002","journal-title":"Mol Cell"},{"key":"2021122708460802300_b9","doi-asserted-by":"crossref","first-page":"2817","DOI":"10.1002\/art.22841","article-title":"Mutations in cryopyrin: bypassing roadblocks in the caspase 1 inflammasome for interleukin-1beta secretion and disease activity","volume":"56","author":"Dinarello","year":"2007","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b10","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1126\/science.1071059","article-title":"The danger model: a renewed sense of self","volume":"296","author":"Matzinger","year":"2002","journal-title":"Science"},{"key":"2021122708460802300_b11","doi-asserted-by":"crossref","first-page":"2426","DOI":"10.1056\/NEJMoa0807865","article-title":"An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist","volume":"360","author":"Aksentijevich","year":"2009","journal-title":"N Engl J Med"},{"key":"2021122708460802300_b12","doi-asserted-by":"crossref","first-page":"2438","DOI":"10.1056\/NEJMoa0809568","article-title":"An autoinflammatory disease due to homozygous deletion of the IL1RN locus","volume":"360","author":"Reddy","year":"2009","journal-title":"N Engl J Med"},{"key":"2021122708460802300_b13","doi-asserted-by":"crossref","first-page":"1614","DOI":"10.1073\/pnas.0708616105","article-title":"Mutations in NALP12 cause hereditary periodic fever syndromes","volume":"105","author":"Jeru","year":"2008","journal-title":"Proc Natl Acad Sci USA"},{"key":"2021122708460802300_b14","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1038\/9691","article-title":"Mutations in MVK, encoding mevalonate kinase, cause hyperimmunoglobulinaemia D and periodic fever syndrome","volume":"22","author":"Houten","year":"1999","journal-title":"Nat Genet"},{"key":"2021122708460802300_b15","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1038\/9696","article-title":"Mutations in the gene encoding mevalonate kinase cause hyper-IgD and periodic fever syndrome. International Hyper-IgD Study Group","volume":"22","author":"Drenth","year":"1999","journal-title":"Nat Genet"},{"key":"2021122708460802300_b16","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1136\/jmg.2005.030759","article-title":"Homozygous mutations in LPIN2 are responsible for the syndrome of chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia (Majeed syndrome)","volume":"42","author":"Ferguson","year":"2005","journal-title":"J Med Genet"},{"key":"2021122708460802300_b17","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1093\/hmg\/11.8.961","article-title":"Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder","volume":"11","author":"Wise","year":"2002","journal-title":"Hum Mol Genet"},{"key":"2021122708460802300_b18","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1038\/ng720","article-title":"CARD15 mutations in Blau syndrome","volume":"29","author":"Miceli-Richard","year":"2001","journal-title":"Nat Genet"},{"key":"2021122708460802300_b19","doi-asserted-by":"crossref","first-page":"1195","DOI":"10.1182\/blood-2004-07-2972","article-title":"Early-onset sarcoidosis and CARD15 mutations with constitutive nuclear factor-kappaB activation: common genetic etiology with Blau syndrome","volume":"105","author":"Kanazawa","year":"2005","journal-title":"Blood"},{"key":"2021122708460802300_b20","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1038\/88832","article-title":"Mutations in the gene encoding c-Abl-binding protein SH3BP2 cause cherubism","volume":"28","author":"Ueki","year":"2001","journal-title":"Nat Genet"},{"key":"2021122708460802300_b21","doi-asserted-by":"crossref","first-page":"2033","DOI":"10.1056\/NEJMoa0907206","article-title":"Inflammatory bowel disease and mutations affecting the interleukin-10 receptor","volume":"361","author":"Glocker","year":"2009","journal-title":"N Engl J Med"},{"key":"2021122708460802300_b22","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1056\/NEJMoa1013068","article-title":"Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis","volume":"365","author":"Marrakchi","year":"2011","journal-title":"N Engl J Med"},{"key":"2021122708460802300_b23","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1016\/j.ajhg.2011.07.022","article-title":"Mutations in IL36RN\/IL1F5 are associated with the severe episodic inflammatory skin disease known as generalized pustular psoriasis","volume":"89","author":"Onoufriadis","year":"2011","journal-title":"Am J Hum Genet"},{"key":"2021122708460802300_b24","doi-asserted-by":"crossref","first-page":"866","DOI":"10.1016\/j.ajhg.2010.10.031","article-title":"PSMB8 encoding the beta5i proteasome subunit is mutated in joint contractures, muscle atrophy, microcytic anemia, and panniculitis-induced lipodystrophy syndrome","volume":"87","author":"Agarwal","year":"2010","journal-title":"Am J Hum Genet"},{"key":"2021122708460802300_b25","doi-asserted-by":"crossref","first-page":"14914","DOI":"10.1073\/pnas.1106015108","article-title":"Proteasome assembly defect due to a proteasome subunit beta type 8 (PSMB8) mutation causes the autoinflammatory disorder, Nakajo\u2013Nishimura syndrome","volume":"108","author":"Arima","year":"2011","journal-title":"Proc Natl Acad Sci USA"},{"key":"2021122708460802300_b26","doi-asserted-by":"crossref","first-page":"4150","DOI":"10.1172\/JCI58414","article-title":"A mutation in the immunoproteasome subunit PSMB8 causes autoinflammation and lipodystrophy in humans","volume":"121","author":"Kitamura","year":"2011","journal-title":"J Clin Invest"},{"key":"2021122708460802300_b27","article-title":"Mutations in PSMB8 cause CANDLE syndrome with evidence of genetic and phenotypic heterogeneity","author":"Liu","year":"2011","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b28","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1146\/annurev.immunol.021908.132612","article-title":"Immunological and inflammatory functions of the interleukin-1 family","volume":"27","author":"Dinarello","year":"2009","journal-title":"Annu Rev Immunol"},{"key":"2021122708460802300_b29","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/j.immuni.2008.07.007","article-title":"Structures of the Toll-like receptor family and its ligand complexes","volume":"29","author":"Jin","year":"2008","journal-title":"Immunity"},{"key":"2021122708460802300_b30","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1038\/ni1253","article-title":"Are innate immune signaling pathways in plants and animals conserved?","volume":"6","author":"Ausubel","year":"2005","journal-title":"Nat Immunol"},{"key":"2021122708460802300_b31","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/S1074-7613(04)00046-9","article-title":"NALP3 forms an IL-1beta-processing inflammasome with increased activity in Muckle\u2013Wells autoinflammatory disorder","volume":"20","author":"Agostini","year":"2004","journal-title":"Immunity"},{"key":"2021122708460802300_b32","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1111\/j.1365-2249.2011.04534.x","article-title":"Immunology in the clinic review series; focus on autoinflammatory diseases: inflammasomes: mechanisms of activation","volume":"167","author":"Mankan","year":"2012","journal-title":"Clin Exp Immun"},{"key":"2021122708460802300_b33","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1126\/science.1184004","article-title":"How the noninflammasome NLRs function in the innate immune system","volume":"327","author":"Ting","year":"2010","journal-title":"Science"},{"key":"2021122708460802300_b34","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1016\/j.it.2011.05.004","article-title":"NLRP3 inflammasomes link inflammation and metabolic disease","volume":"32","author":"De Nardo","year":"2011","journal-title":"Trends Immunol"},{"key":"2021122708460802300_b35","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1038\/nm.2279","article-title":"The NLRP3 inflammasome instigates obesity-induced inflammation and insulin resistance","volume":"17","author":"Vandanmagsar","year":"2011","journal-title":"Nat Med"},{"key":"2021122708460802300_b36","doi-asserted-by":"crossref","first-page":"15324","DOI":"10.1073\/pnas.1100255108","article-title":"Inflammasome is a central player in the induction of obesity and insulin resistance","volume":"108","author":"Stienstra","year":"2011","journal-title":"Proc Natl Acad Sci USA"},{"key":"2021122708460802300_b37","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1038\/ni.1935","article-title":"Activation of the NLRP3 inflammasome by islet amyloid polypeptide provides a mechanism for enhanced IL-1beta in type 2 diabetes","volume":"11","author":"Masters","year":"2010","journal-title":"Nat Immunol"},{"key":"2021122708460802300_b38","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1038\/nature08938","article-title":"NLRP3 inflammasomes are required for atherogenesis and activated by cholesterol crystals","volume":"464","author":"Duewell","year":"2010","journal-title":"Nature"},{"key":"2021122708460802300_b39","doi-asserted-by":"crossref","first-page":"1779","DOI":"10.1016\/S0140-6736(04)17401-1","article-title":"Prevention of cold-associated acute inflammation in familial cold autoinflammatory syndrome by interleukin-1 receptor antagonist","volume":"364","author":"Hoffman","year":"2004","journal-title":"Lancet"},{"key":"2021122708460802300_b40","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/nature09663","article-title":"A role for mitochondria in NLRP3 inflammasome activation","volume":"469","author":"Zhou","year":"2011","journal-title":"Nature"},{"key":"2021122708460802300_b41","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1038\/nature07383","article-title":"Loss of the autophagy protein Atg16L1 enhances endotoxin-induced IL-1beta production","volume":"456","author":"Saitoh","year":"2008","journal-title":"Nature"},{"key":"2021122708460802300_b42","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1038\/ni.1980","article-title":"Autophagy proteins regulate innate immune responses by inhibiting the release of mitochondrial DNA mediated by NALP3 inflammasome","volume":"12","author":"Nakahira","year":"2011","journal-title":"Nat Immunol"},{"key":"2021122708460802300_b43","doi-asserted-by":"crossref","first-page":"1196","DOI":"10.1002\/eji.201141436","article-title":"Mitochondria: sovereign of inflammation?","volume":"41","author":"Tschopp","year":"2011","journal-title":"Eur J Immunol"},{"key":"2021122708460802300_b44","doi-asserted-by":"crossref","first-page":"9789","DOI":"10.1073\/pnas.1000779107","article-title":"Altered redox state of monocytes from cryopyrin-associated periodic syndromes causes accelerated IL-1beta secretion","volume":"107","author":"Tassi","year":"2010","journal-title":"Proc Natl Acad Sci USA"},{"key":"2021122708460802300_b45","doi-asserted-by":"crossref","first-page":"3579","DOI":"10.1002\/art.21404","article-title":"Somatic mosaicism of CIAS1 in a patient with chronic infantile neurologic, cutaneous, articular syndrome","volume":"52","author":"Saito","year":"2005","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b46","doi-asserted-by":"crossref","first-page":"1158","DOI":"10.1002\/art.27342","article-title":"A somatic NLRP3 mutation as a cause of a sporadic case of chronic infantile neurologic, cutaneous, articular syndrome\/neonatal-onset multisystem inflammatory disease: novel evidence of the role of low-level mosaicism as the pathophysiologic mechanism underlying mendelian inherited diseases","volume":"62","author":"Arostegui","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b47","doi-asserted-by":"crossref","first-page":"3625","DOI":"10.1002\/art.30512","article-title":"High incidence of NLRP3 somatic mosaicism in chronic infantile neurological cutaneous and articular syndrome patients: the results of an international multicenter collaborative study","volume":"63","author":"Tanaka","year":"2011","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b48","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1111\/j.1365-2249.2011.04535.x","article-title":"Immunology in the clinic review series; focus on autoinflammatory diseases: role of inflammasomes in autoinflammatory syndromes","volume":"167","author":"Ozkurede","year":"2012","journal-title":"Clin Exp Immun"},{"key":"2021122708460802300_b49","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.1084\/jem.20082481","article-title":"In vivo regulation of interleukin 1beta in patients with cryopyrin-associated periodic syndromes","volume":"206","author":"Lachmann","year":"2009","journal-title":"J Exp Med"},{"key":"2021122708460802300_b50","doi-asserted-by":"crossref","first-page":"57","DOI":"10.3109\/03009748709102523","article-title":"A chronic, infantile, neurological, cutaneous and articular (CINCA) syndrome. A specific entity analysed in 30 patients","volume":"66","author":"Prieur","year":"1987","journal-title":"Scand J Rheumatol Suppl"},{"key":"2021122708460802300_b51","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1007\/s11926-011-0165-y","article-title":"Current status of understanding the pathogenesis and management of patients with NOMID\/CINCA","volume":"13","author":"Goldbach-Mansky","year":"2011","journal-title":"Curr Rheumatol Rep"},{"key":"2021122708460802300_b52","first-page":"235","article-title":"Urticaria, deafness, and amyloidosis: a new heredo-familial syndrome","volume":"31","author":"Muckle","year":"1962","journal-title":"Q J Med"},{"key":"2021122708460802300_b53","doi-asserted-by":"crossref","first-page":"1386","DOI":"10.1001\/archopht.118.10.1386","article-title":"Chronic infantile neurological cutaneous and articular\/neonatal onset multisystem inflammatory disease syndrome: ocular manifestations in a recently recognized chronic inflammatory disease of childhood","volume":"118","author":"Dollfus","year":"2000","journal-title":"Arch Ophthalmol"},{"key":"2021122708460802300_b54","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1177\/0194599811402296","article-title":"Cryopyrin-associated periodic syndromes: otolaryngologic and audiologic manifestations","volume":"145","author":"Ahmadi","year":"2011","journal-title":"Otolaryngol Head Neck Surg"},{"key":"2021122708460802300_b55","doi-asserted-by":"crossref","first-page":"3783","DOI":"10.1002\/art.27696","article-title":"Risk factors for severe Muckle\u2013Wells syndrome","volume":"62","author":"Kummerle-Deschner","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b56","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1056\/NEJMoa055137","article-title":"Neonatal-onset multisystem inflammatory disease responsive to interleukin-1beta inhibition","volume":"355","author":"Goldbach-Mansky","year":"2006","journal-title":"N Engl J Med"},{"key":"2021122708460802300_b57","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1002\/art.25057","article-title":"Long-term efficacy of the interleukin-1 receptor antagonist anakinra in ten patients with neonatal-onset multisystem inflammatory disease\/chronic infantile neurologic, cutaneous, articular syndrome","volume":"62","author":"Neven","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b58","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1212\/WNL.0b013e3181d9ed69","article-title":"Neurologic manifestations of the cryopyrin-associated periodic syndrome","volume":"74","author":"Kitley","year":"2010","journal-title":"Neurology"},{"key":"2021122708460802300_b59","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1007\/s00247-006-0358-0","article-title":"Arthropathy of neonatal onset multisystem inflammatory disease (NOMID\/CINCA)","volume":"37","author":"Hill","year":"2007","journal-title":"Pediatr Radiol"},{"key":"2021122708460802300_b60","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1016\/j.jpeds.2010.02.040","article-title":"Follow-up and quality of life of patients with cryopyrin-associated periodic syndromes treated with Anakinra","volume":"157","author":"Lepore","year":"2010","journal-title":"J Pediatr"},{"key":"2021122708460802300_b61","doi-asserted-by":"crossref","first-page":"840","DOI":"10.1002\/art.30149","article-title":"Efficacy and safety of anakinra therapy in pediatric and adult patients with the autoinflammatory Muckle\u2013Wells syndrome","volume":"63","author":"Kuemmerle-Deschner","year":"2011","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b62","doi-asserted-by":"crossref","first-page":"2443","DOI":"10.1002\/art.23687","article-title":"Efficacy and safety of rilonacept (interleukin-1 Trap) in patients with cryopyrin-associated periodic syndromes: results from two sequential placebo-controlled studies","volume":"58","author":"Hoffman","year":"2008","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b63","doi-asserted-by":"crossref","first-page":"2432","DOI":"10.1002\/art.23620","article-title":"A pilot study to evaluate the safety and efficacy of the long-acting interleukin-1 inhibitor rilonacept (interleukin-1 Trap) in patients with familial cold autoinflammatory syndrome","volume":"58","author":"Goldbach-Mansky","year":"2008","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b64","doi-asserted-by":"crossref","first-page":"2416","DOI":"10.1056\/NEJMoa0810787","article-title":"Use of canakinumab in the cryopyrin-associated periodic syndrome","volume":"360","author":"Lachmann","year":"2009","journal-title":"N Engl J Med"},{"key":"2021122708460802300_b65","doi-asserted-by":"crossref","first-page":"2095","DOI":"10.1136\/ard.2011.152728","article-title":"Two-year results from an open-label, multicentre, phase III study evaluating the safety and efficacy of canakinumab in patients with cryopyrin-associated periodic syndrome across different severity phenotypes","volume":"70","author":"Kuemmerle-Deschner","year":"2011","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b66","volume-title":"Anakinra package insert"},{"key":"2021122708460802300_b67","volume-title":"Rilonacept package insert"},{"key":"2021122708460802300_b68","volume-title":"Canakinumab package insert"},{"key":"2021122708460802300_b69","doi-asserted-by":"crossref","first-page":"4007","DOI":"10.1002\/art.30588","article-title":"A novel mutation of IL1RN in the deficiency of interleukin-1 receptor antagonist syndrome. Description of two unrelated cases from Brazil","volume":"63","author":"Jesus","year":"2011","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b70","doi-asserted-by":"crossref","first-page":"4018","DOI":"10.1002\/art.30565","article-title":"The first case of compound heterozygous IL1RN mutations causing deficiency of the interleukin-1-receptor antagonist","volume":"63","author":"Stenerson","year":"2011","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b71","doi-asserted-by":"crossref","first-page":"1479","DOI":"10.1084\/jem.20050473","article-title":"Role of interleukin-1 (IL-1) in the pathogenesis of systemic onset juvenile idiopathic arthritis and clinical response to IL-1 blockade","volume":"201","author":"Pascual","year":"2005","journal-title":"J Exp Med"},{"key":"2021122708460802300_b72","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1136\/ard.2010.134254","article-title":"A multicentre, randomised, double-blind, placebo-controlled trial with the interleukin-1 receptor antagonist anakinra in patients with systemic-onset juvenile idiopathic arthritis (ANAJIS trial)","volume":"70","author":"Quartier","year":"2011","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b73","doi-asserted-by":"crossref","first-page":"7148","DOI":"10.1073\/pnas.1103681108","article-title":"Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade","volume":"108","author":"Stojanov","year":"2011","journal-title":"Proc Natl Acad Sci USA"},{"key":"2021122708460802300_b74","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1136\/ard.2005.045245","article-title":"Beneficial response to anakinra and thalidomide in Schnitzler's syndrome","volume":"65","author":"de Koning","year":"2006","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b75","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1016\/j.jaci.2007.09.021","article-title":"IL-1 blockade in Schnitzler syndrome: ex vivo findings correlate with clinical remission","volume":"121","author":"Ryan","year":"2008","journal-title":"J Allergy Clin Immunol"},{"key":"2021122708460802300_b76","doi-asserted-by":"crossref","first-page":"1190","DOI":"10.1111\/j.1365-4632.2009.04151.x","article-title":"Schnitzler syndrome: response to anakinra in two cases and a review of the literature","volume":"48","author":"Schuster","year":"2009","journal-title":"Int J Dermatol"},{"key":"2021122708460802300_b77","doi-asserted-by":"crossref","first-page":"284","DOI":"10.7326\/0003-4819-149-4-200808190-00018","article-title":"Resistant Beh\u00e7et disease responsive to anakinra","volume":"149","author":"Botsios","year":"2008","journal-title":"Ann Intern Med"},{"key":"2021122708460802300_b78","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1007\/s10067-009-1279-8","article-title":"Anti-IL-1 treatment for secondary amyloidosis in an adolescent with FMF and Beh\u00e7et's disease","volume":"29","author":"Bilginer","year":"2010","journal-title":"Clin Rheumatol"},{"key":"2021122708460802300_b79","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.bone.2005.07.009","article-title":"A missense mutation in pstpip2 is associated with the murine autoinflammatory disorder chronic multifocal osteomyelitis","volume":"38","author":"Ferguson","year":"2006","journal-title":"Bone"},{"key":"2021122708460802300_b80","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1097\/BOR.0b013e32825f5492","article-title":"Autoinflammatory bone disorders","volume":"19","author":"Ferguson","year":"2007","journal-title":"Curr Opin Rheumatol"},{"key":"2021122708460802300_b81","doi-asserted-by":"crossref","first-page":"1683","DOI":"10.1136\/ard.2007.073759","article-title":"Management of treatment resistant inflammation of acute on chronic tophaceous gout with anakinra","volume":"66","author":"McGonagle","year":"2007","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b82","doi-asserted-by":"crossref","first-page":"1613","DOI":"10.1136\/ard.2009.108936","article-title":"The interleukin 1 inhibitor rilonacept in treatment of chronic gouty arthritis: results of a placebo-controlled, monosequence crossover, non-randomised, single-blind pilot study","volume":"68","author":"Terkeltaub","year":"2009","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b83","doi-asserted-by":"crossref","first-page":"3064","DOI":"10.1002\/art.27600","article-title":"Canakinumab for the treatment of acute flares in difficult-to-treat gouty arthritis: results of a multicenter, phase II, dose-ranging study","volume":"62","author":"So","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b84","doi-asserted-by":"crossref","first-page":"1517","DOI":"10.1056\/NEJMoa065213","article-title":"Interleukin-1-receptor antagonist in type 2 diabetes mellitus","volume":"356","author":"Larsen","year":"2007","journal-title":"N Engl J Med"},{"key":"2021122708460802300_b85","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1038\/ni.1636","article-title":"The NALP3 inflammasome is involved in the innate immune response to amyloid-beta","volume":"9","author":"Halle","year":"2008","journal-title":"Nat Immunol"},{"key":"2021122708460802300_b86","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1126\/science.1156995","article-title":"Innate immune activation through Nalp3 inflammasome sensing of asbestos and silica","volume":"320","author":"Dostert","year":"2008","journal-title":"Science"},{"key":"2021122708460802300_b87","doi-asserted-by":"crossref","first-page":"2603","DOI":"10.1084\/jem.20070157","article-title":"Opposing activities of two novel members of the IL-1 ligand family regulate skin inflammation","volume":"204","author":"Blumberg","year":"2007","journal-title":"J Exp Med"},{"key":"2021122708460802300_b88","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1093\/hmg\/ddq455","article-title":"Protein kinase A regulates caspase-1 via Ets-1 in bone stromal cell-derived lesions: a link between cyclic AMP and pro-inflammatory pathways in osteoblast progenitors","volume":"20","author":"Almeida","year":"2011","journal-title":"Hum Mol Genet"},{"key":"2021122708460802300_b89","doi-asserted-by":"crossref","first-page":"4354","DOI":"10.4049\/jimmunol.1000313","article-title":"IL-1RL2 and its ligands contribute to the cytokine network in psoriasis","volume":"185","author":"Blumberg","year":"2010","journal-title":"J Immunol"},{"key":"2021122708460802300_b90","doi-asserted-by":"crossref","first-page":"5813","DOI":"10.1182\/blood-2011-05-356873","article-title":"IL-36R ligands are potent regulators of dendritic and T cells","volume":"118","author":"Vigne","year":"2011","journal-title":"Blood"},{"key":"2021122708460802300_b91","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1084\/jem.187.4.571","article-title":"The orphan receptor CRF2-4 is an essential subunit of the interleukin 10 receptor","volume":"187","author":"Spencer","year":"1998","journal-title":"J Exp Med"},{"key":"2021122708460802300_b92","doi-asserted-by":"crossref","first-page":"1319","DOI":"10.1038\/ng.221","article-title":"Sequence variants in IL10, ARPC2 and multiple other loci contribute to ulcerative colitis susceptibility","volume":"40","author":"Franke","year":"2008","journal-title":"Nat Genet"},{"key":"2021122708460802300_b93","doi-asserted-by":"crossref","first-page":"1118","DOI":"10.1038\/ng.717","article-title":"Genome-wide meta-analysis increases to 71 the number of confirmed Crohn's disease susceptibility loci","volume":"42","author":"Franke","year":"2010","journal-title":"Nat Genet"},{"key":"2021122708460802300_b94","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1016\/j.humimm.2006.11.010","article-title":"IL-10 genotype analysis in patients with Beh\u00e7et's disease","volume":"68","author":"Wallace","year":"2007","journal-title":"Hum Immunol"},{"key":"2021122708460802300_b95","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1038\/ng.625","article-title":"Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Beh\u00e7et's disease","volume":"42","author":"Remmers","year":"2010","journal-title":"Nat Genet"},{"key":"2021122708460802300_b96","doi-asserted-by":"crossref","first-page":"1053","DOI":"10.1001\/archderm.1985.01660080107026","article-title":"A syndrome with nodular erythema, elongated and thickened fingers, and emaciation","volume":"121","author":"Kitano","year":"1985","journal-title":"Arch Dermatol"},{"key":"2021122708460802300_b97","doi-asserted-by":"crossref","first-page":"42","DOI":"10.2169\/internalmedicine.32.42","article-title":"Hereditary lipo-muscular atrophy with joint contracture, skin eruptions and hyper-gamma-globulinemia: a new syndrome","volume":"32","author":"Tanaka","year":"1993","journal-title":"Intern Med"},{"key":"2021122708460802300_b98","doi-asserted-by":"crossref","first-page":"203","DOI":"10.3109\/s10165-008-0033-4","article-title":"A case of periodic-fever-syndrome-like disorder with lipodystrophy, myositis, and autoimmune abnormalities","volume":"18","author":"Kasagi","year":"2008","journal-title":"Mod Rheumatol"},{"key":"2021122708460802300_b99","doi-asserted-by":"crossref","first-page":"E58","DOI":"10.1210\/jc.2010-0488","article-title":"An autosomal recessive syndrome of joint contractures, muscular atrophy, microcytic anemia, and panniculitis-associated lipodystrophy","volume":"95","author":"Garg","year":"2010","journal-title":"J Clin Endocrinol Metab"},{"key":"2021122708460802300_b100","first-page":"1084","article-title":"An unknown autoinflammatory syndrome associated with short stature and dysmorphic features in a young boy","volume":"29","author":"Megarbane","year":"2002","journal-title":"J Rheumatol"},{"key":"2021122708460802300_b101","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1016\/j.jaad.2009.04.046","article-title":"Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome","volume":"62","author":"Torrelo","year":"2010","journal-title":"J Am Acad Dermatol"},{"key":"2021122708460802300_b102","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1111\/j.1525-1470.2010.01163.x","article-title":"Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome: a case report","volume":"28","author":"Ramot","year":"2011","journal-title":"Pediatr Dermatol"},{"key":"2021122708460802300_b103","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1007\/BF00688252","article-title":"An autopsy case of a syndrome with muscular atrophy, decreased subcutaneous fat, skin eruption and hyper gamma-globulinemia: peculiar vascular changes and muscle fiber degeneration","volume":"73","author":"Oyanagi","year":"1987","journal-title":"Acta Neuropathol"},{"key":"2021122708460802300_b104","doi-asserted-by":"crossref","first-page":"3439","DOI":"10.1002\/eji.201040620","article-title":"Immunoproteasomes are essential for survival and expansion of T cells in virus-infected mice","volume":"40","author":"Moebius","year":"2010","journal-title":"Eur J Immunol"},{"key":"2021122708460802300_b105","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1016\/j.cell.2010.07.036","article-title":"Immunoproteasomes preserve protein homeostasis upon interferon-induced oxidative stress","volume":"142","author":"Seifert","year":"2010","journal-title":"Cell"},{"key":"2021122708460802300_b106","doi-asserted-by":"crossref","first-page":"59","DOI":"10.2119\/2006-00085.Baechler","article-title":"An interferon signature in the peripheral blood of dermatomyositis patients is associated with disease activity","volume":"13","author":"Baechler","year":"2007","journal-title":"Mol Med"},{"key":"2021122708460802300_b107","doi-asserted-by":"crossref","first-page":"1815","DOI":"10.1002\/art.24555","article-title":"Elevated serum interferon-alpha activity in juvenile dermatomyositis: associations with disease activity at diagnosis and after thirty-six months of therapy","volume":"60","author":"Niewold","year":"2009","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b108","doi-asserted-by":"crossref","first-page":"2619","DOI":"10.1002\/art.27525","article-title":"Mast cells and type I interferon responses in the skin of patients with juvenile dermatomyositis: are current therapies just scratching the surface?","volume":"62","author":"Rider","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b109","doi-asserted-by":"crossref","first-page":"2813","DOI":"10.1002\/art.27529","article-title":"Lesional and nonlesional skin from patients with untreated juvenile dermatomyositis displays increased numbers of mast cells and mature plasmacytoid dendritic cells","volume":"62","author":"Shrestha","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b110","first-page":"106","article-title":"SLE like syndrome and functional deficiency of C1q in members of a large family","volume":"55","author":"Hannema","year":"1984","journal-title":"Clin Exp Immunol"},{"key":"2021122708460802300_b111","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1007\/BF00376120","article-title":"A homozygous point mutation results in a stop codon in the C1q B-chain of a C1q-deficient individual","volume":"27","author":"McAdam","year":"1988","journal-title":"Immunogenetics"},{"key":"2021122708460802300_b112","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1038\/ng0598-56","article-title":"Homozygous C1q deficiency causes glomerulonephritis associated with multiple apoptotic bodies","volume":"19","author":"Botto","year":"1998","journal-title":"Nat Genet"},{"key":"2021122708460802300_b113","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1038\/ng1842","article-title":"Mutations in genes encoding ribonuclease H2 subunits cause Aicardi\u2013Goutieres syndrome and mimic congenital viral brain infection","volume":"38","author":"Crow","year":"2006","journal-title":"Nat Genet"},{"key":"2021122708460802300_b114","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1038\/ng1845","article-title":"Mutations in the gene encoding the 3\u2032-5\u2032 DNA exonuclease TREX1 cause Aicardi\u2013Goutieres syndrome at the AGS1 locus","volume":"38","author":"Crow","year":"2006","journal-title":"Nat Genet"},{"key":"2021122708460802300_b115","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1038\/ng.749","article-title":"Genetic deficiency of tartrate-resistant acid phosphatase associated with skeletal dysplasia, cerebral calcifications and autoimmunity","volume":"43","author":"Lausch","year":"2011","journal-title":"Nat Genet"},{"key":"2021122708460802300_b116","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1038\/ng.748","article-title":"Tartrate-resistant acid phosphatase deficiency causes a bone dysplasia with autoimmunity and a type I interferon expression signature","volume":"43","author":"Briggs","year":"2011","journal-title":"Nat Genet"},{"key":"2021122708460802300_b117","doi-asserted-by":"crossref","first-page":"73ra20","DOI":"10.1126\/scitranslmed.3001201","article-title":"Netting neutrophils are major inducers of type I IFN production in pediatric systemic lupus erythematosus","volume":"3","author":"Garcia-Romo","year":"2011","journal-title":"Sci Transl Med"},{"key":"2021122708460802300_b118","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1089\/jir.2011.0045","article-title":"Type I interferon and systemic lupus erythematosus","volume":"31","author":"Elkon","year":"2011","journal-title":"J Interferon Cytokine Res"},{"key":"2021122708460802300_b119","doi-asserted-by":"crossref","first-page":"516","DOI":"10.3899\/jrheum.100718","article-title":"Anti-interleukin 1 treatment for patients with familial Mediterranean fever resistant to colchicine","volume":"38","author":"Ozen","year":"2011","journal-title":"J Rheumatol"},{"key":"2021122708460802300_b120","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/j.semarthrit.2010.11.003","article-title":"Interleukin-1 targeting drugs in familial Mediterranean fever: a case series and a review of the literature","volume":"41","author":"Meinzer","year":"2011","journal-title":"Semin Arthritis Rheum"},{"key":"2021122708460802300_b121","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1093\/ndt\/gfn646","article-title":"Successful treatment of familial Mediterranean fever with Anakinra and outcome after renal transplantation","volume":"24","author":"Moser","year":"2009","journal-title":"Nephrol Dial Transplant"},{"key":"2021122708460802300_b122","doi-asserted-by":"crossref","first-page":"504","DOI":"10.1016\/j.jbspin.2008.04.001","article-title":"Anakinra: new therapeutic approach in children with familial Mediterranean fever resistant to colchicine","volume":"75","author":"Roldan","year":"2008","journal-title":"Joint Bone Spine"},{"key":"2021122708460802300_b123","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1007\/s00431-007-0547-3","article-title":"The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever","volume":"167","author":"Calligaris","year":"2008","journal-title":"Eur J Pediatr"},{"key":"2021122708460802300_b124","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1111\/j.1365-2362.2007.01868.x","article-title":"Anakinra in two adolescent female patients suffering from colchicine-resistant familial Mediterranean fever: effective but risky","volume":"37","author":"Gattringer","year":"2007","journal-title":"Eur J Clin Invest"},{"key":"2021122708460802300_b125","doi-asserted-by":"crossref","first-page":"1545","DOI":"10.1136\/ard.2007.071498","article-title":"Effective treatment of a colchicine-resistant familial Mediterranean fever patient with anakinra","volume":"66","author":"Kuijk","year":"2007","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b126","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1016\/j.amjmed.2004.02.039","article-title":"Beneficial response to interleukin 1 receptor antagonist in traps","volume":"117","author":"Simon","year":"2004","journal-title":"Am J Med"},{"key":"2021122708460802300_b127","first-page":"357","article-title":"Dramatic improvement following interleukin 1beta blockade in tumor necrosis factor receptor-1-associated syndrome (TRAPS) resistant to anti-TNF-alpha therapy","volume":"35","author":"Sacre","year":"2008","journal-title":"J Rheumatol"},{"key":"2021122708460802300_b128","doi-asserted-by":"crossref","first-page":"1516","DOI":"10.1002\/art.23475","article-title":"Persistent efficacy of anakinra in patients with tumor necrosis factor receptor-associated periodic syndrome","volume":"58","author":"Gattorno","year":"2008","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b129","doi-asserted-by":"crossref","first-page":"1511","DOI":"10.1136\/ard.2010.143438","article-title":"Favourable and sustained response to anakinra in tumour necrosis factor receptor-associated periodic syndrome (TRAPS) with or without AA amyloidosis","volume":"70","author":"Obici","year":"2011","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b130","doi-asserted-by":"crossref","first-page":"2155","DOI":"10.1136\/ard.2011.149922","article-title":"On-demand anakinra treatment is effective in mevalonate kinase deficiency","volume":"70","author":"Bodar","year":"2011","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b131","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1007\/s10545-006-0408-7","article-title":"Anakinra is safe and effective in controlling hyperimmunoglobulinaemia D syndrome-associated febrile crisis","volume":"29","author":"Cailliez","year":"2006","journal-title":"J Inherit Metab Dis"},{"key":"2021122708460802300_b132","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1093\/rheumatology\/keh479","article-title":"Anakinra for flares of pyogenic arthritis in PAPA syndrome","volume":"44","author":"Dierselhuis","year":"2005","journal-title":"Rheumatology (Oxf)"},{"key":"2021122708460802300_b133","doi-asserted-by":"crossref","first-page":"1199","DOI":"10.1111\/j.1365-2133.2009.09404.x","article-title":"Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra","volume":"161","author":"Brenner","year":"2009","journal-title":"Br J Dermatol"},{"key":"2021122708460802300_b134","doi-asserted-by":"crossref","first-page":"1505","DOI":"10.1002\/art.23437","article-title":"The pattern of response to anti-interleukin-1 treatment distinguishes two subsets of patients with systemic-onset juvenile idiopathic arthritis","volume":"58","author":"Gattorno","year":"2008","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b135","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1136\/ard.2004.026617","article-title":"Refractory adult onset Still's disease successfully treated with anakinra","volume":"64","author":"Vasques Godinho","year":"2005","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b136","doi-asserted-by":"crossref","first-page":"1794","DOI":"10.1002\/art.21061","article-title":"Rapid responses to anakinra in patients with refractory adult-onset Still's disease","volume":"52","author":"Fitzgerald","year":"2005","journal-title":"Arthritis Rheum"},{"key":"2021122708460802300_b137","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.semarthrit.2007.04.002","article-title":"Anakinra in patients with treatment-resistant adult-onset Still's disease: four case reports with serial cytokine measurements and a review of the literature","volume":"37","author":"Kotter","year":"2007","journal-title":"Semin Arthritis Rheum"},{"key":"2021122708460802300_b138","first-page":"939","article-title":"Effective treatment of steroid refractory adult-onset Still's disease with anakinra","volume":"35","author":"Maier","year":"2008","journal-title":"J Rheumatol"},{"key":"2021122708460802300_b139","doi-asserted-by":"crossref","first-page":"200","DOI":"10.3109\/s10165-009-0254-1","article-title":"Marked effect and steroid-sparing ability of anakinra on a patient with refractory adult-onset Still's disease","volume":"20","author":"Tamaki","year":"2010","journal-title":"Mod Rheumatol"},{"key":"2021122708460802300_b140","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1136\/ard.2009.108068","article-title":"IL1-receptor antagonist anakinra provides long-lasting efficacy in the treatment of refractory adult-onset Still's disease","volume":"69","author":"Naumann","year":"2010","journal-title":"Ann Rheum Dis"},{"key":"2021122708460802300_b141","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1097\/RHU.0b013e318188b8d8","article-title":"Efficacy of anakinra in a case of refractory Still disease","volume":"14","author":"Debiais","year":"2008","journal-title":"J Clin Rheumatol"},{"key":"2021122708460802300_b142","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1038\/ng.373","article-title":"Mutations involved in Aicardi\u2013Goutieres syndrome implicate SAMHD1 as regulator of the innate immune response","volume":"41","author":"Rice","year":"2009","journal-title":"Nat Genet"}],"container-title":["Clinical and Experimental Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-2249.2011.04533.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/academic.oup.com\/cei\/article-pdf\/167\/3\/391\/41907681\/j.1365-2249.2011.04533.x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/cei\/article-pdf\/167\/3\/391\/41907681\/j.1365-2249.2011.04533.x.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,1]],"date-time":"2022-01-01T16:26:03Z","timestamp":1641054363000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/cei\/article\/167\/3\/391\/6422812"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,1,30]]},"references-count":142,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2012,1,30]]},"published-print":{"date-parts":[[2012,1,30]]}},"URL":"https:\/\/doi.org\/10.1111\/j.1365-2249.2011.04533.x","relation":{},"ISSN":["0009-9104","1365-2249"],"issn-type":[{"value":"0009-9104","type":"print"},{"value":"1365-2249","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2012,3]]},"published":{"date-parts":[[2012,1,30]]}}}