{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T21:51:47Z","timestamp":1781646707706,"version":"3.54.5"},"reference-count":40,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T00:00:00Z","timestamp":1774483200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T00:00:00Z","timestamp":1777939200000},"content-version":"vor","delay-in-days":40,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"CAMS Innovation Fund for Medical Sciences","award":["2024-I2M-C&T-B-014"],"award-info":[{"award-number":["2024-I2M-C&T-B-014"]}]},{"name":"CAMS Innovation Fund for Medical Sciences","award":["2021-I2M-1-005, 2025-I2M-KJ-009"],"award-info":[{"award-number":["2021-I2M-1-005, 2025-I2M-KJ-009"]}]},{"name":"Open Research Fund of the State Key Laboratory of Complex, Severe, and Rare Diseases","award":["2025-O-ZD-005"],"award-info":[{"award-number":["2025-O-ZD-005"]}]},{"name":"National High Level Hospital Clinical Research Funding","award":["2022-PUMCH-D-002"],"award-info":[{"award-number":["2022-PUMCH-D-002"]}]},{"name":"National High Level Hospital Clinical Research Funding","award":["2022-PUMCH-C-025"],"award-info":[{"award-number":["2022-PUMCH-C-025"]}]},{"name":"Chinese National Key Technology R&D Program, Ministry of Science and Technology","award":["2017YFC0907601, 2017YFC0907604, 2019YFC08040603"],"award-info":[{"award-number":["2017YFC0907601, 2017YFC0907604, 2019YFC08040603"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arthritis Res Ther"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and objectives<\/jats:title>\n                    <jats:p>\n                      Blau syndrome (BS) is a rare autosomal dominant autoinflammatory disease with limited treatment options, driven by the nucleotide-binding oligomerization domain containing 2 (\n                      <jats:italic>NOD2<\/jats:italic>\n                      ) mutations that constitutively activate RIP2-mediated nuclear factor-\u03baB (NF-\u03baB) and mitogen-activated protein kinases (MAPKs) signaling. We have reported the potent anti-inflammatory activity of carboxyamidotriazole (CAI), originally developed as a non-cytotoxic anticancer drug. In this study, we investigated the therapeutic potential and possible mechanism of CAI for BS.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Peripheral blood mononuclear cells (PBMCs) were isolated from patients with BS. Various BS-specific cellular models and animal models were established. Immunoblot, immunofluorescence and immunohistochemistry were utilized to examine the NOD2-mediated inflammatory signaling pathways. NF-\u03baB activity was measured with a dual-luciferase reporter assay system, and cytokines were detected by ELISA.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>In BS patient-derived PBMCs, CAI did not affect RIP2 activation under unstimulated conditions or following stimulation with muramyl dipeptide (MDP) plus lipopolysaccharide (LPS), but it suppressed NF-\u03baB activation by reducing IKK phosphorylation and p65 nuclear translocation, ultimately leading to a marked attenuation of pro-inflammatory cytokines production. In cellular models, CAI inhibited MDP-induced activation of both NF-\u03baB and MAPKs pathways in RAW264.7 cells, as well as the production of cytokines triggered by MDP alone or in combination with LPS. This anti-inflammatory activity was also confirmed in immortalized bone marrow-derived macrophage (iBMDM) and THP-1 cell models. Furthermore, CAI effectively suppressed constitutive NF-\u03baB activity driven by two prevalent BS-associated NOD2 mutants (R334W and R334Q) in HEK293T cells. Finally, in animal models, CAI treatment effectively alleviated L18-MDP-induced systemic inflammation in a mouse BS model. In a rat model of BS-associated uveitis, CAI\u2019s efficacy was further validated by its ability to ameliorate ocular pathological damage, inhibit the overactivation of NF-\u03baB and MAPKs, and decrease the levels of pro-inflammatory cytokines in ocular tissues.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our findings suggest that CAI may have therapeutic value in BS by targeting the core pathogenic NF-\u03baB\/MAPK signaling axes and reducing cytokines production, which highlights CAI as a new potential medication for BS.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13075-026-03800-2","type":"journal-article","created":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T04:04:16Z","timestamp":1774497856000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Inactivation of NF-\u03baB and MAPKs confers the potent therapeutic effect of carboxyamidotriazole on Blau syndrome"],"prefix":"10.1186","volume":"28","author":[{"given":"Haoxin","family":"Song","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yang","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuxin","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengyuan","family":"Duan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Na","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu","family":"Du","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ru","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Quanlin","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Shen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fang","family":"Wei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lei","family":"Zhu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,3,26]]},"reference":[{"issue":"11","key":"3800_CR1","doi-asserted-by":"publisher","first-page":"1492","DOI":"10.1136\/annrheumdis-2020-217320","volume":"79","author":"T Matsuda","year":"2020","unstructured":"Matsuda T, Kambe N, Ueki Y, Kanazawa N, Izawa K, Honda Y, et al. Clinical characteristics and treatment of 50 cases of Blau syndrome in Japan confirmed by genetic analysis of the NOD2 mutation. Ann Rheum Dis. 2020;79(11):1492\u20139.","journal-title":"Ann Rheum Dis"},{"issue":"21","key":"3800_CR2","doi-asserted-by":"publisher","first-page":"2153","DOI":"10.1056\/NEJMra071714","volume":"357","author":"MC Iannuzzi","year":"2007","unstructured":"Iannuzzi MC, Rybicki BA, Teirstein AS, Sarcoidosis. N Engl J Med. 2007;357(21):2153\u201365.","journal-title":"N Engl J Med"},{"issue":"2","key":"3800_CR3","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1007\/s11882-021-00991-3","volume":"21","author":"KP Kaufman","year":"2021","unstructured":"Kaufman KP, Becker ML. Distinguishing Blau syndrome from systemic sarcoidosis. Curr Allergy Asthma Rep. 2021;21(2):10.","journal-title":"Curr Allergy Asthma Rep"},{"issue":"1","key":"3800_CR4","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1038\/ng720","volume":"29","author":"C Miceli-Richard","year":"2001","unstructured":"Miceli-Richard C, Lesage S, Rybojad M, Prieur AM, Manouvrier-Hanu S, H\u00e4fner R, et al. CARD15 mutations in Blau syndrome. Nat Genet. 2001;29(1):19\u201320.","journal-title":"Nat Genet"},{"issue":"3","key":"3800_CR5","doi-asserted-by":"publisher","first-page":"237","DOI":"10.1111\/imm.12677","volume":"150","author":"CL Feerick","year":"2017","unstructured":"Feerick CL, McKernan DP. Understanding the regulation of pattern recognition receptors in inflammatory diseases - a \u2018Nod\u2019 in the right direction. Immunology. 2017;150(3):237\u201347.","journal-title":"Immunology"},{"issue":"5","key":"3800_CR6","doi-asserted-by":"publisher","first-page":"769","DOI":"10.1016\/j.immuni.2011.04.013","volume":"34","author":"YG Kim","year":"2011","unstructured":"Kim YG, Kamada N, Shaw MH, Warner N, Chen GY, Franchi L, et al. The Nod2 sensor promotes intestinal pathogen eradication via the chemokine CCL2-dependent recruitment of inflammatory monocytes. Immunity. 2011;34(5):769\u201380.","journal-title":"Immunity"},{"issue":"1","key":"3800_CR7","doi-asserted-by":"publisher","first-page":"4043","DOI":"10.1038\/s41467-018-06451-3","volume":"9","author":"E Pellegrini","year":"2018","unstructured":"Pellegrini E, Desfosses A, Wallmann A, Schulze WM, Rehbein K, Mas P, et al. RIP2 filament formation is required for NOD2 dependent NF-\u03baB signalling. Nat Commun. 2018;9(1):4043.","journal-title":"Nat Commun"},{"issue":"1","key":"3800_CR8","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1007\/s10067-017-3758-7","volume":"37","author":"D Wu","year":"2018","unstructured":"Wu D, Shen M. Two Chinese pedigrees of Blau syndrome with thirteen affected members. Clin Rheumatol. 2018;37(1):265\u201370.","journal-title":"Clin Rheumatol"},{"issue":"6","key":"3800_CR9","doi-asserted-by":"publisher","first-page":"1008","DOI":"10.1093\/rheumatology\/keu437","volume":"54","author":"CD Ros\u00e9","year":"2015","unstructured":"Ros\u00e9 CD, Pans S, Casteels I, Anton J, Bader-Meunier B, Brissaud P, et al. Blau syndrome: cross-sectional data from a multicentre study of clinical, radiological and functional outcomes. Rheumatology (Oxford). 2015;54(6):1008\u201316.","journal-title":"Rheumatology (Oxford)"},{"issue":"1","key":"3800_CR10","doi-asserted-by":"publisher","first-page":"44","DOI":"10.1016\/j.autrev.2012.07.028","volume":"12","author":"P Sfriso","year":"2012","unstructured":"Sfriso P, Caso F, Tognon S, Galozzi P, Gava A, Punzi L. Blau syndrome, clinical and genetic aspects. Autoimmun Rev. 2012;12(1):44\u201351.","journal-title":"Autoimmun Rev"},{"issue":"3","key":"3800_CR11","doi-asserted-by":"publisher","first-page":"1195","DOI":"10.1182\/blood-2004-07-2972","volume":"105","author":"N Kanazawa","year":"2005","unstructured":"Kanazawa N, Okafuji I, Kambe N, Nishikomori R, Nakata-Hizume M, Nagai S, et al. Early-onset sarcoidosis and CARD15 mutations with constitutive nuclear factor-kappaB activation: common genetic etiology with Blau syndrome. Blood. 2005;105(3):1195\u20137.","journal-title":"Blood"},{"issue":"4","key":"3800_CR12","doi-asserted-by":"publisher","first-page":"536","DOI":"10.3899\/jrheum.160672","volume":"44","author":"T Nagakura","year":"2017","unstructured":"Nagakura T, Wakiguchi H, Kubota T, Yamatou T, Yamasaki Y, Nonaka Y, et al. Tumor necrosis factor inhibitors provide longterm clinical benefits in pediatric and young adult patients with Blau syndrome. J Rheumatol. 2017;44(4):536\u20138.","journal-title":"J Rheumatol"},{"issue":"5","key":"3800_CR13","doi-asserted-by":"publisher","first-page":"727","DOI":"10.1016\/j.semarthrit.2017.09.010","volume":"47","author":"L Lu","year":"2018","unstructured":"Lu L, Shen M, Jiang D, Li Y, Zheng X, Li Y, et al. Blau syndrome with good responses to tocilizumab: a case report and focused literature review. Semin Arthritis Rheum. 2018;47(5):727\u201331.","journal-title":"Semin Arthritis Rheum"},{"issue":"1","key":"3800_CR14","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s00431-023-05204-9","volume":"183","author":"H Lassoued Ferjani","year":"2024","unstructured":"Lassoued Ferjani H, Kharrat L, Ben Nessib D, Kaffel D, Maatallah K, Hamdi W. Management of Blau syndrome: review and proposal of a treatment algorithm. Eur J Pediatr. 2024;183(1):1\u20137.","journal-title":"Eur J Pediatr"},{"issue":"4","key":"3800_CR15","first-page":"935","volume":"54","author":"EC Kohn","year":"1994","unstructured":"Kohn EC, Felder CC, Jacobs W, Holmes KA, Day A, Freer R, et al. Structure-function analysis of signal and growth inhibition by carboxyamido-triazole, CAI. Cancer Res. 1994;54(4):935\u201342.","journal-title":"Cancer Res"},{"issue":"3","key":"3800_CR16","doi-asserted-by":"publisher","first-page":"279","DOI":"10.1016\/S0169-5002(02)00525-1","volume":"39","author":"TW Moody","year":"2003","unstructured":"Moody TW, Chiles J, Moody E, Sieczkiewicz GJ, Kohn EC. CAI inhibits the growth of small cell lung cancer cells. Lung Cancer. 2003;39(3):279\u201388.","journal-title":"Lung Cancer"},{"issue":"6","key":"3800_CR17","first-page":"911","volume":"43","author":"Y He","year":"2024","unstructured":"He Y, Zhu L. Inhibitory effect of carboxyamidotriazole on the proliferation of human eosinophil EOL-1. Herald Med. 2024;43(6):911\u20135.","journal-title":"Herald Med"},{"issue":"13","key":"3800_CR18","doi-asserted-by":"publisher","first-page":"3446","DOI":"10.1111\/bph.13138","volume":"172","author":"L Zhu","year":"2015","unstructured":"Zhu L, Li J, Guo L, Yu X, Wu D, Luo L, et al. Activation of NALP1 inflammasomes in rats with adjuvant arthritis; a novel therapeutic target of carboxyamidotriazole in a model of rheumatoid arthritis. Br J Pharmacol. 2015;172(13):3446\u201359.","journal-title":"Br J Pharmacol"},{"key":"3800_CR19","doi-asserted-by":"publisher","first-page":"16","DOI":"10.1016\/j.intimp.2017.01.015","volume":"45","author":"X Du","year":"2017","unstructured":"Du X, Chen W, Wang Y, Chen C, Guo L, Ju R, et al. Therapeutic efficacy of carboxyamidotriazole on 2,4,6-trinitrobenzene sulfonic acid-induced colitis model is associated with the inhibition of NLRP3 inflammasome and NF-\u03baB activation. Int Immunopharmacol. 2017;45:16\u201325.","journal-title":"Int Immunopharmacol"},{"issue":"9","key":"3800_CR20","doi-asserted-by":"publisher","first-page":"e20105","DOI":"10.1016\/j.heliyon.2023.e20105","volume":"9","author":"Y Zhou","year":"2023","unstructured":"Zhou Y, Yang X, Liu J, Yang M, Ye C, Zhu L. Carboxyamidotriazole alleviates pannus formation and cartilage erosion in rats with adjuvant arthritis. Heliyon. 2023;9(9):e20105.","journal-title":"Heliyon"},{"issue":"6","key":"3800_CR21","doi-asserted-by":"publisher","first-page":"1635","DOI":"10.1007\/s10067-022-06443-1","volume":"42","author":"M Duan","year":"2023","unstructured":"Duan M, Shen M, Zhou Y, He Y, Guo Z, Ye C, et al. AIM2 and NLRC4-driven inflammasome activation in adult-onset Still\u2019s disease and the preliminary therapeutic effect exploration of carboxyamidotriazole. Clin Rheumatol. 2023;42(6):1635\u201343.","journal-title":"Clin Rheumatol"},{"issue":"10","key":"3800_CR22","first-page":"1502","volume":"42","author":"M Duan","year":"2023","unstructured":"Duan M, Shen M, He Y, Ye C, Zhu L. Therapeutic effect of carboxyamidotriazole on IL-1\u03b2-mediated autoinflammatory dseases. Herald Med. 2023;42(10):1502\u20137.","journal-title":"Herald Med"},{"issue":"11","key":"3800_CR23","first-page":"3085","volume":"59","author":"J Liu","year":"2024","unstructured":"Liu J, Yang M, Zhu L. Carboxyamidotriazole ameliorates experimental psoriasis via downregulating the expressions of cytokines and antimicrobial peptide S100A7. Acta Pharm Sinica. 2024;59(11):3085\u201393.","journal-title":"Acta Pharm Sinica"},{"key":"3800_CR24","doi-asserted-by":"publisher","first-page":"123440","DOI":"10.1016\/j.lfs.2025.123440","volume":"364","author":"M Yang","year":"2025","unstructured":"Yang M, Lu S, Li J, Zhu L, Carboxyaminotriazole. A bone savior in collagen-induced arthritis-Halting osteoclastogenesis via interleukin-1\u03b2 downregulation. Life Sci. 2025;364:123440.","journal-title":"Life Sci"},{"issue":"10","key":"3800_CR25","doi-asserted-by":"publisher","first-page":"2362","DOI":"10.7150\/ijms.111897","volume":"22","author":"X Zhang","year":"2025","unstructured":"Zhang X, Liu J, Yang M, Li J, Zhu L. Carboxyamidotriazole regulates the function of salivary gland epithelial cells and B cells to alleviate experimental Sj\u00f6gren\u2019s disease in mice. Int J Med Sci. 2025;22(10):2362\u201372.","journal-title":"Int J Med Sci"},{"issue":"2","key":"3800_CR26","doi-asserted-by":"publisher","first-page":"1455","DOI":"10.3892\/etm.2020.8889","volume":"20","author":"S Lu","year":"2020","unstructured":"Lu S, Duan M, Guo Z, Zhou Y, Wu D, Zhang X, et al. Carboxyamidotriazole exerts anti-inflammatory activity in lipopolysaccharide-induced RAW264.7 macrophages by inhibiting NF-\u03baB and MAPKs pathways. Exp Ther Med. 2020;20(2):1455\u201366.","journal-title":"Exp Ther Med"},{"issue":"1","key":"3800_CR27","doi-asserted-by":"publisher","first-page":"236","DOI":"10.1186\/s13075-019-2017-5","volume":"21","author":"J Chen","year":"2019","unstructured":"Chen J, Luo Y, Zhao M, Wu D, Yang Y, Zhang W, et al. Effective treatment of TNF\u03b1 inhibitors in Chinese patients with Blau syndrome. Arthritis Res Ther. 2019;21(1):236.","journal-title":"Arthritis Res Ther"},{"issue":"3","key":"3800_CR28","first-page":"406","volume":"42","author":"H Song","year":"2022","unstructured":"Song H, Ye C, Zhu L. Establishment of cell models for Blau syndrome. Basic Clin Med. 2022;42(3):406\u201310.","journal-title":"Basic Clin Med"},{"issue":"6","key":"3800_CR29","first-page":"1195","volume":"39","author":"H Song","year":"2023","unstructured":"Song H, Yang X, Ye C, Zhu L. Establishment of animal model of Blau syndrome. Chin Pharmacol Bull. 2023;39(6):1195\u20139.","journal-title":"Chin Pharmacol Bull"},{"key":"3800_CR30","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1007\/978-1-60761-720-4_22","volume":"900","author":"RK Agarwal","year":"2012","unstructured":"Agarwal RK, Silver PB, Caspi RR. Rodent models of experimental autoimmune uveitis. Methods Mol Biol. 2012;900:443\u201369.","journal-title":"Methods Mol Biol"},{"issue":"4","key":"3800_CR31","first-page":"899","volume":"58","author":"X Yang","year":"2023","unstructured":"Yang X, Ye C, Zhu L. Research progress of NOD2-mediated signaling pathways and relationship with autoinflammatory diseases and its inhibitors. Acta Pharm Sinica. 2023;58(4):899\u2013908.","journal-title":"Acta Pharm Sinica"},{"issue":"1","key":"3800_CR32","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1080\/09273948.2016.1185529","volume":"26","author":"N Ebrahimiadib","year":"2018","unstructured":"Ebrahimiadib N, Samra KA, Domina AM, Stiles ER, Ewer R, Bocian CP, et al. A Novel NOD2-associated mutation and variant Blau syndrome: phenotype and molecular analysis. Ocul Immunol Inflamm. 2018;26(1):57\u201364.","journal-title":"Ocul Immunol Inflamm"},{"issue":"6","key":"3800_CR33","doi-asserted-by":"publisher","first-page":"3455","DOI":"10.1073\/pnas.0530276100","volume":"100","author":"M Chamaillard","year":"2003","unstructured":"Chamaillard M, Philpott D, Girardin SE, Zouali H, Lesage S, Chareyre F, et al. Gene-environment interaction modulated by allelic heterogeneity in inflammatory diseases. Proc Natl Acad Sci U S A. 2003;100(6):3455\u201360.","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"1","key":"3800_CR34","doi-asserted-by":"publisher","first-page":"250","DOI":"10.1002\/art.25035","volume":"62","author":"K Yasui","year":"2010","unstructured":"Yasui K, Yashiro M, Tsuge M, Manki A, Takemoto K, Yamamoto M, et al. Thalidomide dramatically improves the symptoms of early-onset sarcoidosis\/Blau syndrome: its possible action and mechanism. Arthritis Rheum. 2010;62(1):250\u20137.","journal-title":"Arthritis Rheum"},{"issue":"11","key":"3800_CR35","doi-asserted-by":"publisher","first-page":"3805","DOI":"10.1002\/art.22966","volume":"56","author":"JI Ar\u00f3stegui","year":"2007","unstructured":"Ar\u00f3stegui JI, Arnal C, Merino R, Modesto C, Antonia Carballo M, Moreno P, et al. NOD2 gene-associated pediatric granulomatous arthritis: clinical diversity, novel and recurrent mutations, and evidence of clinical improvement with interleukin-1 blockade in a Spanish cohort. Arthritis Rheum. 2007;56(11):3805\u201313.","journal-title":"Arthritis Rheum"},{"issue":"10","key":"3800_CR36","doi-asserted-by":"publisher","first-page":"2040","DOI":"10.1016\/S0161-6420(03)00717-6","volume":"110","author":"T Kurokawa","year":"2003","unstructured":"Kurokawa T, Kikuchi T, Ohta K, Imai H, Yoshimura N. Ocular manifestations in Blau syndrome associated with a CARD15\/Nod2 mutation. Ophthalmology. 2003;110(10):2040\u20134.","journal-title":"Ophthalmology"},{"issue":"6","key":"3800_CR37","doi-asserted-by":"publisher","first-page":"532","DOI":"10.1097\/ICU.0000000000000705","volume":"31","author":"S Suresh","year":"2020","unstructured":"Suresh S, Tsui E. Ocular manifestations of Blau syndrome. Curr Opin Ophthalmol. 2020;31(6):532\u20137.","journal-title":"Curr Opin Ophthalmol"},{"key":"3800_CR38","doi-asserted-by":"publisher","first-page":"103401","DOI":"10.1016\/j.jaut.2025.103401","volume":"153","author":"I Maccora","year":"2025","unstructured":"Maccora I, Wouters C, Ros\u00e8 CD, Maniscalco V, de Masi S, Mastrolia MV, et al. Treatment of uveitis in Blau syndrome: a systematic review and meta-analysis. J Autoimmun. 2025;153:103401.","journal-title":"J Autoimmun"},{"issue":"11","key":"3800_CR39","first-page":"1004","volume":"57","author":"Chinese Society of Dermatology, China Dermatologist Association","year":"2024","unstructured":"Chinese Society of Dermatology, China Dermatologist Association. Expert consensus on diagnosis and treatment of Blau syndrome (2024). Chin J Dermatology. 2024;57(11):1004\u201310.","journal-title":"Chin J Dermatology"},{"issue":"50","key":"3800_CR40","doi-asserted-by":"publisher","first-page":"36223","DOI":"10.1074\/jbc.M703079200","volume":"282","author":"Y Yang","year":"2007","unstructured":"Yang Y, Yin C, Pandey A, Abbott D, Sassettic C, Kelliher MA. NOD2 pathway activation by MDP or Mycobacterium tuberculosis infection involves the stable polyubiquitination of Rip2. J Biol Chem. 2007;282(50):36223\u20139.","journal-title":"J Biol Chem"}],"container-title":["Arthritis Research &amp; Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13075-026-03800-2","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-026-03800-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-026-03800-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T20:53:19Z","timestamp":1781643199000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1186\/s13075-026-03800-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,26]]},"references-count":40,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["3800"],"URL":"https:\/\/doi.org\/10.1186\/s13075-026-03800-2","relation":{},"ISSN":["1478-6362"],"issn-type":[{"value":"1478-6362","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,26]]},"assertion":[{"value":"23 November 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 March 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"26 March 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by Institutional Review Board of Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences (002-2018). All patients (parent in the case of child) provided informed consent to participate in this study.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"105"}}