{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T01:52:59Z","timestamp":1785462779464,"version":"3.56.0"},"reference-count":55,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T00:00:00Z","timestamp":1648512000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T00:00:00Z","timestamp":1648512000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Rheumatol"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Objective<\/jats:title>\n                <jats:p>The aim of the present study was to assess the clinical characteristic of hypocomplementemia (HC) in primary Sjogren\u2019s syndrome (pSS), and to address possible risk factors and the prognosis associated with HC in pSS patients.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>pSS patients with HC in Hebei General Hospital from September 2016 to March 2019 were retrospectively analyzed and compared to those with normocomplementemia (NC). Logistic regression analysis was used to detect risk factors.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Of the 333 patients with pSS, 84 patients (25.23%) were presented with HC at diagnosis. The presence of hyper-IgG and anti-Ro52 antibodies was significantly more common in patients with HC. In addition to systemic involvement, pSS patients with HC had more hematological, renal, and nervous system involvement, and received more immunosuppressant treatments than NC group (<jats:italic>p<\/jats:italic> &lt; 0.05). ESSDAI score was significantly higher in patients with HC (<jats:italic>p<\/jats:italic> &lt; 0.05). Multivariate logistic analysis indicated that leukopenia (OR = 2.23) and hyper-IgG (OR = 2.13) were independent risk factors for pSS with HC. In addition, profound CD16\/CD56+ NK-cell lymphopenia was found in pSS-HC patients. More pSS patients developed SLE in the HC group than NC group (4.76% vs. 0.80%, <jats:italic>p<\/jats:italic> = 0.04) during the follow-up.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>HC was not an uncommon manifestation of pSS and had an independent association with the main clinical and immunological features. Patients with pSS-HC had an increased possibility to develop SLE that required more positive treatment with glucocorticoids and immunosuppressants.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Key Points:<\/jats:title>\n                <jats:p>\u2022 <jats:italic>Hypocomplementemia had an independent association with the main clinical and immunological features in primary Sjogren\u2019s syndrome patients.<\/jats:italic><\/jats:p>\n                <jats:p>\u2022 <jats:italic>ESSDAI score was significantly higher in patients with hypocomplementemia.<\/jats:italic><\/jats:p>\n                <jats:p>\u2022 <jats:italic> The pSS patients with hypocomplementemia had an increased possibility to develop SLE.<\/jats:italic><\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s10067-022-06135-w","type":"journal-article","created":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T08:07:07Z","timestamp":1648541227000},"page":"2091-2102","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Hypocomplementemia in primary Sjogren\u2019s syndrome: association with serological, clinical features, and outcome"],"prefix":"10.1007","volume":"41","author":[{"given":"Wei","family":"Lin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhifei","family":"Xin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jialan","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiuying","family":"Ren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yixuan","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liu","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaoying","family":"Guo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yupeng","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yang","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingjing","family":"Cao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoran","family":"Ning","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meilu","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yashuang","family":"Su","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lijun","family":"Sun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5366-2708","authenticated-orcid":false,"given":"Fengxiao","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,3,29]]},"reference":[{"key":"6135_CR1","doi-asserted-by":"publisher","first-page":"695","DOI":"10.1016\/j.autrev.2016.03.004","volume":"15","author":"AV Goules","year":"2016","unstructured":"Goules AV, Tzioufas AG (2016) Primary Sj\u04e7gren's syndrome: clinical phenotypes, outcome and the development of biomarkers. Autoimmun Rev 15:695\u2013703. https:\/\/doi.org\/10.1016\/j.autrev.2016.03.004","journal-title":"Autoimmun Rev"},{"key":"6135_CR2","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1016\/j.jdsr.2019.09.006","volume":"55","author":"HM Ibrahem","year":"2019","unstructured":"Ibrahem HM (2019) B cell dysregulation in primary Sj\u00f6gren's syndrome: a review. Jpn Dental Sci Rev 55:139\u2013144. https:\/\/doi.org\/10.1016\/j.jdsr.2019.09.006","journal-title":"Jpn Dental Sci Rev"},{"key":"6135_CR3","doi-asserted-by":"publisher","first-page":"R118","DOI":"10.1186\/ar4571","volume":"16","author":"W Lin","year":"2014","unstructured":"Lin W, Jin L, Chen H, Wu Q, Fei Y, Zheng W, Wang Q, Li P, Li Y, Zhang W, Zhao Y, Zeng X, Zhang F (2014) B cell subsets and dysfunction of regulatory B cells in IgG4-related diseases and primary Sj\u00f6gren's syndrome: the similarities and differences. Arthritis Res Ther 16:R118. https:\/\/doi.org\/10.1186\/ar4571","journal-title":"Arthritis Res Ther"},{"key":"6135_CR4","doi-asserted-by":"publisher","first-page":"e475","DOI":"10.1016\/j.lpm.2012.05.021","volume":"41","author":"N Hamza","year":"2012","unstructured":"Hamza N, Bos NA, Kallenberg CG (2012) B-cell populations and sub-populations in Sj\u00f6gren's syndrome. Presse Medicale (Paris, France : 1983) 41:e475\u2013e483. https:\/\/doi.org\/10.1016\/j.lpm.2012.05.021","journal-title":"Presse Medicale (Paris, France : 1983)"},{"key":"6135_CR5","doi-asserted-by":"publisher","first-page":"2299","DOI":"10.3390\/jcm9072299","volume":"9","author":"D Parisis","year":"2020","unstructured":"Parisis D, Chivasso C, Perret J, Soyfoo MS, Delporte C (2020) Current state of knowledge on Primary Sj\u00f6gren's Syndrome, an autoimmune exocrinopathy. J Clin Med 9:2299. https:\/\/doi.org\/10.3390\/jcm9072299","journal-title":"J Clin Med"},{"key":"6135_CR6","doi-asserted-by":"publisher","first-page":"133","DOI":"10.1038\/nrrheum.2018.1","volume":"14","author":"G Nocturne","year":"2018","unstructured":"Nocturne G, Mariette X (2018) B cells in the pathogenesis of primary Sj\u00f6gren syndrome. Nat Rev Rheumatol 14:133\u2013145. https:\/\/doi.org\/10.1038\/nrrheum.2018.1","journal-title":"Nat Rev Rheumatol"},{"key":"6135_CR7","doi-asserted-by":"publisher","unstructured":"Wei L, Zhifei X, Xiaoran N, Meilu L, Yang L, Yixuan L, Xiuying R, Yashuang S, Jingjing C, Shaoying G, Liu Y, Lijun S, Fengxiao ZWen Z (2021) Patients with early-onset primary Sj\u00f6gren's syndrome have distinctive clinical manifestations and circulating lymphocyte profiles. Rheumatology (Oxford, England). https:\/\/doi.org\/10.1093\/rheumatology\/keab367","DOI":"10.1093\/rheumatology\/keab367"},{"key":"6135_CR8","doi-asserted-by":"publisher","unstructured":"Lin W, Xin Z, Ning X, Li Y, Ren X, Su Y, Liu M, Guo S, Yang L, Liu Y, Zhang F, Zhang W (2021) Clinical features and risk factors of Raynaud's phenomenon in primary Sj\u00f6gren's syndrome. Clin Rheumatol. https:\/\/doi.org\/10.1007\/s10067-021-05749-w","DOI":"10.1007\/s10067-021-05749-w"},{"key":"6135_CR9","doi-asserted-by":"publisher","first-page":"1554","DOI":"10.1002\/acr.22859","volume":"68","author":"AN Baer","year":"2016","unstructured":"Baer AN, Medrano L, McAdams De Marco MG, Niadek TJ (2016) Association of anticentromere antibodies with more severe exocrine glandular dysfunction in Sj\u00f6gren's syndrome: analysis of the Sj\u00f6gren's International Collaborative Clinical Alliance Cohort. Arthritis Care Res 68:1554\u20131559. https:\/\/doi.org\/10.1002\/acr.22859","journal-title":"Arthritis Care Res"},{"key":"6135_CR10","doi-asserted-by":"publisher","first-page":"210","DOI":"10.1097\/MD.0b013e318181e6af","volume":"87","author":"M Ramos-Casals","year":"2008","unstructured":"Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino-Montes J, Calvo-Alen J, Jim\u00e9nez-Alonso J, Mic\u00f3 ML, Beltr\u00e1n J, Belenguer R, Pallar\u00e9s L (2008) Primary Sj\u00f6gren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine 87:210\u2013219. https:\/\/doi.org\/10.1097\/MD.0b013e318181e6af","journal-title":"Medicine"},{"key":"6135_CR11","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1159\/000024423","volume":"123","author":"CP Mavragani","year":"2000","unstructured":"Mavragani CP, Tzioufas AG, Moutsopoulos HM (2000) Sj\u00f6gren's syndrome: autoantibodies to cellular antigens. Clinical and molecular aspects. Int Arch Allergy Immunol 123:46\u201357. https:\/\/doi.org\/10.1159\/000024423","journal-title":"Int Arch Allergy Immunol"},{"key":"6135_CR12","doi-asserted-by":"publisher","first-page":"1164","DOI":"10.1093\/rheumatology\/keq047","volume":"49","author":"AL Fauchais","year":"2010","unstructured":"Fauchais AL, Ouattara B, Gondran G, Lallou\u00e9 F, Petit D, Ly K, Lambert M, Launay D, Loustaud-Ratti V, Bezanahari H, Liozon E, Hachulla E, Jauberteau MO, Vidal EHatron PY (2010) Articular manifestations in primary Sj\u00f6gren's syndrome: clinical significance and prognosis of 188 patients. Rheumatology (Oxford, England) 49:1164\u20131172. https:\/\/doi.org\/10.1093\/rheumatology\/keq047","journal-title":"Rheumatology (Oxford, England)"},{"key":"6135_CR13","doi-asserted-by":"publisher","first-page":"929","DOI":"10.1111\/1756-185x.13464","volume":"22","author":"M Tsukamoto","year":"2019","unstructured":"Tsukamoto M, Suzuki K, Takeuchi T (2019) Ten-year observation of patients with primary Sj\u00f6gren's syndrome: initial presenting characteristics and the associated outcomes. Int J Rheum Dis 22:929\u2013933. https:\/\/doi.org\/10.1111\/1756-185x.13464","journal-title":"Int J Rheum Dis"},{"key":"6135_CR14","doi-asserted-by":"publisher","first-page":"875","DOI":"10.1111\/1756-185x.13070","volume":"20","author":"EJ Ter Borg","year":"2017","unstructured":"Ter Borg EJ, Kelder JC (2017) Is extra-glandular organ damage in primary Sj\u00f6gren's syndrome related to the presence of systemic auto-antibodies and\/or hypergammaglobulinemia? A long-term cohort study with 110 patients from the Netherlands. Int J Rheum Dis 20:875\u2013881. https:\/\/doi.org\/10.1111\/1756-185x.13070","journal-title":"Int J Rheum Dis"},{"key":"6135_CR15","doi-asserted-by":"publisher","first-page":"284","DOI":"10.1097\/MD.0b013e3181b76ab5","volume":"88","author":"E Baimpa","year":"2009","unstructured":"Baimpa E, Dahabreh IJ, Voulgarelis M, Moutsopoulos HM (2009) Hematologic manifestations and predictors of lymphoma development in primary Sj\u00f6gren syndrome: clinical and pathophysiologic aspects. Medicine 88:284\u2013293. https:\/\/doi.org\/10.1097\/MD.0b013e3181b76ab5","journal-title":"Medicine"},{"key":"6135_CR16","doi-asserted-by":"publisher","first-page":"641","DOI":"10.1016\/j.autrev.2015.03.004","volume":"14","author":"A Papageorgiou","year":"2015","unstructured":"Papageorgiou A, Voulgarelis M, Tzioufas AG (2015) Clinical picture, outcome and predictive factors of lymphoma in Sj\u04e7gren syndrome. Autoimmun Rev 14:641\u2013649. https:\/\/doi.org\/10.1016\/j.autrev.2015.03.004","journal-title":"Autoimmun Rev"},{"key":"6135_CR17","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1016\/j.semarthrit.2011.04.006","volume":"41","author":"R Solans-Laqu\u00e9","year":"2011","unstructured":"Solans-Laqu\u00e9 R, L\u00f3pez-Hernandez A, Bosch-Gil JA, Palacios A, Campillo M, Vilardell-Tarres M (2011) Risk, predictors, and clinical characteristics of lymphoma development in primary Sj\u00f6gren's syndrome. Semin Arthritis Rheum 41:415\u2013423. https:\/\/doi.org\/10.1016\/j.semarthrit.2011.04.006","journal-title":"Semin Arthritis Rheum"},{"key":"6135_CR18","doi-asserted-by":"publisher","first-page":"554","DOI":"10.1136\/ard.61.6.554","volume":"61","author":"C Vitali","year":"2002","unstructured":"Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH (2002) Classification criteria for Sj\u00f6gren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61:554\u2013558. https:\/\/doi.org\/10.1136\/ard.61.6.554","journal-title":"Ann Rheum Dis"},{"key":"6135_CR19","doi-asserted-by":"publisher","first-page":"859","DOI":"10.1136\/annrheumdis-2013-204615","volume":"74","author":"R Seror","year":"2015","unstructured":"Seror R, Theander E, Brun JG, Ramos-Casals M, Valim V, D\u00f6rner T, Bootsma H, Tzioufas A, Solans-Laqu\u00e9 R, Mandl T, Gottenberg JE, Hachulla E, Sivils KL, Ng WF, Fauchais AL, Bombardieri S, Valesini G, Bartoloni E, Saraux A et al (2015) Validation of EULAR primary Sj\u00f6gren's syndrome disease activity (ESSDAI) and patient indexes (ESSPRI). Ann Rheum Dis 74:859\u2013866. https:\/\/doi.org\/10.1136\/annrheumdis-2013-204615","journal-title":"Ann Rheum Dis"},{"key":"6135_CR20","doi-asserted-by":"publisher","first-page":"1103","DOI":"10.1136\/ard.2009.110619","volume":"69","author":"R Seror","year":"2010","unstructured":"Seror R, Ravaud P, Bowman SJ, Baron G, Tzioufas A, Theander E, Gottenberg JE, Bootsma H, Mariette X, Vitali C (2010) EULAR Sjogren's syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren's syndrome. Ann Rheum Dis 69:1103\u20131109. https:\/\/doi.org\/10.1136\/ard.2009.110619","journal-title":"Ann Rheum Dis"},{"key":"6135_CR21","doi-asserted-by":"publisher","first-page":"382","DOI":"10.1136\/annrheumdis-2014-206008","volume":"75","author":"R Seror","year":"2016","unstructured":"Seror R, Bootsma H, Saraux A, Bowman SJ, Theander E, Brun JG, Baron G, Le Guern V, Devauchelle-Pensec V, Ramos-Casals M, Valim V, Dorner T, Tzioufas A, Gottenberg JE, Solans Laque R, Mandl T, Hachulla E, Sivils KL, Ng WF et al (2016) Defining disease activity states and clinically meaningful improvement in primary Sjogren's syndrome with EULAR primary Sjogren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI). Ann Rheum Dis 75:382\u2013389. https:\/\/doi.org\/10.1136\/annrheumdis-2014-206008","journal-title":"Ann Rheum Dis"},{"key":"6135_CR22","doi-asserted-by":"publisher","first-page":"839","DOI":"10.1093\/rheumatology\/ket427","volume":"53","author":"C Baldini","year":"2014","unstructured":"Baldini C, Pepe P, Quartuccio L, Priori R, Bartoloni E, Alunno A, Gattamelata A, Maset M, Modesti M, Tavoni A, De Vita S, Gerli R, Valesini G, Bombardieri S (2014) Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology (Oxford, England) 53:839\u2013844. https:\/\/doi.org\/10.1093\/rheumatology\/ket427","journal-title":"Rheumatology (Oxford, England)"},{"key":"6135_CR23","doi-asserted-by":"publisher","first-page":"112","DOI":"10.5152\/eurjrheum.2020.19132","volume":"7","author":"P Jord\u00e1n-Gonz\u00e1lez","year":"2020","unstructured":"Jord\u00e1n-Gonz\u00e1lez P, Gago-Pi\u00f1ero R, Varela-Rosario N, P\u00e9rez-R\u00edos N, Vil\u00e1 LM (2020) Characterization of a subset of patients with primary Sj\u00f6gren's syndrome initially presenting with C3 or C4 hypocomplementemia. Eur J Rheumatol 7:112\u2013117. https:\/\/doi.org\/10.5152\/eurjrheum.2020.19132","journal-title":"Eur J Rheumatol"},{"key":"6135_CR24","first-page":"3252","volume":"123","author":"DF Lin","year":"2010","unstructured":"Lin DF, Yan SM, Zhao Y, Zhang W, Li MT, Zeng XF, Zhang FC, Dong Y (2010) Clinical and prognostic characteristics of 573 cases of primary Sj\u00f6gren's syndrome. Chin Med J 123:3252\u20133257","journal-title":"Chin Med J"},{"key":"6135_CR25","first-page":"102","volume":"36","author":"P Brito-Zer\u00f3n","year":"2018","unstructured":"Brito-Zer\u00f3n P, Acar-Denizli N, Ng WF, Zeher M, Rasmussen A, Mandl T, Seror R, Li X, Baldini C, Gottenberg JE, Danda D, Quartuccio L, Priori R, Hernandez-Molina G, Armagan B, Kruize AA, Kwok SK, Kvarnstr\u00f6m M, Praprotnik S et al (2018) How immunological profile drives clinical phenotype of primary Sj\u00f6gren's syndrome at diagnosis: analysis of 10,500 patients (Sj\u00f6gren Big Data Project). Clin Exp Rheumatol 36:102\u2013112","journal-title":"Clin Exp Rheumatol"},{"key":"6135_CR26","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1093\/rheumatology\/ket349","volume":"53","author":"M Ramos-Casals","year":"2014","unstructured":"Ramos-Casals M, Brito-Zeron P, Solans R, Camps MT, Casanovas A, Sopena B, Diaz-Lopez B, Rascon FJ, Qanneta R, Fraile G, Perez-Alvarez R, Callejas JL, Ripoll M, Pinilla B, Akasbi M, Fonseca E, Canora J, Nadal ME, de la Red G et al (2014) Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology (Oxford) 53:321\u2013331. https:\/\/doi.org\/10.1093\/rheumatology\/ket349","journal-title":"Rheumatology (Oxford)"},{"key":"6135_CR27","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1093\/rheumatology\/kev354","volume":"55","author":"AG Singh","year":"2016","unstructured":"Singh AG, Singh S, Matteson EL (2016) Rate, risk factors and causes of mortality in patients with Sjogren's syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford) 55:450\u2013460. https:\/\/doi.org\/10.1093\/rheumatology\/kev354","journal-title":"Rheumatology (Oxford)"},{"key":"6135_CR28","doi-asserted-by":"publisher","first-page":"374","DOI":"10.1111\/j.1365-3083.2009.02229.x","volume":"69","author":"AF Zadura","year":"2009","unstructured":"Zadura AF, Theander E, Blom AM, Trouw LA (2009) Complement inhibitor C4b-binding protein in primary Sj\u00f6gren's syndrome and its association with other disease markers. Scand J Immunol 69:374\u2013380. https:\/\/doi.org\/10.1111\/j.1365-3083.2009.02229.x","journal-title":"Scand J Immunol"},{"key":"6135_CR29","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1007\/s00296-013-2885-y","volume":"34","author":"G Sudzius","year":"2014","unstructured":"Sudzius G, Mieliauskaite D, Siaurys A, Viliene R, Butrimiene I, Characiejus D, Dumalakiene I (2014) Could the complement component C4 or its fragment C4d be a marker of the more severe conditions in patients with primary Sj\u00f6gren's syndrome? Rheumatol Int 34:235\u2013241. https:\/\/doi.org\/10.1007\/s00296-013-2885-y","journal-title":"Rheumatol Int"},{"key":"6135_CR30","doi-asserted-by":"publisher","first-page":"2","DOI":"10.1186\/s12891-015-0858-x","volume":"17","author":"RD Evans","year":"2016","unstructured":"Evans RD, Laing CM, Ciurtin C, Walsh SB (2016) Tubulointerstitial nephritis in primary Sj\u00f6gren syndrome: clinical manifestations and response to treatment. BMC Musculoskelet Disord 17:2. https:\/\/doi.org\/10.1186\/s12891-015-0858-x","journal-title":"BMC Musculoskelet Disord"},{"key":"6135_CR31","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1186\/s12882-019-1341-y","volume":"20","author":"W Xia","year":"2019","unstructured":"Xia W, Gao B, Duan L, Li Y, Wen Y, Chen L, Li X, Zheng F, Li M (2019) Clinical significance of C4d deposition in renal tissues from patients with primary Sj\u00f6gren's syndrome-a preliminary study. BMC Nephrol 20:189. https:\/\/doi.org\/10.1186\/s12882-019-1341-y","journal-title":"BMC Nephrol"},{"key":"6135_CR32","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1016\/j.imbio.2014.03.018","volume":"219","author":"X Min","year":"2014","unstructured":"Min X, Liu C, Wei Y, Wang N, Yuan G, Liu D, Li Z, Zhou W, Li K (2014) Expression and regulation of complement receptors by human natural killer cells. Immunobiology 219:671\u2013679. https:\/\/doi.org\/10.1016\/j.imbio.2014.03.018","journal-title":"Immunobiology"},{"key":"6135_CR33","doi-asserted-by":"publisher","first-page":"3952392","DOI":"10.1155\/2019\/3952392","volume":"2019","author":"J Luo","year":"2019","unstructured":"Luo J, Huo YW, Wang JW, Guo H (2019) High-risk indicators of renal involvement in Primary Sjogren's Syndrome: a clinical study of 1002 cases. J Immunol Res 2019:3952392. https:\/\/doi.org\/10.1155\/2019\/3952392","journal-title":"J Immunol Res"},{"key":"6135_CR34","doi-asserted-by":"publisher","first-page":"182","DOI":"10.1111\/1756-185x.13429","volume":"22","author":"J Luo","year":"2019","unstructured":"Luo J, Xu S, Lv Y, Huang X, Zhang H, Zhu X, Wang X (2019) Clinical features and potential relevant factors of renal involvement in primary Sj\u00f6gren's syndrome. Int J Rheum Dis 22:182\u2013190. https:\/\/doi.org\/10.1111\/1756-185x.13429","journal-title":"Int J Rheum Dis"},{"key":"6135_CR35","doi-asserted-by":"publisher","first-page":"e667","DOI":"10.1097\/md.0000000000000667","volume":"94","author":"Y Zhao","year":"2015","unstructured":"Zhao Y, Li Y, Wang L, Li XF, Huang CB, Wang GC, Zhang XW, Zhang ZL, Zhang X, Xiao WG, Dai L, Wang YF, Hu SX, Li HB, Gong L, Liu B, Sun LY, Zhang MJ, Zhang X et al (2015) Primary Sj\u00f6gren syndrome in Han Chinese: clinical and immunological characteristics of 483 patients. Medicine 94:e667. https:\/\/doi.org\/10.1097\/md.0000000000000667","journal-title":"Medicine"},{"key":"6135_CR36","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1111\/1756-185x.13182","volume":"21","author":"HX Yang","year":"2018","unstructured":"Yang HX, Wang J, Wen YB, Fei YY, Jiang MD, Zhou MY, Zhang W, Li H, Li XM, Zhang FC, Li XW, Zhang X, Chen LM (2018) Renal involvement in primary Sj\u00f6gren's syndrome: a retrospective study of 103 biopsy-proven cases from a single center in China. Int J Rheum Dis 21:223\u2013229. https:\/\/doi.org\/10.1111\/1756-185x.13182","journal-title":"Int J Rheum Dis"},{"key":"6135_CR37","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1186\/s12868-018-0427-y","volume":"19","author":"W Ye","year":"2018","unstructured":"Ye W, Chen S, Huang X, Qin W, Zhang T, Zhu X, Zhu X, Lin C, Wang X (2018) Clinical features and risk factors of neurological involvement in Sj\u00f6gren's syndrome. BMC Neurosci 19:26. https:\/\/doi.org\/10.1186\/s12868-018-0427-y","journal-title":"BMC Neurosci"},{"key":"6135_CR38","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1186\/1742-2094-9-137","volume":"9","author":"FH Brennan","year":"2012","unstructured":"Brennan FH, Anderson AJ, Taylor SM, Woodruff TM, Ruitenberg MJ (2012) Complement activation in the injured central nervous system: another dual-edged sword? J Neuroinflammation 9:137. https:\/\/doi.org\/10.1186\/1742-2094-9-137","journal-title":"J Neuroinflammation"},{"key":"6135_CR39","doi-asserted-by":"publisher","first-page":"362","DOI":"10.3389\/fimmu.2019.00362","volume":"10","author":"SM Carpanini","year":"2019","unstructured":"Carpanini SM, Torvell M, Morgan BP (2019) Therapeutic inhibition of the complement system in diseases of the central nervous system. Front Immunol 10:362. https:\/\/doi.org\/10.3389\/fimmu.2019.00362","journal-title":"Front Immunol"},{"key":"6135_CR40","doi-asserted-by":"publisher","first-page":"2021","DOI":"10.3389\/fimmu.2020.02021","volume":"11","author":"K Mallah","year":"2020","unstructured":"Mallah K, Couch C, Borucki DM, Toutonji A, Alshareef M, Tomlinson S (2020) Anti-inflammatory and neuroprotective agents in clinical trials for CNS disease and injury: where do we go from here? Front Immunol 11:2021. https:\/\/doi.org\/10.3389\/fimmu.2020.02021","journal-title":"Front Immunol"},{"key":"6135_CR41","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1111\/1756-185x.12088","volume":"16","author":"Y Yang","year":"2013","unstructured":"Yang Y, Li Z, Wang L, Zhang F (2013) The clinical and laboratory characteristics of Sj\u00f6gren's syndrome that progresses to systemic lupus erythematosus: a retrospective case-control study. Int J Rheum Dis 16:173\u2013177. https:\/\/doi.org\/10.1111\/1756-185x.12088","journal-title":"Int J Rheum Dis"},{"key":"6135_CR42","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1111\/1756-185X.12088","volume":"16","author":"Y Yang","year":"2013","unstructured":"Yang Y, Li Z, Wang L, Zhang F (2013) The clinical and laboratory characteristics of Sj\u00f6gren's syndrome that progresses to systemic lupus erythematosus: a retrospective case-control study. Int J Rheum Dis 16:173\u2013177","journal-title":"Int J Rheum Dis"},{"key":"6135_CR43","doi-asserted-by":"publisher","first-page":"212","DOI":"10.7326\/0003-4819-92-2-212","volume":"92","author":"HMCT Moutsopoulos","year":"1980","unstructured":"Moutsopoulos HMCT, Mann DL, Klippel JH, Fauci AS, Frank MM, Lawley TJ, Hamburger MI (1980) Sj\u00f6gren's syndrome (Sicca syndrome): current issues. Ann Intern Med 92:212\u2013226","journal-title":"Ann Intern Med"},{"key":"6135_CR44","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1177\/096120339500400106","volume":"4","author":"P Zufferey","year":"1995","unstructured":"Zufferey P, Meyer OC, Bourgeois P, Vayssairat M, Kahn MF (1995) Primary systemic Sj\u00f6gren syndrome (SS) preceding systemic lupus erythematosus: a retrospective study of 4 cases in a cohort of 55 SS patients. Lupus 4:23\u201327","journal-title":"Lupus"},{"key":"6135_CR45","doi-asserted-by":"publisher","first-page":"882","DOI":"10.1002\/art.20093","volume":"50","author":"MN Manoussakis","year":"2004","unstructured":"Manoussakis MN, Georgopoulou C, Zintzaras E, Spyropoulou M, Stavropoulou A, Skopouli FN, Moutsopoulos HM (2004) Sjogren's syndrome associated with systemic lupus erythematosus: clinical and laboratory profiles and comparison with primary Sjogren's syndrome. Arthritis Rheum 50:882\u2013891. https:\/\/doi.org\/10.1002\/art.20093","journal-title":"Arthritis Rheum"},{"key":"6135_CR46","doi-asserted-by":"publisher","first-page":"28","DOI":"10.1097\/RHU.0b013e31823ecbdf","volume":"18","author":"Q Yao","year":"2012","unstructured":"Yao Q, Altman RD, Wang X (2012) Systemic lupus erythematosus with Sjogren syndrome compared to systemic lupus erythematosus alone: a meta-analysis. J Clin Rheumatol 18:28\u201332. https:\/\/doi.org\/10.1097\/RHU.0b013e31823ecbdf","journal-title":"J Clin Rheumatol"},{"key":"6135_CR47","doi-asserted-by":"publisher","first-page":"1053","DOI":"10.1016\/j.autrev.2018.05.005","volume":"17","author":"K Psianou","year":"2018","unstructured":"Psianou K, Panagoulias I, Papanastasiou AD, de Lastic AL, Rodi M, Spantidea PI, Degn SE, Georgiou P, Mouzaki A (2018) Clinical and immunological parameters of Sjogren's syndrome. Autoimmun Rev 17:1053\u20131064. https:\/\/doi.org\/10.1016\/j.autrev.2018.05.005","journal-title":"Autoimmun Rev"},{"key":"6135_CR48","doi-asserted-by":"publisher","first-page":"298506","DOI":"10.1155\/2015\/298506","volume":"2015","author":"R Aggarwal","year":"2015","unstructured":"Aggarwal R, Anaya JM, Koelsch KA, Kurien BT, Scofield RH (2015) Association between secondary and primary Sjogren's syndrome in a large collection of lupus families. Autoimmune Dis 2015:298506. https:\/\/doi.org\/10.1155\/2015\/298506","journal-title":"Autoimmune Dis"},{"key":"6135_CR49","doi-asserted-by":"publisher","first-page":"33","DOI":"10.2147\/OARRR.S167783","volume":"11","author":"SG Pasoto","year":"2019","unstructured":"Pasoto SG, Adriano de Oliveira Martins V, Bonfa E (2019) Sjogren's syndrome and systemic lupus erythematosus: links and risks. Open Access Rheumatol 11:33\u201345. https:\/\/doi.org\/10.2147\/OARRR.S167783","journal-title":"Open Access Rheumatol"},{"key":"6135_CR50","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1016\/j.molimm.2004.03.017","volume":"41","author":"MC Carroll","year":"2004","unstructured":"Carroll MC (2004) The complement system in B cell regulation. Mol Immunol 41:141\u2013146. https:\/\/doi.org\/10.1016\/j.molimm.2004.03.017","journal-title":"Mol Immunol"},{"key":"6135_CR51","doi-asserted-by":"publisher","first-page":"157","DOI":"10.1111\/j.1600-065X.2007.00556.x","volume":"219","author":"R Roozendaal","year":"2007","unstructured":"Roozendaal R, Carroll MC (2007) Complement receptors CD21 and CD35 in humoral immunity. Immunol Rev 219:157\u2013166. https:\/\/doi.org\/10.1111\/j.1600-065X.2007.00556.x","journal-title":"Immunol Rev"},{"key":"6135_CR52","doi-asserted-by":"publisher","first-page":"1262","DOI":"10.1002\/art.20176","volume":"50","author":"E Theander","year":"2004","unstructured":"Theander E, Manthorpe R, Jacobsson LT (2004) Mortality and causes of death in primary Sj\u00f6gren's syndrome: a prospective cohort study. Arthritis Rheum 50:1262\u20131269. https:\/\/doi.org\/10.1002\/art.20176","journal-title":"Arthritis Rheum"},{"key":"6135_CR53","doi-asserted-by":"publisher","first-page":"639","DOI":"10.3881\/j.issn.1000-503X.2014.06.015","volume":"36","author":"XH Shi","year":"2014","unstructured":"Shi XH, Ma J, Li C, Wen YB, Li H, Li MX, Li XW, Li XM (2014) Clinical features of 30 patients with cryoglobulinemia. Zhongguo yi xue ke xue yuan xue bao. Acta Acad Med Sin 36:639\u2013644. https:\/\/doi.org\/10.3881\/j.issn.1000-503X.2014.06.015","journal-title":"Acta Acad Med Sin"},{"key":"6135_CR54","doi-asserted-by":"publisher","first-page":"467","DOI":"10.1136\/ard.60.5.467","volume":"60","author":"M Pertovaara","year":"2001","unstructured":"Pertovaara M, Pukkala E, Laippala P, Miettinen A, Pasternack A (2001) A longitudinal cohort study of Finnish patients with primary Sj\u00f6gren's syndrome: clinical, immunological, and epidemiological aspects. Ann Rheum Dis 60:467\u2013472. https:\/\/doi.org\/10.1136\/ard.60.5.467","journal-title":"Ann Rheum Dis"},{"key":"6135_CR55","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1093\/rheumatology\/kei107","volume":"45","author":"Y Alamanos","year":"2006","unstructured":"Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, Siozos C, Drosos AA (2006) Epidemiology of primary Sj\u00f6gren's syndrome in north-west Greece, 1982-2003. Rheumatology (Oxford, England) 45:187\u2013191. https:\/\/doi.org\/10.1093\/rheumatology\/kei107","journal-title":"Rheumatology (Oxford, England)"}],"container-title":["Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-022-06135-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10067-022-06135-w\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-022-06135-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,10]],"date-time":"2022-06-10T11:15:23Z","timestamp":1654859723000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10067-022-06135-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3,29]]},"references-count":55,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2022,7]]}},"alternative-id":["6135"],"URL":"https:\/\/doi.org\/10.1007\/s10067-022-06135-w","relation":{},"ISSN":["0770-3198","1434-9949"],"issn-type":[{"value":"0770-3198","type":"print"},{"value":"1434-9949","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,3,29]]},"assertion":[{"value":"9 November 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 February 2022","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 March 2022","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 March 2022","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"None.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Disclosures"}}]}}