Polygenic autoinflammatory diseases (AIDs), such as systemic juvenile idiopathic arthritis (sJIA), adult-onset Still's disease, Kawasaki disease, idiopathic recurrent pericarditis (IRP), Behçet's Syndrome, Crystal-induced arthropatihes such as gout or Calcium pyrophosphate deposition disease are characterized by the overexpression of inflammasome-associated genes, leading to a dysregulation of the innate immune response. The IL-1 cytokine family (IL-1α, IL-1β, IL-1Ra, IL-18, IL-36Ra, IL-36α, IL-37, IL-36β, IL-36g, IL-38, IL-33) was defined to be principally responsible for the inflammatory nature of polygenic AIDs. Several clinical trials were initiated, and IL-1 blockade has been proven to cause a rapid reduction of clinical symptoms and normalization of laboratory parameters in the majority of cases. Randomized, placebo-controlled, clinical trials, together with registry-based clinical trials and open-label, retrospective and prospective observational studies, supported the efficacy and safety of IL-1 inhibitors in the treatment of polygenic AIDs. Most of the current data are focused on the therapeutic use of anakinra, an IL-1 receptor antagonist, canakinumab, an anti-IL-1β monoclonal antibody, and rilonacept, a soluble decoy receptor. However, other promising agents, such as gevokizumab, IL-1β blocking monoclonal antibody, tadekinig alfa, a human recombinant IL-18-binding protein, and tranilast, an analog of a tryptophan metabolite, are currently being tested. Anakinra, canakinumab and rilonacept caused impressive improvements in both systemic and musculoskeletal symptoms. Furthermore, the anti-IL-1 therapy allowed corticosteroid tapering and, in some cases, even withdrawal. This article reviews the current IL-1 inhibitors and the results of all clinical trials in which they have been tested for the management of broad spectrum of polygenic AIDs.

Department of Clinical Immunology and Allergology Institute for Clinical and Experimental Medicine Prague Czech Republic

Department of Immunology 2nd Faculty of Medicine Charles University and University Hospital Motol Prague Czechia

Department of Paediatric and Adult Rheumatology University Hospital Motol Prague Czechia

Zobrazit více v PubMed

Agarwal S., Agrawal D. K. (2017). Kawasaki disease: etiopathogenesis and novel treatment strategies. Expet Rev. Clin. Immunol. 13 (3), 247–258. 10.1080/1744666X.2017.1232165 PubMed DOI PMC

Banse C., Vittecoq O., Benhamou Y., Gauthier-Prieur M., Lequerré T., Lévesque H. (2013). Reactive macrophage activation syndrome possibly triggered by canakinumab in a patient with adult-onset Still's disease. Joint Bone Spine 80 (6), 653–655. 10.1016/j.jbspin.2013.04.011 PubMed DOI

Barsotti S., Neri R., Iacopetti V., d'Ascanio A., Talarico R., Tripoli A., et al. (2014). Successful treatment of refractory adult-onset still disease with canakinumab: a case report. J. Clin. Rheumatol. 20 (2), 121. 10.1097/RHU.0000000000000082 PubMed DOI

Bettiol A., Silvestri E., Di Scala G., Amedei A., Becatti M., Fiorillo C., et al. (2019). The right place of interleukin-1 inhibitors in the treatment of Behçet's syndrome: a systematic review. Rheumatol. Int. 39 (6), 971–990. 10.1007/s00296-019-04259-y PubMed DOI

Blonz G., Lacroix S., Benbrik N., Warin-Fresse K., Masseau A., Trewick D., et al. (2018). Severe late-onset Kawasaki disease successfully treated with anakinra. J. Clin. Rheumatol. 26, e42–e43. 10.1097/RHU.0000000000000814 PubMed DOI

Boraschi D., Italiani P., Weil S., Martin M. U. (2018). The family of the interleukin-1 receptors. Immunol. Rev. 281 (1), 197–232. 10.1111/imr.12606 PubMed DOI

Botsios C., Sfriso P., Furlan A., Punzi L., Dinarello C. A. (2008). Resistant Behçet disease responsive to anakinra. Ann. Intern. Med. 149 (4), 284–286. 10.7326/0003-4819-149-4-200808190-00018 PubMed DOI

Brucato A., Imazio M., Gattorno M., Lazaros G., Maestroni S., Carraro M., et al. (2016). Effect of anakinra on recurrent pericarditis among patients with colchicine resistance and corticosteroid dependence: the AIRTRIP randomized clinical trial. J. Am. Med. Assoc. 316 (18), 1906–1912. 10.1001/jama.2016.15826 PubMed DOI

Brunner H. I., Quartier P., Alexeeva E., Constantin T., Kone-Paut I., Marzan K., et al. Paediatric Rheumatology International Trials Organisation (2020). Efficacy and safety of canakinumab in sJIA patients with and without fever at baseline: results from an open-label, active treatment extension study. Arthritis Rheumatol. 72, 2147–2158. 10.1002/art.41436 PubMed DOI

Cantarini L., Lopalco G., Selmi C., Napodano S., De Rosa G., Caso F., et al. (2015). Autoimmunity and autoinflammation as the yin and yang of idiopathic recurrent acute pericarditis. Autoimmun. Rev. 14 (2), 90–97. 10.1016/j.autrev.2014.10.005 PubMed DOI

Cantarini L., Vitale A., Borri M., Galeazzi M., Franceschini R. (2012). Successful use of canakinumab in a patient with resistant Behçet's disease. Clin. Exp. Rheumatol. 30 (3 Suppl. 72), S115. PubMed

Cavalli G., Dinarello C. A. (2018). Anakinra therapy for non-cancer inflammatory diseases. Front. Pharmacol. 9, 1157. 10.3389/fphar.2018.01157 PubMed DOI PMC

Cavalli G., Franchini S., Aiello P., Guglielmi B., Berti A., Campochiaro C., et al. (2015). Efficacy and safety of biological agents in adult-onset Still's disease. Scand. J. Rheumatol. 44 (4), 309–314. 10.3109/03009742.2014.992949 PubMed DOI

Chen K., Fields T., Mancuso C. A., Bass A. R., Vasanth L. (2010). Anakinra's efficacy is variable in refractory gout: report of ten cases. Semin. Arthritis Rheum. 40 (3), 210–214. 10.1016/j.semarthrit.2010.03.001 PubMed DOI

Cimaz R. (2016). Systemic-onset juvenile idiopathic arthritis. Autoimmun. Rev. 15 (9), 931–934. 10.1016/j.autrev.2016.07.004 PubMed DOI

Clancy D. M., Sullivan G. P., Moran H. B. T., Henry C. M., Reeves E. P., McElvaney N. G., et al. (2018). Extracellular neutrophil proteases are efficient regulators of IL-1, IL-33, and IL-36 cytokine activity but poor effectors of microbial killing. Cell Rep. 22 (11), 2937–2950. 10.1016/j.celrep.2018.02.062 PubMed DOI

Clinical Trials (2020). ClinicalTrials. U.S. National Library of Medicine; Available at: https://clinicaltrials.gov (Accessed December 16 , 2020).

Cohen S., Tacke C. E., Straver B., Meijer N., Kuipers I. M., Kuijpers T. W. (2012). A child with severe relapsing Kawasaki disease rescued by IL-1 receptor blockade and extracorporeal membrane oxygenation. Ann. Rheum. Dis. 71 (12), 2059–2061. 10.1136/annrheumdis-2012-201658 PubMed DOI

Dalbeth N., Merriman T. R., Stamp L. K. (2016). Gout. Lancet 388 (10055), 2039–2052. 10.1016/S0140-6736(16)00346-9 PubMed DOI

Dalbeth N., Stamp L. K., Merriman T. R. (2017). The genetics of gout: towards personalised medicine? BMC Med. 15 (1), 108. 10.1186/s12916-017-0878-5 PubMed DOI PMC

Dall'Ara F., Frassi M., Tincani A., Airò P. (2016). A retrospective study of patients with adult-onset Still's disease: is pericarditis a possible predictor for biological disease-modifying anti-rheumatic drugs need? Clin. Rheumatol. 35 (8), 2117–2123. 10.1007/s10067-015-3164-y PubMed DOI

De Rosa G., Pardeo M., Rigante D. (2007). Current recommendations for the pharmacologic therapy in Kawasaki syndrome and management of its cardiovascular complications. Eur. Rev. Med. Pharmacol. Sci. 11 (5), 301–308. PubMed

Di Paolo N. C., Shayakhmetov D. M. (2016). Interleukin 1α and the inflammatory process. Nat. Immunol. 17 (8), 906–913. 10.1038/ni.3503 PubMed DOI PMC

Dinarello C. A. (2018). Overview of the IL-1 family in innate inflammation and acquired immunity. Immunol. Rev. 281 (1), 8–27. 10.1111/imr.12621 PubMed DOI PMC

Dinarello C. A. (2019). The IL-1 family of cytokines and receptors in rheumatic diseases. Nat. Rev. Rheumatol. 15 (10), 612–632. 10.1038/s41584-019-0277-8 PubMed DOI

Dinarello C. A. (2011). Interleukin-1 in the pathogenesis and treatment of inflammatory diseases. Blood 117 (14), 3720–3732. 10.1182/blood-2010-07-273417 PubMed DOI PMC

Efthimiou P., Paik P. K., Bielory L. (2006). Diagnosis and management of adult onset Still's disease. Ann. Rheum. Dis. 65 (5), 564–572. 10.1136/ard.2005.042143 PubMed DOI PMC

El Hasbani G., Jawad A., Uthman I. (2019). Update on the management of colchicine resistant familial mediterranean fever (FMF). Orphanet J. Rare Dis. 14 (1), 224. 10.1186/s13023-019-1201-7 PubMed DOI PMC

Emmi G., Talarico R., Lopalco G., Cimaz R., Cantini F., Viapiana O., et al. (2016). Efficacy and safety profile of anti-interleukin-1 treatment in Behçet's disease: a multicenter retrospective study. Clin. Rheumatol. 35 (5), 1281–1286. 10.1007/s10067-015-3004-0 PubMed DOI

Epçaçan S., Sahin S., Kasapcopur O. (2019). Anaphylactic reaction to anakinra in a child with steroid-dependent idiopathic recurrent pericarditis and successful management with canakinumab. Cardiol. Young 29 (4), 549–551. 10.1017/S1047951119000672 PubMed DOI

European Medicines Agency (2019a). European Medicines agency summary of product characteristics—kineret. Available at: https://www.ema.europa.eu/documents/product-information/kineret-epar-product-information_en.pdf (Accessed March 8 , 2019).

European Medicines Agency (2019b). European Medicines agency summary of product characteristics—Ilaris. Available at: https://www.ema.europa.eu/en/medicines/human/EPAR/ilaris (Accessed April 17 , 2019).

Fabiani C., Vitale A., Emmi G., Lopalco G., Vannozzi L., Guerriero S., et al. (2017). Interleukin (IL)-1 inhibition with anakinra and canakinumab in Behçet's disease-related uveitis: a multicenter retrospective observational study. Clin. Rheumatol. 36 (1), 191–197. 10.1007/s10067-016-3506-4 PubMed DOI

Fabiani C., Vitale A., Rigante D., Emmi G., Lopalco G., Di Scala G., et al. (2020). The presence of uveitis is associated with a sustained response to the interleukin (IL)-1 inhibitors anakinra and canakinumab in behçet's disease. Ocul. Immunol. Inflamm. 28 (2), 298–304. 10.1080/09273948.2018.1511810 PubMed DOI

Feist E., Mitrovic S., Fautrel B. (10-2018). Mechanisms, biomarkers and targets for adult-onset Still's disease. Nat. Rev. Rheumatol. 14 (10), 603–618. 10.1038/s41584-018-0081-x PubMed DOI PMC

Feist E., Quartier P., Fautrel B., Schneider R., Sfriso P., Efthimiou P., et al. (8-2018). Efficacy and safety of canakinumab in patients with Still's disease: exposure-response analysis of pooled systemic juvenile idiopathic arthritis data by age groups. Clin. Exp. Rheumatol. 36 (4), 668–675. PubMed

Fields J. K., Günther S., Sundberg E. J. (2019). Structural basis of IL-1 family cytokine signaling. Front. Immunol. 10, 1412. 10.3389/fimmu.2019.01412 PubMed DOI PMC

Finetti M., Insalaco A., Cantarini L., Meini A., Breda L., Alessio M., et al. (2014). Long-term efficacy of interleukin-1 receptor antagonist (anakinra) in corticosteroid-dependent and colchicine-resistant recurrent pericarditis. J. Pediatr. 164 (6), e1421–e1425. 10.1016/j.jpeds.2014.01.065 PubMed DOI

Firinu D., Garcia-Larsen V., Manconi P. E., Del Giacco S. R. (2016). SAPHO syndrome: current developments and approaches to clinical treatment. Curr. Rheumatol. Rep. 18 (6), 35. 10.1007/s11926-016-0583-y PubMed DOI

Gabay C., Fautrel B., Rech J., Spertini F., Feist E., Kötter I., et al. (2018). Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alfa (IL-18BP) in adult-onset Still's disease. Ann. Rheum. Dis. 77 (6), 840–847. 10.1136/annrheumdis-2017-212608 PubMed DOI PMC

Garlanda C., Dinarello C. A., Mantovani A. (2013). The interleukin-1 family: back to the future. Immunity 39 (6), 1003–1018. 10.1016/j.immuni.2013.11.010 PubMed DOI PMC

Gasparyan A. Y., Ayvazyan L., Blackmore H., Kitas G. D. (2011). Writing a narrative biomedical review: considerations for authors, peer reviewers, and editors. Rheumatol. Int. 31 (11), 1409–1417. 10.1007/s00296-011-1999-3 PubMed DOI

Gattorno M., Piccini A., Lasigliè D., Tassi S., Brisca G., Carta S., et al. (2008). The pattern of response to anti-interleukin-1 treatment distinguishes two subsets of patients with systemic-onset juvenile idiopathic arthritis. Arthritis Rheum. 58 (5), 1505–1515. 10.1002/art.23437 PubMed DOI

Ghosh P., Cho M., Rawat G., Simkin P. A., Gardner G. C. (2013). Treatment of acute gouty arthritis in complex hospitalized patients with anakinra. Arthritis Care Res. 65 (8), 1381–1384. 10.1002/acr.21989 PubMed DOI

Giacomelli R., Ruscitti P., Shoenfeld Y. (2018). A comprehensive review on adult onset Still's disease. J. Autoimmun. 93, 24–36. 10.1016/j.jaut.2018.07.018 PubMed DOI

Giampietro C., Ridene M., Lequerre T., Costedoat Chalumeau N., Amoura Z., Sellam J., et al. (2013). Anakinra in adult-onset Still's disease: long-term treatment in patients resistant to conventional therapy. Arthritis Care Res. 65 (5), 822–826. 10.1002/acr.21901 PubMed DOI

Gram H. (2020). The long and winding road in pharmaceutical development of canakinumab from rare genetic autoinflammatory syndromes to myocardial infarction and cancer. Pharmacol. Res. 154, 104139. 10.1016/j.phrs.2019.01.023 PubMed DOI

Grayson P. C., Yazici Y., Merideth M., Sen H. N., Davis M., Novakovich E., et al. (2017). Treatment of mucocutaneous manifestations in Behçet's disease with anakinra: a pilot open-label study. Arthritis Res. Ther. 19 (1), 69. 10.1186/s13075-017-1222-3 PubMed DOI PMC

Grom A. A., Ilowite N. T., Pascual V., Brunner H. I., Martini A., Lovell D., et al. (2016). Rate and clinical presentation of macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis treated with canakinumab, Arthritis Rheum. 68 (1), 218–228. 10.1002/art.39407 PubMed DOI

Guillaume M. P., Reumaux H., Dubos F. (2018). Usefulness and safety of anakinra in refractory Kawasaki disease complicated by coronary artery aneurysm. Cardiol. Young 28 (5), 739–742. 10.1017/S1047951117002864 PubMed DOI

Gül A., Tugal-Tutkun I., Dinarello C. A., Reznikov L., Esen B. A., Mirza A., et al. (2012). Interleukin-1β-regulating antibody XOMA 052 (gevokizumab) in the treatment of acute exacerbations of resistant uveitis of Behcet's disease: an open-label pilot study. Ann. Rheum. Dis. 71 (4), 563–566. 10.1136/annrheumdis-2011-155143 PubMed DOI

Hatemi G., Christensen R., Bang D., Bodaghi B., Celik A. F., Fortune F., et al. (2018). 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann. Rheum. Dis. 77 (6), 808–818. 10.1136/annrheumdis-2018-213225 PubMed DOI

Hatemi G., Silman A., Bang D., Bodaghi B., Chamberlain A. M., Gul A., et al. (2008). EULAR recommendations for the management of Behçet disease. Ann. Rheum. Dis. 67 (12), 1656–1662. 10.1136/ard.2007.080432 PubMed DOI

Hedrich C. M., Bruck N., Fiebig B., Gahr M. (2012). Anakinra: a safe and effective first-line treatment in systemic onset juvenile idiopathic arthritis (SoJIA). Rheumatol. Int. 32 (11), 3525–3530. 10.1007/s00296-011-2249-4 PubMed DOI

Hess J., Angel P., Schorpp-Kistner M. (2004). AP-1 subunits: quarrel and harmony among siblings. J. Cell Sci. 117 (Pt 25), 5965–5973. 10.1242/jcs.01589 PubMed DOI

Horneff G., Schulz A. C., Klotsche J., Hospach A., Minden K., Foeldvari I., et al. (2017). Experience with etanercept, tocilizumab and interleukin-1 inhibitors in systemic onset juvenile idiopathic arthritis patients from the BIKER registry. Arthritis Res. Ther. 19 (1), 256. 10.1186/s13075-017-1462-2 PubMed DOI PMC

Huang Y., Jiang H., Chen Y., Wang X., Yang Y., Tao J., et al. (2018). Tranilast directly targets NLRP3 to treat inflammasome-driven diseases. EMBO Mol. Med. 10 (4), e8689. 10.15252/emmm.201708689 PubMed DOI PMC

Iliou C., Papagoras C., Tsifetaki N., Voulgari P. V., Drosos A. A. (2013). Adult-onset Still's disease: clinical, serological and therapeutic considerations. Clin. Exp. Rheumatol. 31 (1), 47–52. PubMed

Ilowite N., Porras O., Reiff A., Rudge S., Punaro M., Martin A., et al. (2009). Anakinra in the treatment of polyarticular-course juvenile rheumatoid arthritis: safety and preliminary efficacy results of a randomized multicenter study. Clin. Rheumatol. 28 (2), 129–137. 10.1007/s10067-008-0995-9 PubMed DOI

Ilowite N. T., Prather K., Lokhnygina Y., Schanberg L. E., Elder M., Milojevic D., et al. (2014). Randomized, double-blind, placebo-controlled trial of the efficacy and safety of rilonacept in the treatment of systemic juvenile idiopathic arthritis. Arthritis Rheum. 66 (9), 2570–2579. 10.1002/art.38699 PubMed DOI PMC

Imazio M., Andreis A., De Ferrari G. M., Cremer P. C., Mardigyan V., Maestroni S., et al. (2020). Anakinra for corticosteroid-dependent and colchicine-resistant pericarditis: the IRAP (international registry of anakinra for pericarditis) study. Eur J Prev Cardiol 27 (9), 956–964. 10.1177/2047487319879534 PubMed DOI

Imazio M., Brucato A., Pluymaekers N., Breda L., Calabri G., Cantarini L., et al. (2016). Recurrent pericarditis in children and adolescents: a multicentre cohort study. J. Cardiovasc. Med. 17 (9), 707–712.10.2459/JCM.0000000000000300 PubMed DOI

International Team for the Revision of the International Criteria for Behcet's Disease (2014). The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J. Eur. Acad. Dermatol. Venereol. 28 (3), 338–347. 10.1111/jdv.12107 PubMed DOI

Iqbal S. M., Qadir S., Aslam H. M., Qadir M. A. (2019). Updated treatment for calcium pyrophosphate deposition disease: an insight. Cureus 11 (1), e3840. 10.7759/cureus.3840 PubMed DOI PMC

Janssen C. A., Oude Voshaar M. A. H., Vonkeman H. E., Jansen T., Janssen M., Kok M. R., et al. (2019). Anakinra for the treatment of acute gout flares: a randomized, double-blind, placebo-controlled, active-comparator, non-inferiority trial. Rheumatology [Epub ahead of print]. 10.1093/rheumatology/key402 PubMed DOI

Junge G., Mason J., Feist E. (2017). Adult onset Still's disease-The evidence that anti-interleukin-1 treatment is effective and well-tolerated (a comprehensive literature review). Semin. Arthritis Rheum. 47 (2), 295–302. 10.1016/j.semarthrit.2017.06.006 PubMed DOI

Kalden J. R., Schulze-Koops H. (2017). Immunogenicity and loss of response to TNF inhibitors: implications for rheumatoid arthritis treatment. Nat. Rev. Rheumatol. 13 (12), 707–718. 10.1038/nrrheum.2017.187 PubMed DOI

Kearsley-Fleet L., Beresford M. W., Davies R., De Cock D., Baildam E., Foster H. E., et al. (2019). Short-term outcomes in patients with systemic juvenile idiopathic arthritis treated with either tocilizumab or anakinra. Rheumatology 58 (1), 94–102. 10.1093/rheumatology/key262 PubMed DOI PMC

Kedor C., Listing J., Zernicke J., Weiss A., Behrens F., Blank N., et al. (2020). Canakinumab for treatment of adult-onset still's disease to achieve reduction of arthritic manifestation (CONSIDER): phase II, randomised, double-blind, placebo-controlled, multicentre, investigator-initiated trial. Ann. Rheum. Dis. 79, 1090–1097. 10.1136/annrheumdis-2020-217155 PubMed DOI PMC

Kelley N., Jeltema D., Duan Y., He Y. (2019). The NLRP3 inflammasome: an overview of mechanisms of activation and regulation. Int. J. Mol. Sci. 20 (13), 3328. 10.3390/ijms20133328 PubMed DOI PMC

Kimura Y., Grevich S., Beukelman T., Morgan E., Nigrovic P. A., Mieszkalski K., et al. (2017). Pilot study comparing the childhood arthritis & rheumatology research alliance (CARRA) systemic juvenile idiopathic arthritis Consensus treatment plans. Pediatr. Rheumatol. Online J. 15 (1), 23. 10.1186/s12969-017-0157-1 PubMed DOI PMC

Kluck V., Liu R., Joosten L. A. B. (4-2020).The role of interleukin-1 family members in hyperuricemia and gout. Joint Bone Spine, 105092. PubMed

Klück V., Jansen T. L. T. A., Janssen M., Comarniceanu A., Efdé M., Tengesdal I. W., et al. (10-2020). Dapansutrile, an oral selective NLRP3 inflammasome inhibitor, for treatment of gout flares: an open-label, dose-adaptive, proof-of-concept, phase 2a trial. Lancet Rheumatol. 2 (5), e270–e280. 10.1016/s2665-9913(20)30065-5 PubMed DOI PMC

Kone-Paut I., Cimaz R., Herberg J., Bates O., Carbasse A., Saulnier J. P., et al. (2018). The use of interleukin 1 receptor antagonist (anakinra) in Kawasaki disease: a retrospective cases series. Autoimmun. Rev. 17 (8), 768–774. 10.1016/j.autrev.2018.01.024 PubMed DOI

Kone-Paut I., Tellier S., Belot A., Brochard K., Guitton C., Marie I., et al. (2020). Open label, phase II study with anakinra in intravenous immunoglobulin-resistant Kawasaki disease. Arthritis Rheumatol. 73, 151–161. 10.1002/art.41481 PubMed DOI

Kontzias A., Efthimiou P. (2012). The use of Canakinumab, a novel IL-1β long-acting inhibitor, in refractory adult-onset Still's disease. Semin. Arthritis Rheum. 42 (2), 201–205. 10.1016/j.semarthrit.2012.03.004 PubMed DOI

Kotyla P. J. (2018). Bimodal function of anti-TNF treatment: shall we Be concerned about anti-TNF treatment in patients with rheumatoid arthritis and heart failure? Int. J. Mol. Sci. 19 (6), 1739. 10.3390/ijms19061739 PubMed DOI PMC

Kougkas N., Fanouriakis A., Papalopoulos I., Bertsias G., Avgoustidis N., Repa A., et al. (2018). Canakinumab for recurrent rheumatic disease associated-pericarditis: a case series with long-term follow-up. Rheumatology 57 (8), 1494–1495. 10.1093/rheumatology/key077 PubMed DOI

Krainer J., Siebenhandl S., Weinhäusel A. (2020). Systemic autoinflammatory diseases. J. Autoimmun. 109, 102421. 10.1016/j.jaut.2020.102421 PubMed DOI PMC

Krause K., Tsianakas A., Wagner N., Fischer J., Weller K., Metz M., et al. (2017). Efficacy and safety of canakinumab in Schnitzler syndrome: a multicenter randomized placebo-controlled study. J. Allergy Clin. Immunol. 139 (4), 1311–1320. 10.1016/j.jaci.2016.07.041 PubMed DOI

Krause K., Weller K., Stefaniak R., Wittkowski H., Altrichter S., Siebenhaar F., et al. (2012). Efficacy and safety of the interleukin-1 antagonist rilonacept in Schnitzler syndrome: an open-label study. Allergy 67 (7), 943–950. 10.1111/j.1398-9995.2012.02843.x PubMed DOI

Laskari K., Tzioufas A. G., Moutsopoulos H. M. (2011). Efficacy and long-term follow-up of IL-1R inhibitor anakinra in adults with Still's disease: a case-series study. Arthritis Res. Ther. 13 (3), R91. 10.1186/ar3366 PubMed DOI PMC

Lazaros G., Antonatou K., Vassilopoulos D. (2017). The therapeutic role of interleukin-1 inhibition in idiopathic recurrent pericarditis: current evidence and future challenges. Front. Med. 4, 78.10.3389/fmed.2017.00078 PubMed DOI PMC

Lazaros G., Vasileiou P., Koutsianas C., Antonatou K., Stefanadis C., Pectasides D., et al. (2014). Anakinra for the management of resistant idiopathic recurrent pericarditis. Initial experience in 10 adult cases. Ann. Rheum. Dis. 73 (12), 2215–2217. 10.1136/annrheumdis-2014-205990 PubMed DOI

Lee J. J. Y., Schneider R. (2018). Systemic juvenile idiopathic arthritis. Pediatr. Clin. 65 (4), 691–709. 10.1016/j.pcl.2018.04.005 PubMed DOI

Lequerré T., Quartier P., Rosellini D., Alaoui F., De Bandt M., Mejjad O., et al. (2008). Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France. Ann. Rheum. Dis. 67 (3), 302–308. 10.1136/ard.2007.076034 PubMed DOI

Liew J. W., Gardner G. C. (2019). Use of anakinra in hospitalized patients with crystal-associated arthritis. J. Rheumatol. 46 (10), 1345–1349. 10.3899/jrheum.181018 PubMed DOI

Liu T., Zhang L., Joo D., Sun S. C. (2017). NF-κB signaling in inflammation. Signal Transduct. Target Ther. 2, 17023. 10.1038/sigtrans.2017.23 PubMed DOI PMC

Lo Gullo A., Caruso A., Pipitone N., Macchioni P., Pazzola G., Salvarani C. (2014). Canakinumab in a case of adult onset still's disease: efficacy only on systemic manifestations. Joint Bone Spine 81 (4), 376–377. 10.1016/j.jbspin.2013.12.011 PubMed DOI

Lovell D. J., Giannini E. H., Reiff A. O., Kimura Y., Li S., Hashkes P. J., et al. (2013). Long-term safety and efficacy of rilonacept in patients with systemic juvenile idiopathic arthritis. Arthritis Rheum. 65 (9), 2486–2496. 10.1002/art.38042 PubMed DOI

Macmullan P., McCarthy G. (2012). Treatment and management of pseudogout: insights for the clinician. Ther. Adv. Musculoskelet. Dis. 4 (2), 121–131. 10.1177/1759720X11432559 PubMed DOI PMC

Mantovani A., Dinarello C. A., Molgora M., Garlanda C. (2019). Interleukin-1 and related cytokines in the regulation of inflammation and immunity. Immunity 50 (4), 778–795. 10.1016/j.immuni.2019.03.012 PubMed DOI PMC

Marrani E., Burns J. C., Cimaz R. (2018). How should we classify Kawasaki disease? Front. Immunol. 9, 2974. 10.3389/fimmu.2018.02974 PubMed DOI PMC

Mitha E., Schumacher H. R., Fouche L., Luo S. F., Weinstein S. P., Yancopoulos G. D., et al. (2013). Rilonacept for gout flare prevention during initiation of uric acid-lowering therapy: results from the PRESURGE-2 international, phase 3, randomized, placebo-controlled trial. Rheumatology 52 (7), 1285–1292. 10.1093/rheumatology/ket114 PubMed DOI

Murias Loza S., García-Guereta Silva L., Alcobendas Rueda R. M., Remesal Camba A. (2018). [Efficacy of anakinra as a treatment for recurrent pericarditis]. An. Pediatr. 88 (4), 223–224. 10.1016/j.anpedi.2017.05.002 PubMed DOI

Nigrovic P. A., Mannion M., Prince F. H., Zeft A., Rabinovich C. E., van Rossum M. A., et al. (2011). Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: report of forty-six patients from an international multicenter series. Arthritis Rheum. 63 (2), 545–555. 10.1002/art.30128 PubMed DOI

Nigrovic P. A. (2014). Review: is there a window of opportunity for treatment of systemic juvenile idiopathic arthritis?. Arthritis Rheum. 66 (6), 1405–1413. 10.1002/art.38615 PubMed DOI

Nirmala N., Brachat A., Feist E., Blank N., Specker C., Witt M., et al. (2015). Gene-expression analysis of adult-onset Still's disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity. Pediatr. Rheumatol. Online J. 13, 50. 10.1186/s12969-015-0047-3 PubMed DOI PMC

Nordström D., Knight A., Luukkainen R., van Vollenhoven R., Rantalaiho V., Kajalainen A., et al. (2012). Beneficial effect of interleukin 1 inhibition with anakinra in adult-onset Still's disease. An open, randomized, multicenter study. J. Rheumatol. 39 (10), 2008–2011. 10.3899/jrheum.111549 PubMed DOI

Ortiz-Sanjuán F., Blanco R., Riancho-Zarrabeitia L., Castañeda S., Olivé A., Riveros A., et al. (2015). Efficacy of anakinra in refractory adult-onset still's disease: multicenter study of 41 patients and literature review. Medicine 94 (39), e1554. 10.1097/MD.0000000000001554 PubMed DOI PMC

Ottaviani S., Moltó A., Ea H. K., Neveu S., Gill G., Brunier L., et al. (2013). Efficacy of anakinra in gouty arthritis: a retrospective study of 40 cases. Arthritis Res. Ther. 15 (5), R123. 10.1186/ar4303 PubMed DOI PMC

Owyang A. M., Issafras H., Corbin J., Ahluwalia K., Larsen P., Pongo E., et al. (2011). XOMA 052, a potent, high-affinity monoclonal antibody for the treatment of IL-1β-mediated diseases. mAbs 3 (1), 49–60. 10.4161/mabs.3.1.13989 PubMed DOI PMC

Ozen S., Kone-Paut I., Gül A. (2017). Colchicine resistance and intolerance in familial mediterranean fever: definition, causes, and alternative treatments. Semin. Arthritis Rheum. 47 (1), 115–120. 10.1016/j.semarthrit.2017.03.006 PubMed DOI

Pardeo M., Pires Marafon D., Insalaco A., Bracaglia C., Nicolai R., Messia V., et al. (2015). Anakinra in systemic juvenile idiopathic arthritis: a single-center experience. J. Rheumatol. 42 (8), 1523–1527. 10.3899/jrheum.141567 PubMed DOI

Pascual V., Allantaz F., Arce E., Punaro M., Banchereau J. (2005). Role of interleukin-1 (IL-1) in the pathogenesis of systemic onset juvenile idiopathic arthritis and clinical response to IL-1 blockade. J. Exp. Med. 201 (9), 1479–1486. 10.1084/jem.20050473 PubMed DOI PMC

Patel R. M., Shulman S. T. (2015). Kawasaki disease: a comprehensive review of treatment options. J. Clin. Pharm. Therapeut. 40 (6), 620–625. 10.1111/jcpt.12334 PubMed DOI

Picco P., Brisca G., Traverso F., Loy A., Gattorno M., Martini A. (2009). Successful treatment of idiopathic recurrent pericarditis in children with interleukin-1beta receptor antagonist (anakinra): an unrecognized autoinflammatory disease? Arthritis Rheum. 60 (1), 264–268. 10.1002/art.24174 PubMed DOI

Poetker D. M., Reh D. D. (2010). A comprehensive review of the adverse effects of systemic corticosteroids. Otolaryngol. Clin. 43 (4), 753–768. 10.1016/j.otc.2010.04.003 PubMed DOI

Principi N., Rigante D., Esposito S. (2013). The role of infection in Kawasaki syndrome. J. Infect. 67 (1), 1–10. 10.1016/j.jinf.2013.04.004 PubMed DOI PMC

Quartier P., Allantaz F., Cimaz R., Pillet P., Messiaen C., Bardin C., et al. (2011). 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). Ann. Rheum. Dis. 70 (5), 747–754. 10.1136/ard.2010.134254 PubMed DOI PMC

Quartier P., Alexeeva E., Tamas C., Chasnyk V., Wulffraat N., Palmblad K., et al. (2020). Pediatric rheumatology international trials OrganizationTapering canakinumab monotherapy in patients with systemic juvenile idiopathic arthritis in clinical remission: results from an open-label, randomized phase IIIb/IV study. Arthritis Rheum. 10.1002/art.41488 PubMed DOI PMC

Richette P., Doherty M., Pascual E., Barskova V., Becce F., Castañeda-Sanabria J., et al. (2017). 2016 updated EULAR evidence-based recommendations for the management of gout. Ann. Rheum. Dis. 76 (1), 29–42. 10.1136/annrheumdis-2016-209707 PubMed DOI

Ridker P. M., Everett B. M., Thuren T., MacFadyen J. G., Chang W. H., Ballantyne C., et al. (2017). Antiinflammatory therapy with canakinumab for atherosclerotic disease. N. Engl. J. Med. 377 (12), 1119–1131. 10.1056/NEJMoa1707914 PubMed DOI

Rigante D., Andreozzi L., Fastiggi M., Bracci B., Natale M. F., Esposito S. (2016). Critical overview of the risk scoring systems to predict non-responsiveness to intravenous immunoglobulin in Kawasaki syndrome. Int. J. Mol. Sci. 17 (3), 278. 10.3390/ijms17030278 PubMed DOI PMC

Rigante D. (2018). The broad-ranging panorama of systemic autoinflammatory disorders with specific focus on acute painful symptoms and hematologic manifestations in children. Mediterr. J. Hematol. Infect. Dis. 10 (1), e2018067. 10.4084/MJHID.2018.067 PubMed DOI PMC

Romano M., Pontikaki I., Gattinara M., Ardoino I., Donati C., Boracchi P., et al. (2014). Drug survival and reasons for discontinuation of the first course of biological therapy in 301 juvenile idiopathic arthritis patients. Reumatismo 65 (6), 278–285. 10.4081/reumatismo.2013.682 PubMed DOI

Rosenthal A. K., Ryan L. M. (2016). Calcium pyrophosphate deposition disease. N. Engl. J. Med. 374 (26), 2575–2584. 10.1056/NEJMra1511117 PubMed DOI PMC

Rossi-Semerano L., Fautrel B., Wendling D., Hachulla E., Galeotti C., Semerano L., et al. (2015). Tolerance and efficacy of off-label anti-interleukin-1 treatments in France: a nationwide survey. Orphanet. J. Rare Dis. 10, 19. 10.1186/s13023-015-0228-7 PubMed DOI PMC

Ruperto N., Brunner H. I., Quartier P., Constantin T., Wulffraat N. M., Horneff G., et al. (2018). Canakinumab in patients with systemic juvenile idiopathic arthritis and active systemic features: results from the 5-year long-term extension of the phase III pivotal trials. Ann. Rheum. Dis. 77 (12), 1710–1719. 10.1136/annrheumdis-2018-213150 PubMed DOI PMC

Ruscitti P., Ursini F., Cipriani P., De Sarro G., Giacomelli R. (2017). Biologic drugs in adult onset Still's disease: a systematic review and meta-analysis of observational studies. Expet. Rev. Clin. Immunol. 13 (11), 1089–1097. 10.1080/1744666X.2017.1375853 PubMed DOI

Ruscitti P., Ursini F., Sota J., De Giorgio R., Cantarini L., Giacomelli R. (2020). The reduction of concomitant glucocorticoids dosage following treatment with IL-1 receptor antagonist in adult onset Still's disease. A systematic review and meta-analysis of observational studies. Ther. Adv. Musculoskelet. Dis. 12, 1759720X20933133. 10.1177/1759720X20933133 PubMed DOI PMC

Sánchez-Manubens J., Bou R., Anton J. (2014). Diagnosis and classification of Kawasaki disease. J. Autoimmun. 48-49, 113–117. 10.1016/j.jaut.2014.01.010 PubMed DOI

Scardapane A., Brucato A., Chiarelli F., Breda L. (2013). Efficacy of an interleukin-1β receptor antagonist (anakinra) in idiopathic recurrent pericarditis. Pediatr. Cardiol. 34 (8), 1989–1991. 10.1007/s00246-012-0532-0 PubMed DOI

Schlesinger N., Alten R. E., Bardin T., Schumacher H. R., Bloch M., Gimona A., et al. (2012). Canakinumab for acute gouty arthritis in patients with limited treatment options: results from two randomised, multicentre, active-controlled, double-blind trials and their initial extensions. Ann. Rheum. Dis. 71 (11), 1839–1848. 10.1136/annrheumdis-2011-200908 PubMed DOI

Schlesinger N., Mysler E., Lin H. Y., De Meulemeester M., Rovensky J., Arulmani U., et al. (2011). Canakinumab reduces the risk of acute gouty arthritis flares during initiation of allopurinol treatment: results of a double-blind, randomised study. Ann. Rheum. Dis. 70 (7), 1264–1271. 10.1136/ard.2010.144063 PubMed DOI PMC

Schumacher H. R., Jr., Evans R. R., Saag K. G., Clower J., Jennings W., Weinstein S. P., et al. (2012). Rilonacept (interleukin-1 trap) for prevention of gout flares during initiation of uric acid-lowering therapy: results from a phase III randomized, double-blind, placebo-controlled, confirmatory efficacy study. Arthritis Care Res. 64 (10), 1462–1470. 10.1002/acr.21690 PubMed DOI

Scott I. C., Hajela V., Hawkins P. N., Lachmann H. J. (2011). A case series and systematic literature review of anakinra and immunosuppression in idiopathic recurrent pericarditis. J. Cardiol. Cases 4 (2), e93–e97. 10.1016/j.jccase.2011.07.003 PubMed DOI PMC

Scott I. C., Majithiya J. B., Sanden C., Thornton P., Sanders P. N., Moore T., et al. (2018). Interleukin-33 is activated by allergen- and necrosis-associated proteolytic activities to regulate its alarmin activity during epithelial damage. Sci. Rep. 8 (1), 3363. 10.1038/s41598-018-21589-2 PubMed DOI PMC

Sfriso P., Priori R., Valesini G., Rossi S., Montecucco C. M., D'Ascanio A., et al. (2016). Adult-onset Still's disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients. Clin. Rheumatol. 35 (7), 1683–1689. 10.1007/s10067-016-3308-8 PubMed DOI

Shafferman A., Birmingham J. D., Cron R. Q. (2014). High dose Anakinra for treatment of severe neonatal Kawasaki disease: a case report. Pediatr. Rheumatol. Online J. 12, 26. 10.1186/1546-0096-12-26 PubMed DOI PMC

Shenoi S., Wallace C. A. (2016). Diagnosis and treatment of systemic juvenile idiopathic arthritis. J. Pediatr. 177, 19–26. 10.1016/j.jpeds.2016.06.056 PubMed DOI

Shenoi S. (2017). Juvenile idiopathic arthritis - changing times, changing terms, changing treatments. Pediatr. Rev. 38 (5), 221–232. 10.1542/pir.2016-0148 PubMed DOI

So A., De Meulemeester M., Pikhlak A., Yücel A. E., Richard D., Murphy V., et al. (2010). Canakinumab for the treatment of acute flares in difficult-to-treat gouty arthritis: results of a multicenter, phase II, dose-ranging study. Arthritis Rheum. 62 (10), 3064–3076. 10.1002/art.27600 PubMed DOI

So A., De Smedt T., Revaz S., Tschopp J. (2007). A pilot study of IL-1 inhibition by anakinra in acute gout. Arthritis Res. Ther. 9 (2), R28. 10.1186/ar2143 PubMed DOI PMC

Sota J., Insalaco A., Cimaz R., Alessio M., Cattalini M., Gallizzi R., et al. (12-2018). Drug retention rate and predictive factors of drug survival for interleukin-1 inhibitors in systemic juvenile idiopathic arthritis. Front. Pharmacol. 9, 1526. 10.3389/fphar.2018.01526 PubMed DOI PMC

Sota J., Rigante D., Ruscitti P., Insalaco A., Sfriso P., de Vita S., et al. (2019). Anakinra drug retention rate and predictive factors of long-term response in systemic juvenile idiopathic arthritis and adult onset still disease. Front. Pharmacol. 10, 918. 10.3389/fphar.2019.00918 PubMed DOI PMC

Sota J., Vitale A., Insalaco A., Sfriso P., Lopalco G., Emmi G., et al. (8-2018). Safety profile of the interleukin-1 inhibitors anakinra and canakinumab in real-life clinical practice: a nationwide multicenter retrospective observational study. Clin. Rheumatol. 37 (8), 2233–2240. 10.1007/s10067-018-4119-x PubMed DOI

Sundy J. S., Schumacher H. R., Kivitz A., Weinstein S. P., Wu R., King-Davis S., et al. (2014). Rilonacept for gout flare prevention in patients receiving uric acid-lowering therapy: results of RESURGE, a phase III, international safety study. J. Rheumatol. 41 (8), 1703–1711. 10.3899/jrheum.131226 PubMed DOI

Szekely Y., Arbel Y. (2018). A review of interleukin-1 in heart disease: where do we stand today? Cardiol. Ther. 7 (1), 25–44. 10.1007/s40119-018-0104-3 PubMed DOI PMC

Tarp S., Amarilyo G., Foeldvari I., Christensen R., Woo J. M., Cohen N., et al. (2016). Efficacy and safety of biological agents for systemic juvenile idiopathic arthritis: a systematic review and meta-analysis of randomized trials. Rheumatology 55 (4), 669–679. 10.1093/rheumatology/kev382 PubMed DOI PMC

Terkeltaub R., Sundy J. S., Schumacher H. R., Murphy F., Bookbinder S., Biedermann S., et al. (2009). The interleukin 1 inhibitor rilonacept in treatment of chronic gouty arthritis: results of a placebo-controlled, monosequence crossover, non-randomised, single-blind pilot study. Ann. Rheum. Dis. 68 (10), 1613–1617. 10.1136/ard.2009.108936 PubMed DOI PMC

Terkeltaub R. A., Schumacher H. R., Carter J. D., Baraf H. S., Evans R. R., Wang J., et al. (2013). Rilonacept in the treatment of acute gouty arthritis: a randomized, controlled clinical trial using indomethacin as the active comparator. Arthritis Res. Ther. 15 (1), R25. 10.1186/ar4159 PubMed DOI PMC

Thueringer J. T., Doll N. K., Gertner E. (2015). Anakinra for the treatment of acute severe gout in critically ill patients. Semin. Arthritis Rheum. 45 (1), 81–85. 10.1016/j.semarthrit.2015.02.006 PubMed DOI

Tombetti E., Mulè A., Tamanini S., Matteucci L., Negro E., Brucato A., et al. (2020). Novel pharmacotherapies for recurrent pericarditis: current options in 2020. Curr. Cardiol. Rep. 22 (8), 59. 10.1007/s11886-020-01308-y PubMed DOI PMC

Tugal-Tutkun I., Pavesio C., De Cordoue A., Bernard-Poenaru O., Gül A. (2018). Use of gevokizumab in patients with behçet's disease uveitis: an international, randomized, double-masked, placebo-controlled study and open-label extension study. Ocul. Immunol. Inflamm. 26 (7), 1023–1033. 10.1080/09273948.2017.1421233 PubMed DOI

Ugurlu S., Ucar D., Seyahi E., Hatemi G., Yurdakul S. (2012). Canakinumab in a patient with juvenile Behcet's syndrome with refractory eye disease. Ann. Rheum. Dis. 71 (9), 1589–1591. 10.1136/annrheumdis-2012-201383 PubMed DOI

Vassilopoulos D., Lazaros G., Tsioufis C., Vasileiou P., Stefanadis C., Pectasides D. (2012). Successful treatment of adult patients with idiopathic recurrent pericarditis with an interleukin-1 receptor antagonist (anakinra). Int. J. Cardiol. 160 (1), 66–68. 10.1016/j.ijcard.2012.05.086 PubMed DOI

Vastert S. J., de Jager W., Noordman B. J., Holzinger D., Kuis W., Prakken B. J., et al. (2014). Effectiveness of first-line treatment with recombinant interleukin-1 receptor antagonist in steroid-naive patients with new-onset systemic juvenile idiopathic arthritis: results of a prospective cohort study. Arthritis Rheum. 66 (4), 1034–1043. 10.1002/art.38296 PubMed DOI

Vastert S. J., Jamilloux Y., Quartier P., Ohlman S., Osterling Koskinen L., Kullenberg T., et al. (2019). Anakinra in children and adults with Still's disease. Rheumatology 58 (Suppl. 6), vi9–vi22. 10.1093/rheumatology/kez350 PubMed DOI PMC

Vitale A., Berlengiero V., Sota J., Ciarcia L., Ricco N., Barneschi S., et al. (10-2020). Real-life data on the efficacy of canakinumab in patients with adult-onset still's disease. Mediat. Inflamm. 2020, 8054961. 10.1155/2020/8054961 PubMed DOI PMC

Vitale A., Cavalli G., Colafrancesco S., Priori R., Valesini G., Argolini L. M., et al. (2019). Long-term retention rate of anakinra in adult onset still's disease and predictive factors for treatment response. Front. Pharmacol. 10, 296. 10.3389/fphar.2019.00296 PubMed DOI PMC

Vitale A., Cavalli G., Ruscitti P., Sota J., Colafrancesco S., Priori R., et al. (2-2020). Comparison of early vs. Delayed anakinra treatment in patients with adult onset still's disease and effect on clinical and laboratory outcomes. Front. Med. 7, 42. 10.3389/fmed.2020.00042 PubMed DOI PMC

Vitale A., Rigante D., Caso F., Brizi M. G., Galeazzi M., Costa L., et al. (2014). Inhibition of interleukin-1 by canakinumab as a successful mono-drug strategy for the treatment of refractory Behçet's disease: a case series. Dermatology 228 (3), 211–214. 10.1159/000358125 PubMed DOI

Werman A., Werman-Venkert R., White R., Lee J. K., Werman B., Krelin Y., et al. (2004). The precursor form of IL-1alpha is an intracrine proinflammatory activator of transcription. Proc. Natl. Acad. Sci. U.S.A. 101 (8), 2434–2439. 10.1073/pnas.0308705101 PubMed DOI PMC

Williams C. J., Qazi U., Bernstein M., Charniak A., Gohr C., Mitton-Fitzgerald E., et al. (2018). Mutations in osteoprotegerin account for the CCAL1 locus in calcium pyrophosphate deposition disease. Osteoarthritis Cartilage 26 (6), 797–806.10.1016/j.joca.2018.03.005 PubMed DOI PMC

Woerner A., Uettwiller F., Melki I., Mouy R., Wouters C., Bader-Meunier B., et al. (2015). Biological treatment in systemic juvenile idiopathic arthritis: achievement of inactive disease or clinical remission on a first, second or third biological agent. RMD Open 1 (1), e000036. 10.1136/rmdopen-2014-000036 PubMed DOI PMC

Yang Y., Wang H., Kouadir M., Song H., Shi F. (2019). Recent advances in the mechanisms of NLRP3 inflammasome activation and its inhibitors. Cell Death Dis. 10 (2), 128. 10.1038/s41419-019-1413-8 PubMed DOI PMC

Yazici H., Yazici Y. (2014). Criteria for Behçet's disease with reflections on all disease criteria. J. Autoimmun. 48-49, 104–107. 10.1016/j.jaut.2014.01.014 PubMed DOI

Yazici Y. (2020). Management of Behçet syndrome. Curr. Opin. Rheumatol. 32 (1), 35–40. 10.1097/BOR.0000000000000666 PubMed DOI

Yazici Y., Yurdakul S., Yazici H. (2010). Behçet's syndrome. Curr. Rheumatol. Rep. 12 (6), 429–435. 10.1007/s11926-010-0132-z PubMed DOI

Yuan S., Akey C. W. (2013). Apoptosome structure, assembly, and procaspase activation. Structure 21 (4), 501–515. 10.1016/j.str.2013.02.024 PubMed DOI PMC

Zhang J., Macartney T., Peggie M., Cohen P. (2017). Interleukin-1 and TRAF6-dependent activation of TAK1 in the absence of TAB2 and TAB3. Biochem. J. 474 (13), 2235–2248. 10.1042/BCJ20170288 PubMed DOI PMC

Autoimmunity and hernia mesh: fact or fiction?