{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T21:49:12Z","timestamp":1791582552441,"version":"4.3.4"},"reference-count":161,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2025,1,12]],"date-time":"2025-01-12T00:00:00Z","timestamp":1736640000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Schnitzler syndrome is a unique autoinflammatory disease, of which 747 cases have been described worldwide to date. The main features of the syndrome are a triad of recurrent urticaria, monoclonal IgM gammopathy, systemic inflammation associated with recurrent fever, joint and bone pain, and atypical bone remodeling (osteosclerosis). The abnormal activation of the NLRP3 inflammasome produces IL-1, which drives the disease pathology, but it also involves IL-6 and IL-18. Unlike other autoinflammatory diseases, Schnitzler syndrome lacks evidence of the gene divergence causing the abnormal activation of NLRP3. However, mutations in the MEFV and MYD88 genes can be associated with the development of the disease. Due to its rarity, diagnosing the disease can be a challenging task. IL-1 inhibitors (i.e., anakinra, canakinumab, and rilonacept) are prominent in the treatment of the disease, but the IL-6 receptor inhibitor tocilizumab and the Bruton\u2019s tyrosine kinase inhibitor ibrutinib are also promising alternatives. In this summary article, we aim to provide a comprehensive overview of the clinical and molecular background of the disease and potential therapeutic targets, based on the cases reported so far. We diagnosed a patient who, to the best of our knowledge, represents the 748th documented case of this specific pathology. In the context of this patient, we would also like to draw attention to the potential pathogenic role of two novel gene mutations (variants of the MEFV gene \u201cc.2084A&gt;G\u201d and the F2 gene \u201c3\u2032UTR c.*97G&gt;A\u201d).<\/jats:p>","DOI":"10.3390\/ijms26020598","type":"journal-article","created":{"date-parts":[[2025,1,13]],"date-time":"2025-01-13T07:40:08Z","timestamp":1736754008000},"page":"598","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Background and Clinical Features of a Unique and Mysterious Autoinflammatory Disease, Schnitzler Syndrome"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9099-0372","authenticated-orcid":false,"given":"Gy\u00f6rgyi","family":"M\u0171zes","sequence":"first","affiliation":[{"name":"Immunology Division, Department of Internal Medicine and Hematology, Semmelweis University, 1088 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2767-7746","authenticated-orcid":false,"given":"Ferenc","family":"Sipos","sequence":"additional","affiliation":[{"name":"Immunology Division, Department of Internal Medicine and Hematology, Semmelweis University, 1088 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,1,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Braud, A., and Lipsker, D. (2024). Schnitzler Syndrome: Insights into Its Pathogenesis, Clinical Manifestations, and Current Management. Biomolecules, 14.","DOI":"10.3390\/biom14060646"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1186\/s43046-024-00237-z","article-title":"Utility of clinical, laboratory, and lymph node MYD88 L265P mutation in risk assessment of diffuse large B-cell lymphoma patients","volume":"36","author":"Hanbal","year":"2024","journal-title":"J. Egypt. Natl. Cancer Inst."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"252","DOI":"10.3960\/jslrt.24033","article-title":"MYD88 mutation-positive indolent B-cell lymphoma with CNS involvement: Bing-Neel syndrome mimickers","volume":"64","author":"Takeda","year":"2024","journal-title":"J. Clin. Exp. Hematop."},{"key":"ref_4","first-page":"e2024061","article-title":"Waldenstr\u00f6m Macroglobulinemia\u2014A State-of-the-Art Review: Part 1: Epidemiology, Pathogenesis, Clinicopathologic Characteristics, Differential Diagnosis, Risk Stratification, and Clinical Problems","volume":"16","author":"Bibas","year":"2024","journal-title":"Mediterr. J. Hematol. Infect. Dis."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1016\/j.alit.2023.02.005","article-title":"Similarities and differences in autoinflammatory diseases with urticarial rash, cryopyrin-associated periodic syndrome and Schnitzler syndrome","volume":"72","author":"Matsuda","year":"2023","journal-title":"Allergol. Int."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2121","DOI":"10.1002\/art.41030","article-title":"Exploratory study of MYD88 L265P, rare NLRP3 variants, and clonal hematopoiesis prevalence in patients with Schnitzler Syndrome","volume":"71","author":"Pathak","year":"2019","journal-title":"Arthritis Rheumatol."},{"key":"ref_7","first-page":"595","article-title":"Clonal haematopoiesis and dysregulation of the immune system. Nature reviews","volume":"23","author":"Belizaire","year":"2023","journal-title":"Immunology"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1186\/2045-7022-4-41","article-title":"Schnitzler\u2019s syndrome: Lessons from 281 cases","volume":"4","year":"2014","journal-title":"Clin. Transl. Allergy"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"75","DOI":"10.2500\/aap.2014.35.3703","article-title":"Schnitzler Syndrome","volume":"35","author":"Badawi","year":"2014","journal-title":"Allergy Asthma Proc."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.lpm.2013.06.028","article-title":"Two cases of anakinra-induced neutropenia during auto-inflammatory diseases: Drug reintroduction can be successful","volume":"43","author":"Perrin","year":"2014","journal-title":"La Presse M\u00e9dicale"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1007\/s00256-014-1857-y","article-title":"Imaging findings in 22 cases of Schnitzler syndrome: Characteristic para-articular osteosclerosis, and the \u201chot knees\u201d sign differential diagnosis","volume":"43","author":"Niederhauser","year":"2014","journal-title":"Skelet. Radiol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"111","DOI":"10.14735\/amko2014111","article-title":"Anakinra Treatment in Schnitzler Syndrome-Results of the First Retrospective Multi-center Study in Six Patients from the Czech Republic","volume":"27","author":"Sturz","year":"2014","journal-title":"Klin. Onkol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1684\/ejd.2014.2345","article-title":"Schnitzler\u2019s syndrome; a case highlighting the complications of long-standing acquired autoinflammation","volume":"24","author":"Conlon","year":"2014","journal-title":"Eur. J. Dermatol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1016\/j.autrev.2014.08.031","article-title":"Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler\u2019s syndrome: A french multicenter study","volume":"13","author":"Henry","year":"2014","journal-title":"Autoimmun. Rev."},{"key":"ref_15","first-page":"E11","article-title":"Successful treatment of Schnitzler syndrome with canakinumab","volume":"94","author":"Pesek","year":"2014","journal-title":"Cutis"},{"key":"ref_16","first-page":"229","article-title":"Hyperimmunoglobulinemia E and efficacy of elimination diet in two patients with Schnitzler syndrome","volume":"46","author":"Kolkhir","year":"2014","journal-title":"Eur. Ann. Allergy Clin. Immunol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1002\/ajh.23873","article-title":"Chronic urticarial eruption associated with monoclonal gammopathy","volume":"90","author":"Barbosa","year":"2015","journal-title":"Am. J. Hematol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.reuma.2014.07.002","article-title":"S\u00edndrome de Schnitzler","volume":"11","author":"Gallo","year":"2015","journal-title":"Reumatol. Cl\u00ednica"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1159\/000368349","article-title":"Complete Remission of Schnitzler Syndrome and Waldenstr\u00f6m Macroglobulinemia under Rituximab-Cyclophosphamide-Dexamethasone","volume":"230","author":"Aouba","year":"2014","journal-title":"Dermatology"},{"key":"ref_20","first-page":"71","article-title":"Correlative bone imaging in a case of Schnitzler\u2019s syndrome and brief review of the literature","volume":"18","author":"Willekens","year":"2015","journal-title":"Hell. J. Nucl. Med."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"428","DOI":"10.3109\/03009742.2015.1033008","article-title":"Urticarial skin rash, fever, and arthralgia: A rare case of Schnitzler\u2019s syndrome","volume":"44","author":"Thummala","year":"2015","journal-title":"Scand. J. Rheumatol."},{"key":"ref_22","first-page":"19","article-title":"Schnitzler syndrome responding to interleukin-1 antagonist anakinra","volume":"29","author":"Koch","year":"2015","journal-title":"J. Biol. Regul. Homeost. Agents"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1186\/s13075-015-0696-0","article-title":"The role of interleukin-1 beta in the pathophysiology of Schnitzler\u2019s syndrome","volume":"17","author":"Schalkwijk","year":"2015","journal-title":"Arthritis Res. Ther."},{"key":"ref_24","first-page":"V12140756","article-title":"Schnitzlers syndrom er en diagnostisk udfordring [Schnitzler syndrome is a diagnostic challenge]","volume":"177","author":"Duhn","year":"2015","journal-title":"Ugeskr. Laeger"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"521","DOI":"10.3109\/03009742.2015.1071421","article-title":"Delayed development of the IgM paraprotein in Schnitzler\u2019s syndrome","volume":"44","author":"Mulla","year":"2015","journal-title":"Scand. J. Rheumatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1002\/art.39556","article-title":"Atypical bone sclerosis","volume":"68","author":"Ricci","year":"2016","journal-title":"Arthritis Rheumatol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"436","DOI":"10.3109\/09546634.2015.1136048","article-title":"Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome","volume":"27","author":"Saulite","year":"2016","journal-title":"J. Dermatol. Treat."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"e63","DOI":"10.1016\/j.medcli.2015.12.016","article-title":"S\u00edndrome de Schnitzler: Descripci\u00f3n de 3 casos","volume":"146","author":"Iranzo","year":"2016","journal-title":"Med. Cl\u00ednica"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1007\/s13555-016-0108-7","article-title":"Schnitzler Syndrome: A dramatic response to Anakinra","volume":"6","author":"Gouveia","year":"2016","journal-title":"Dermatol. Ther."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1111\/ced.12828","article-title":"Clinical characterization and long-term follow-up of Schnitzler syndrome","volume":"41","author":"Gameiro","year":"2016","journal-title":"Clin. Exp. Dermatol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1177\/1203475416657999","article-title":"Schnitzler Syndrome without a monoclonal gammopathy","volume":"20","author":"Urbanski","year":"2016","journal-title":"J. Cutan. Med. Surg."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1447","DOI":"10.3899\/jrheum.151466","article-title":"Occurrence of Rheumatoid Arthritis in a Patient Treated with Anakinra for Schnitzler Syndrome: A Case Report","volume":"43","author":"Margerin","year":"2016","journal-title":"J. Rheumatol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"377","DOI":"10.5606\/ArchRheumatol.2016.6000","article-title":"Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler\u2019s Syndrome","volume":"31","author":"Baresic","year":"2016","journal-title":"Arch. Rheumatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1111\/all.13035","article-title":"Schnitzler syndrome: Validation and applicability of diagnostic criteria in real-life patients","volume":"72","author":"Gusdorf","year":"2016","journal-title":"Allergy"},{"key":"ref_35","first-page":"713","article-title":"Schnitzlerov\u00e9 syndromDiferenci\u00e1ln\u00ed diagnostika, p\u0159ehled l\u00e9\u010debn\u00fdch mo\u017enost\u00ed a popis 5 p\u0159\u00edpad\u016f l\u00e9\u010den\u00fdch anakinrou [Schnitzlers SyndromeDifferential diagnostics, an overview of therapeutic options and description of 5 cases treated with anakinra]","volume":"62","author":"Adam","year":"2016","journal-title":"Vnitr. Lek."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"478","DOI":"10.4274\/balkanmedj.2017.0259","article-title":"Beyond urticaria: Schnitzler Syndrome","volume":"34","year":"2017","journal-title":"Balk. Med. J."},{"key":"ref_37","first-page":"625","article-title":"Schnitzler Syndrome With Delirium and Vertigo: The Utility of Neurologic Manifestations in Diagnosis","volume":"16","author":"Tolkachjov","year":"2017","journal-title":"J. Drugs Dermatol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1016\/j.ad.2017.03.015","article-title":"S\u00edndrome de Schnitzler con respuesta a anakinra en monoterapia: 7 a\u00f1os de seguimiento","volume":"108","author":"Gonzalvo","year":"2017","journal-title":"Actas Dermo-Sifiliogr\u00e1ficas"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1016\/j.rpor.2017.08.006","article-title":"Radiotherapy for a breast cancer patient with Schnitzler syndrome: Report of acute toxicity and early follow-up","volume":"22","author":"Hanna","year":"2017","journal-title":"Rep. Pract. Oncol. Radiother."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1016\/j.jdcr.2017.06.016","article-title":"Aortitis: A new feature of Schnitzler syndrome","volume":"3","author":"Bursztejn","year":"2017","journal-title":"JAAD Case Rep."},{"key":"ref_41","first-page":"137","article-title":"Exantema urticarial refractario: S\u00edndrome de Schnitzler [Refractory urticaria: Schnitzler syndrome]","volume":"29","year":"2017","journal-title":"Emergencias"},{"key":"ref_42","first-page":"111","article-title":"Schnitzler syndrome: A review of literature, the authors\u2019 observation, and experience with the interleukin-1 inhibitor canakinumab","volume":"89","author":"Salugina","year":"2017","journal-title":"Ter. Arh."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1182\/blood-2017-10-810366","article-title":"Molecular genetic investigation, clinical features, and response to treatment in 21 patients with Schnitzler syndrome","volume":"131","author":"Rowczenio","year":"2018","journal-title":"Blood"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1018","DOI":"10.4081\/cp.2017.1018","article-title":"Schnitzler syndrome, a rare autoinflammatory disease. Complete response to IL-1 blockade","volume":"7","author":"Faggioli","year":"2017","journal-title":"Clin. Pract."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1016\/j.jaci.2016.07.041","article-title":"Efficacy and safety of canakinumab in Schnitzler syndrome: A multicenter randomized placebo-controlled study","volume":"139","author":"Krause","year":"2017","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.anai.2017.10.006","article-title":"Schnitzler syndrome with IgG gammopathy and elevated IL-1\u03b2 and IL-17 in skin biopsy","volume":"120","author":"Villarreal","year":"2018","journal-title":"Ann. Allergy Asthma Immunol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"154","DOI":"10.3349\/ymj.2018.59.1.154","article-title":"A Case of Schnitzler\u2019s Syndrome without Monoclonal Gammopathy-Associated Chronic Urticaria Treated with Anakinra","volume":"59","author":"Ahn","year":"2018","journal-title":"Yonsei Med. J."},{"key":"ref_48","first-page":"e222","article-title":"Schnitzler syndrome: A rare cause of chronic recalcitrant urticaria successfully treated with Anakinra","volume":"59","author":"Sharma","year":"2017","journal-title":"Australas. J. Dermatol."},{"key":"ref_49","doi-asserted-by":"crossref","unstructured":"Wilmas, K., Aria, A., Torres-Cabala, C.A., Lu, H., and Duvic, M. (2018). Schnitzler syndrome in a patient with a family history of monoclonal gammopathy. Dermatol. Online J., 24.","DOI":"10.5070\/D3241037926"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1200\/JOP.18.00050","article-title":"Ibrutinib for the Management of Schnitzler Syndrome: A novel therapy for a rare condition","volume":"14","author":"Jani","year":"2018","journal-title":"J. Oncol. Pract."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1016\/j.jdcr.2017.12.012","article-title":"Schnitzler syndrome associated with hairy cell leukemia presenting with chronic urticaria and arthralgias","volume":"4","author":"Fank","year":"2018","journal-title":"JAAD Case Rep."},{"key":"ref_52","first-page":"1","article-title":"A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome","volume":"2018","author":"Bashir","year":"2018","journal-title":"Case Rep. Rheumatol."},{"key":"ref_53","first-page":"372","article-title":"Urticarial eruption in a patient with intermittent fever and monoclonal IgM gammopathy","volume":"155","author":"Genovese","year":"2020","journal-title":"G. Ital. Di Dermatol. E Venereol."},{"key":"ref_54","first-page":"1","article-title":"Increasing the interval of canakinumab administration effectively supports the remission of Schnitzler\u2019s syndrome","volume":"2018","author":"Gorodetskiy","year":"2018","journal-title":"Case Rep. Rheumatol."},{"key":"ref_55","first-page":"523","article-title":"Schnitzler syndrome: A case report","volume":"39","author":"Wang","year":"2018","journal-title":"Chin. J. Hematol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"483","DOI":"10.5021\/ad.2018.30.4.483","article-title":"Schnitzler Syndrome: A case Report and Review of literature","volume":"30","author":"Kim","year":"2018","journal-title":"Ann. Dermatol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/j.retram.2018.06.001","article-title":"Schnitzler syndrome co-occurring with idiopathic multicentric Castleman disease that responds to anti-IL-1 therapy: A case report and clue to pathophysiology","volume":"66","author":"Soudet","year":"2018","journal-title":"Curr. Res. Transl. Med."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1080\/03009742.2018.1550210","article-title":"Cytokines and soluble receptors of the interleukin-1 family in Schnitzler syndrome","volume":"48","author":"Migliorini","year":"2019","journal-title":"Scand. J. Rheumatol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/j.jdcr.2019.02.002","article-title":"Schnitzler syndrome associated with MYD88 L265P mutation","volume":"5","author":"Goodman","year":"2019","journal-title":"JAAD Case Rep."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"e228546","DOI":"10.1136\/bcr-2018-228546","article-title":"Refractory urticaria and the importance of diagnosing Schnitzler\u2019s syndrome","volume":"12","author":"Nham","year":"2019","journal-title":"BMJ Case Rep."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1007\/s10875-019-00644-1","article-title":"Variable Responses to Tocilizumab in Four Patients with Schnitzler Syndrome","volume":"39","author":"Claus","year":"2019","journal-title":"J. Clin. Immunol."},{"key":"ref_62","unstructured":"Rossi, L., Casucci, F., Teutonico, A., Libutti, P., Lisi, P., Lomonte, C., Basile, C., and Manna, R. (2019). Membranoproliferative glomerulonephritis with relapsing episodes of acute kidney injury in the Schnitzler syndrome. G. Ital. Nefrol., 36."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1080\/09273948.2018.1563704","article-title":"The First Case Report of Schnitzler Syndrome Presenting with Eye Pain","volume":"28","author":"Sadig","year":"2019","journal-title":"Ocul. Immunol. Inflamm."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"229","DOI":"10.7861\/clinmed.2019-0466","article-title":"Images of the month 1: Schnitzler syndrome: An acquired autoinflammatory syndrome","volume":"20","author":"Zampeli","year":"2020","journal-title":"Clin. Med."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"3847","DOI":"10.1007\/s10067-020-05204-2","article-title":"A Chinese case series of Schnitzler syndrome and complete remission in one tocilizumab-treated patient","volume":"39","author":"Yan","year":"2020","journal-title":"Clin. Rheumatol."},{"key":"ref_66","first-page":"38","article-title":"Incomplete Schnitzler Syndrome","volume":"28","author":"Henning","year":"2020","journal-title":"Acta Dermatovenerol. Croat."},{"key":"ref_67","first-page":"553","article-title":"Le syndrome de Schnitzler [Schnitzler syndrome]","volume":"75","author":"Ilbert","year":"2020","journal-title":"Rev. Medicale De Liege"},{"key":"ref_68","first-page":"1034","article-title":"Schnitzler syndrome with basophil infiltration","volume":"18","author":"Shimokata","year":"2020","journal-title":"J. Der Dtsch. Dermatol. Ges."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"713","DOI":"10.2147\/IJGM.S265482","article-title":"Schnitzler Syndrome in a 27-Year-Old Man: Diagnostic and Therapeutic Dilemma in Adult Auto-Inflammatory Syndromes A Case Report and Literature Review","volume":"13","author":"Dziuk","year":"2020","journal-title":"Int. J. Gen. Med."},{"key":"ref_70","doi-asserted-by":"crossref","unstructured":"Hodl, I., Bosch, P., Dreo, B., and Stradner, M.H. (2020). Case report: Extensive phosphorylation of interleukin-1 Receptor-Associated kinase 4 in a patient with Schnitzler syndrome. Front. Immunol., 11.","DOI":"10.3389\/fimmu.2020.576200"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"2973","DOI":"10.1007\/s10067-020-05501-w","article-title":"Efficacy of interleukin-1 blockade in Schnitzler\u2019s syndrome without detectable monoclonal gammopathy: A case-based review","volume":"40","author":"Bixio","year":"2020","journal-title":"Clin. Rheumatol."},{"key":"ref_72","doi-asserted-by":"crossref","unstructured":"Regnault, M.M., Frouin, E., J\u00e9ru, I., Delwail, A., Charreau, S., Barbarot, S., N\u00e9el, A., Masseau, A., Pu\u00e9chal, X., and Kyndt, X. (2020). Cytokine signature in Schnitzler syndrome: Proinflammatory cytokine production associated to th suppression. Front. Immunol., 11.","DOI":"10.3389\/fimmu.2020.588322"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/j.medcli.2020.06.046","article-title":"Schnitzler\u2019s syndrome: A case report","volume":"157","author":"Ruiz","year":"2021","journal-title":"Med. Cl\u00ednica"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1106","DOI":"10.1002\/acr.24142","article-title":"A man with recurrent fever, episodic rash, and pain","volume":"73","author":"Aslam","year":"2021","journal-title":"Arthritis Care Res."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"704","DOI":"10.55563\/clinexprheumatol\/1r3i7i","article-title":"Resolution of Schnitzler\u2019s syndrome after haematopoietic stem cell transplantation","volume":"39","author":"Paladini","year":"2021","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_76","first-page":"e13338","article-title":"Schnitzler Syndrome: A recherche entity","volume":"13","author":"Kumar","year":"2021","journal-title":"Cur\u0113us"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"1380","DOI":"10.1007\/s10875-021-01038-y","article-title":"Dramatic Efficacy of Ibrutinib in a Schnitzler Syndrome Case with Indolent Lymphoma","volume":"41","author":"Claves","year":"2021","journal-title":"J. Clin. Immunol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"15","DOI":"10.36290\/vnl.2021.046","article-title":"Transformation of IgM-MGUS into Waldenstr\u00f6m\u2019s macroglobulinemia in two of six patients treated for Schnitzler\u2019s syndrome","volume":"67","author":"Adam","year":"2021","journal-title":"Vnitr. Lek."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"143","DOI":"10.14740\/jh800","article-title":"Schnitzler\u2019s Syndrome: A diagnostic consideration in evaluating the constellation of monoclonal gammopathy and Chronic urticaria","volume":"10","author":"Mamadgi","year":"2021","journal-title":"J. Hematol."},{"key":"ref_80","first-page":"e14817","article-title":"AA amyloidosis complicating monoclonal gammopathies, an unusual feature validating the concept of \u201cmonoclonal gammopathy of inflammatory significance\u201d?","volume":"75","author":"Colombat","year":"2021","journal-title":"Int. J. Clin. Pract."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"191","DOI":"10.55563\/clinexprheumatol\/mjt9x1","article-title":"Anakinra-induced psoriasis in a patient with Schnitzler\u2019s syndrome","volume":"40","year":"2022","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_82","first-page":"2011","article-title":"Retention rate of IL-1 inhibitors in Schnitzler\u2019s syndrome","volume":"40","author":"Crisafulli","year":"2021","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_83","first-page":"1","article-title":"En mann i 50-\u00e5rene med tilbakevendende urtikaria, feber og leddsmerter","volume":"142","author":"Simonsen","year":"2022","journal-title":"Tidsskr. Nor. L\u00e6geforen."},{"key":"ref_84","first-page":"S27","article-title":"A patient with urticarial lesions, recurrent fever, and IgM-type monoclonal gammopathy","volume":"31","author":"Benko","year":"2022","journal-title":"Acta Dermatovenerol. Alp. Pannonica Et Adriat."},{"key":"ref_85","doi-asserted-by":"crossref","unstructured":"Huang, Y., Wang, Y., Yu, F., Mao, X., Wang, B., Li, J., and Li, L. (2022). Case Report: Therapeutic use of ibrutinib in a patient with Schnitzler syndrome. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.894464"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"3435","DOI":"10.1111\/all.15411","article-title":"Absence of NLRP3 somatic mutations and VEXAS-related UBA1 mutations in a large cohort of patients with Schnitzler syndrome","volume":"77","author":"Louvrier","year":"2022","journal-title":"Allergy"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1111\/1756-185X.14457","article-title":"Case report: Successful treatment with tofacitinib and colchicine in a patient with Schnitzler syndrome","volume":"26","author":"Li","year":"2022","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1016\/j.alit.2022.11.004","article-title":"Summary of the current status of clinically diagnosed cases of Schnitzler syndrome in Japan","volume":"72","author":"Kambe","year":"2023","journal-title":"Allergol. Int."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.nefro.2022.02.008","article-title":"Revisiting Schnitzler syndrome: A rare severe form of acute kidney injury and monoclonal gammopathy","volume":"43","author":"Barata","year":"2023","journal-title":"Nefrolog\u00eda"},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"368","DOI":"10.31138\/mjr.33.3.368","article-title":"Fever, inflammatory response, and a persistent rash","volume":"33","author":"Daoussis","year":"2022","journal-title":"Mediterr. J. Rheumatol."},{"key":"ref_91","doi-asserted-by":"crossref","unstructured":"Bossart, S., Jafari, S.M.S., Heidemeyer, K., Yan, K., Feldmeyer, L., Borradori, L., and Yawalkar, N. (2023). Canakinumab leads to rapid reduction of neutrophilic inflammation and long-lasting response in Schnitzler syndrome. Front. Med., 10.","DOI":"10.3389\/fmed.2023.1050230"},{"key":"ref_92","doi-asserted-by":"crossref","unstructured":"Wesselmann, A.S., K\u00fcnstner, A., F\u00e4hnrich, A., Rose, C., Lamprecht, P., Busch, H., Ludwig, R.J., and Recke, A. (2023). Case report: Schnitzler-like syndrome without monoclonal gammopathy. Front. Immunol., 14.","DOI":"10.3389\/fimmu.2023.1166620"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"2085","DOI":"10.1111\/1756-185X.14718","article-title":"An unusual presentation of immunoglobulin A gammopathy in a patient with Schnitzler\u2019s syndrome","volume":"26","author":"Kaganov","year":"2023","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"e254334","DOI":"10.1136\/bcr-2022-254334","article-title":"Schnitzler syndrome","volume":"16","author":"Sankar","year":"2023","journal-title":"BMJ Case Rep."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"e1264","DOI":"10.1111\/jdv.19305","article-title":"Schnitzler syndrome associated with recurrent subacute thyroiditis and SARS-CoV-2 vaccine\u2014A case review","volume":"37","author":"Kanabaj","year":"2023","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1186\/s13223-023-00819-x","article-title":"Therapy resistant urticaria as a long-term symptom of an incomplete Schnitzler syndrome","volume":"19","author":"Puxkandl","year":"2023","journal-title":"Allergy Asthma Clin. Immunol."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"2242705","DOI":"10.1080\/09546634.2023.2242705","article-title":"Schnitzler syndrome refractory to anakinra: Successful treatment with canakinumab","volume":"34","year":"2023","journal-title":"J. Dermatol. Treat."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"100815","DOI":"10.1016\/j.waojou.2023.100815","article-title":"Proposal of a new clinical entity: Paraprotein negative IL-1 mediated inflammatory dermatosis (PANID) that may precede Schnitzler syndrome","volume":"16","author":"Fagan","year":"2023","journal-title":"World Allergy Organ. J."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1111\/bjh.19112","article-title":"Clinical and clonal characteristics of monoclonal immunoglobulin M-associated type I cryoglobulinaemia","volume":"204","author":"Khwaja","year":"2024","journal-title":"Br. J. Haematol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"e14925","DOI":"10.1111\/1756-185X.14925","article-title":"Schnitzler syndrome-An underdiagnosed adult-onset autoinflammatory disease","volume":"27","author":"Chu","year":"2024","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1111\/1346-8138.17067","article-title":"Neutrophilic epitheliotropism, proposed as an auto-inflammatory condition of neutrophilic urticarial dermatosis including Schnitzler syndrome, is also observed in Japanese cases","volume":"51","author":"Nakaizumi","year":"2024","journal-title":"J. Dermatol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"23247096231220480","DOI":"10.1177\/23247096231220480","article-title":"Clinical presentation of Schnitzler\u2019s Syndrome: A rare autoimmune disease","volume":"11","author":"Patel","year":"2024","journal-title":"J. Investig. Med. High Impact Case Rep."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.jdcr.2024.02.024","article-title":"A case of Schnitzler-like syndrome with basement membrane IgM deposition but without monoclonal gammopathy","volume":"47","author":"Ghali","year":"2024","journal-title":"JAAD Case Rep."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1007\/s42000-024-00567-6","article-title":"The co-occurrence of SAT, hypophysitis, and Schnitzler syndrome after COVID-19 vaccination: The first described case","volume":"23","author":"Szklarz","year":"2024","journal-title":"Hormones"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"T744","DOI":"10.1016\/j.ad.2024.05.006","article-title":"S\u00edndrome de Schnitzler y variante en el gen MEFV","volume":"115","year":"2024","journal-title":"Actas Dermo-Sifiliogr\u00e1ficas"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1134\/S1607672924700923","article-title":"Schnitzler\u2019s Syndrome\u2014Diagnostic Experience, Approaches to Therapy, and Patient Management according to a Multicenter Russian Cohort","volume":"517","author":"Salugina","year":"2024","journal-title":"Dokl. Biochem. Biophys."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1016\/j.abd.2023.10.004","article-title":"Mimicking urticaria: A Schnitzler syndrome case","volume":"99","author":"Cruz","year":"2024","journal-title":"An. Bras. De Dermatol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"1519","DOI":"10.1111\/1346-8138.17251","article-title":"A case of Schnitzler syndrome with unusual immunoglobulin A gammopathy exacerbated by COVID-19 infection","volume":"51","author":"Ikuno","year":"2024","journal-title":"J. Dermatol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1001\/archderm.143.8.1046","article-title":"Chronic urticaria and monoclonal IGM gammopathy (Schnitzler syndrome)","volume":"143","author":"Asli","year":"2007","journal-title":"Arch. Dermatol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/j.semarthrit.2007.04.001","article-title":"Schnitzler Syndrome: Beyond the Case Reports: Review and Follow-Up of 94 Patients with an Emphasis on Prognosis and Treatment","volume":"37","author":"Bodar","year":"2007","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1016\/j.jaci.2014.07.050","article-title":"Myeloid lineage-restricted somatic mosaicism of NLRP3 mutations in patients with variant Schnitzler syndrome","volume":"135","author":"Stoffels","year":"2015","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1016\/j.gene.2017.10.068","article-title":"Genotype-phenotype correlation in FMF patients: A \u201cnon classic\u201d recessive autosomal or \u201catypical\u201d dominant autosomal inheritance?","volume":"641","author":"Procopio","year":"2018","journal-title":"Gene"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"e82","DOI":"10.1016\/j.anpedi.2013.11.005","article-title":"Taponamiento cardiaco como forma de inicio de fiebre mediterr\u00e1nea familiar con herencia autos\u00f3mica dominante [Cardiac tamponade as first manifestation in Mediterranean fever with autosomal dominant form]","volume":"82","year":"2015","journal-title":"An. De Pediatr."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"108547","DOI":"10.1016\/j.clim.2020.108547","article-title":"Successful canakinumab treatment for activated innate response in idiopathic Castleman\u2019s disease with multiple heterozygous MEFV exon 2 variants","volume":"219","author":"Endo","year":"2020","journal-title":"Clin. Immunol."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"40","DOI":"10.4166\/kjg.2014.64.1.40","article-title":"A case of recurrent abdominal pain with fever and urticarial eruption","volume":"64","author":"Lee","year":"2014","journal-title":"Korean J. Gastroenterol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"1017","DOI":"10.2169\/internalmedicine.1401-18","article-title":"A Rare Case of Cryopyrin-associated Periodic Syndrome in an Elderly Woman with NLRP3 and MEFV Mutations","volume":"58","author":"Nakamichi","year":"2019","journal-title":"Intern. Med."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"787","DOI":"10.4049\/jimmunol.0901363","article-title":"Cutting edge: NF-kappaB activating pattern recognition and cytokine receptors license NLRP3 inflammasome activation by regulating NLRP3 expression","volume":"183","author":"Bauernfeind","year":"2009","journal-title":"J. Immunol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1038\/gene.2008.66","article-title":"Initial description of the human NLRP3 promoter","volume":"9","author":"Anderson","year":"2008","journal-title":"Genes Immun."},{"key":"ref_119","doi-asserted-by":"crossref","unstructured":"Chilton, P.M., Embry, C.A., and Mitchell, T.C. (2012). Effects of Differences in Lipid A Structure on TLR4 Pro-Inflammatory Signaling and Inflammasome Activation. Front. Immunol., 3.","DOI":"10.3389\/fimmu.2012.00154"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1186\/s13023-019-1117-2","article-title":"Schnitzler\u2019s syndrome\u2014A novel hypothesis of a shared pathophysiologic mechanism with Waldenstr\u00f6m\u2019s disease","volume":"14","author":"Potjewijd","year":"2019","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1002\/ajh.26082","article-title":"Waldenstr\u00f6m macroglobulinemia: 2021 update on diagnosis, risk stratification, and management","volume":"96","author":"Gertz","year":"2021","journal-title":"Am. J. Hematol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1038\/s41408-023-00896-6","article-title":"The oncogenic human B-cell lymphoma MYD88 L265P mutation genocopies activation by phosphorylation at the Toll\/interleukin-1 receptor (TIR) domain","volume":"13","author":"Minderman","year":"2023","journal-title":"Blood Cancer J."},{"key":"ref_123","doi-asserted-by":"crossref","unstructured":"Avbelj, M., Hafner-Bratkovi\u010d, I., Lain\u0161\u010dek, D., Man\u010dek-Keber, M., Peternelj, T.T., Panter, G., Treon, S.P., Gole, B., Poto\u010dnik, U., and Jerala, R. (2022). Cleavage-Mediated Regulation of Myd88 Signaling by Inflammasome-Activated Caspase-1. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.790258"},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1111\/bjd.17334","article-title":"Association of CCL2 with systemic inflammation in Schnitzler syndrome","volume":"180","author":"Krause","year":"2019","journal-title":"Br. J. Dermatol."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"738","DOI":"10.1038\/ni.2919","article-title":"The NLRP3 inflammasome is released as a particulate danger signal that amplifies the inflammatory response","volume":"15","author":"Gomez","year":"2014","journal-title":"Nat. Immunol."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1111\/bjd.13857","article-title":"Mast-cell interleukin-1\u03b2, neutrophil interleukin-17 and epidermal antimicrobial proteins in the neutrophilic urticarial dermatosis in Schnitzler\u2019s syndrome","volume":"173","author":"Zeeuwen","year":"2015","journal-title":"Br. J. Dermatol."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"1037","DOI":"10.1084\/jem.20082179","article-title":"Mast cells mediate neutrophil recruitment and vascular leakage through the NLRP3 inflammasome in histamine-independent urticaria","volume":"206","author":"Nakamura","year":"2009","journal-title":"J. Exp. Med."},{"key":"ref_128","doi-asserted-by":"crossref","unstructured":"Bonnekoh, H., Scheffel, J., Wu, J., Hoffmann, S., Maurer, M., and Krause, K. (2019). Skin and Systemic Inflammation in Schnitzler\u2019s Syndrome Are Associated With Neutrophil Extracellular Trap Formation. Front. Immunol., 10.","DOI":"10.3389\/fimmu.2019.00546"},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1038\/s41420-023-01787-2","article-title":"Neutrophil extracellular traps and neutrophilic dermatosis: An update review","volume":"10","author":"Li","year":"2024","journal-title":"Cell Death Discov."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"1217","DOI":"10.4049\/jimmunol.1202388","article-title":"Neutrophil extracellular trap-associated protein activation of the NLRP3 inflammasome is enhanced in lupus macrophages","volume":"190","author":"Kahlenberg","year":"2013","journal-title":"J. Immunol."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1038\/nm.3547","article-title":"Aggregated neutrophil extracellular traps limit inflammation by degrading cytokines and chemokines","volume":"20","author":"Schauer","year":"2014","journal-title":"Nat. Med."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1080\/03009740510017733","article-title":"Schnitzler\u2019s syndrome treated successfully with intravenous pulse cyclophosphamide","volume":"34","author":"Peterlana","year":"2005","journal-title":"Scand. J. Rheumatol."},{"key":"ref_133","doi-asserted-by":"crossref","unstructured":"Pathak, S., Rowczenio, D., Lara-Reyna, S., Kacar, M., Owen, R., Doody, G., Krause, K., Lachmann, H., Doffinger, R., and Newton, D. (2020). Evidence of B Cell Clonality and Investigation Into Properties of the IgM in Patients With Schnitzler Syndrome. Front. Immunol., 11.","DOI":"10.3389\/fimmu.2020.569006"},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1159\/000354194","article-title":"Impact of interleukin-6 in hematological malignancies","volume":"40","author":"Burger","year":"2013","journal-title":"Transfus. Med. Hemotherapy"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1159\/000505099","article-title":"Interleukin-6: A Masterplayer in the Cytokine Network","volume":"98","author":"Uciechowski","year":"2020","journal-title":"Oncology"},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1002\/ajh.26796","article-title":"Waldenstr\u00f6m macroglobulinemia: 2023 update on diagnosis, risk stratification, and management","volume":"98","author":"Gertz","year":"2023","journal-title":"Am. J. Hematol."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"225","DOI":"10.3816\/CLM.2005.n.004","article-title":"Autoantibody activity in Waldenstrom\u2019s macroglobulinemia","volume":"5","author":"Stone","year":"2005","journal-title":"Clin. Lymphoma"},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1097\/MD.0b013e3181943f5e","article-title":"Neutrophilic urticarial dermatosis: A variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature","volume":"88","author":"Kieffer","year":"2009","journal-title":"Medicine"},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1186\/s13075-020-02318-5","article-title":"Rheumatic involvement and bone scan features in Schnitzler syndrome: Initial and follow-up data from a single-center cohort of 25 patients","volume":"22","author":"Ansquer","year":"2020","journal-title":"Arthritis Res. Ther."},{"key":"ref_140","first-page":"838","article-title":"Chronic vasculitis urticaria associated to a monoclonal gammopathy of IgM and IgA type, a Schnitzler Syndrome?","volume":"20","author":"Carlesimo","year":"2010","journal-title":"Eur. J. Dermatol."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"1581","DOI":"10.3324\/haematol.2013.084830","article-title":"Schnitzler syndrome: An under-diagnosed clinical entity","volume":"98","author":"Jain","year":"2013","journal-title":"Haematologica"},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1046\/j.1468-3083.2002.00458.x","article-title":"Schnitzler\u2019s syndrome: What\u2019s new?","volume":"16","author":"Almerigogna","year":"2002","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"1699","DOI":"10.3324\/haematol.2012.067306","article-title":"Increased angiogenesis and enhanced bone formation in patients with IgM monoclonal gammopathy and urticarial skin rash: New insight into the biology of Schnitzler syndrome","volume":"97","author":"Terpos","year":"2012","journal-title":"Haematologica"},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"944","DOI":"10.1182\/blood-2018-01-824862","article-title":"The elusive pathogenesis of Schnitzler syndrome","volume":"131","author":"Palladini","year":"2018","journal-title":"Blood"},{"key":"ref_145","first-page":"182","article-title":"Another devastating complication of the Schnitzler syndrome: AA amyloidosis","volume":"158","author":"Claes","year":"2008","journal-title":"Br. J. Dermatol."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.anai.2011.04.006","article-title":"Spontaneous remission of Schnitzler syndrome","volume":"107","author":"Asli","year":"2011","journal-title":"Ann. Allergy Asthma Immunol."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1097\/00005792-200101000-00004","article-title":"The Schnitzler syndrome. Four new cases and review of the literature","volume":"80","author":"Lipsker","year":"2001","journal-title":"Medicine"},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1111\/all.12129","article-title":"Schnitzler\u2019s syndrome: Diagnosis, treatment, and follow-up","volume":"68","author":"Simon","year":"2013","journal-title":"Allergy"},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"2226","DOI":"10.1002\/art.21101","article-title":"Successful treatment of refractory Schnitzler syndrome with anakinra: Comment on the article by Hawkins et al","volume":"52","author":"Fontalba","year":"2005","journal-title":"Arthritis Rheum."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"947","DOI":"10.1517\/21678707.2014.939170","article-title":"Management of Schnitzler\u2019s Syndrome","volume":"2","author":"Lipsker","year":"2014","journal-title":"Expert Opin. Orphan Drugs"},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"1681","DOI":"10.1016\/j.jaci.2019.12.909","article-title":"Long-term efficacy of canakinumab in the treatment of Schnitzler syndrome","volume":"145","author":"Krause","year":"2020","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1111\/j.1398-9995.2012.02843.x","article-title":"Efficacy and safety of the interleukin-1 antagonist rilonacept in Schnitzler syndrome: An open-label study","volume":"67","author":"Krause","year":"2012","journal-title":"Allergy"},{"key":"ref_153","first-page":"2486","article-title":"Tocilizumab treatment in patients with Schnitzler syndrome: An open-label study. The journal of allergy and clinical immunology","volume":"9","author":"Bonnekoh","year":"2021","journal-title":"Practice"},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"848","DOI":"10.1016\/j.jaci.2011.10.031","article-title":"Complete remission in 3 of 3 anti-IL-6-treated patients with Schnitzler syndrome","volume":"129","author":"Krause","year":"2012","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"1054","DOI":"10.1016\/j.jaci.2017.01.017","article-title":"Human NACHT, LRR, and PYD domain-containing protein 3 (NLRP3) inflammasome activity is regulated by and potentially targetable through Bruton tyrosine kinase","volume":"140","author":"Liu","year":"2017","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"1706","DOI":"10.1007\/s10875-021-01105-4","article-title":"Killing Two Birds with One Stone: The Therapeutic Role of Ibrutinib in Schnitzler Syndrome","volume":"41","author":"Wang","year":"2021","journal-title":"J. Clin. Immunol."},{"key":"ref_157","unstructured":"National Center for Biotechnology Information (2024, November 30). ClinVar; [VCV000002547.94], Available online: https:\/\/www.ncbi.nlm.nih.gov\/clinvar\/variation\/VCV000002547.94."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"104883","DOI":"10.1016\/j.yexmp.2024.104883","article-title":"Experimental models in Familial Mediterranean Fever (FMF): Insights into pathophysiology and therapeutic strategies","volume":"135","author":"Mezher","year":"2024","journal-title":"Exp. Mol. Pathol."},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1136\/annrheumdis-2019-216701","article-title":"Blood-based test for diagnosis and functional subtyping of familial Mediterranean fever","volume":"79","author":"Huang","year":"2020","journal-title":"Ann. Rheum. Dis."},{"key":"ref_160","doi-asserted-by":"crossref","unstructured":"Li, C., Ren, H., Chen, H., Song, J., Li, S., Lee, C., Liu, J., and Cui, Y. (2017). Prothrombin G20210A (rs1799963) polymorphism increases myocardial infarction risk in an age-related manner: A systematic review and meta-analysis. Sci. Rep., 7.","DOI":"10.1038\/s41598-017-13623-6"},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1111\/j.1538-7836.2003.00493.x","article-title":"G20210A is a functional mutation in the prothrombin gene; effect on protein levels and 3\u2032-end formation","volume":"2","author":"Ceelie","year":"2004","journal-title":"J. Thromb. Haemost."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/26\/2\/598\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T10:27:22Z","timestamp":1759919242000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/26\/2\/598"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,12]]},"references-count":161,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2025,1]]}},"alternative-id":["ijms26020598"],"URL":"https:\/\/doi.org\/10.3390\/ijms26020598","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1,12]]}}}