{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,21]],"date-time":"2026-02-21T09:58:28Z","timestamp":1771667908168,"version":"3.50.1"},"reference-count":103,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2020,2,1]],"date-time":"2020-02-01T00:00:00Z","timestamp":1580515200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,2,1]],"date-time":"2020-02-01T00:00:00Z","timestamp":1580515200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,1,9]],"date-time":"2020-01-09T00:00:00Z","timestamp":1578528000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100007054","name":"Meso Scale Diagnostics","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007054","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004337","name":"Roche","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004337","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004325","name":"AstraZeneca","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004325","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008349","name":"Boehringer Ingelheim","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008349","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004312","name":"Eli Lilly and Company","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004312","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Best Practice & Research Clinical Rheumatology"],"published-print":{"date-parts":[[2020,2]]},"DOI":"10.1016\/j.berh.2020.101485","type":"journal-article","created":{"date-parts":[[2020,2,15]],"date-time":"2020-02-15T02:34:58Z","timestamp":1581734098000},"page":"101485","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":18,"title":["Targeted therapies in systemic sclerosis, myositis, antiphospholipid syndrome, and Sj\u00f6gren's syndrome"],"prefix":"10.1016","volume":"34","author":[{"given":"Lucas L.","family":"van den Hoogen","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacob M.","family":"van Laar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.berh.2020.101485_bib1","doi-asserted-by":"crossref","first-page":"1685","DOI":"10.1016\/S0140-6736(17)30933-9","article-title":"Systemic sclerosis","volume":"390","author":"Denton","year":"2017","journal-title":"Lancet"},{"key":"10.1016\/j.berh.2020.101485_bib2","doi-asserted-by":"crossref","first-page":"2490","DOI":"10.1001\/jama.2014.6368","article-title":"Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial","volume":"311","author":"Van Laar","year":"2014","journal-title":"JAMA, J Am Med Assoc"},{"key":"10.1016\/j.berh.2020.101485_bib3","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.imlet.2018.01.002","article-title":"Pathogenic roles of B lymphocytes in systemic sclerosis","volume":"195","author":"Yoshizaki","year":"2018","journal-title":"Immunol Lett"},{"key":"10.1016\/j.berh.2020.101485_bib4","doi-asserted-by":"crossref","first-page":"582","DOI":"10.1016\/j.autrev.2017.12.010","article-title":"Efficacy and safety of rituximab in systemic sclerosis: French retrospective study and literature review","volume":"17","author":"Thiebaut","year":"2018","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.berh.2020.101485_bib5","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1136\/annrheumdis-2018-214816","article-title":"Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study","volume":"78","author":"Elhai","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib6","doi-asserted-by":"crossref","first-page":"1188","DOI":"10.1136\/annrheumdis-2013-204522","article-title":"Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group","volume":"74","author":"Jordan","year":"2015","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib7","doi-asserted-by":"crossref","first-page":"2106","DOI":"10.1093\/rheumatology\/key213","article-title":"Intravenous cyclophosphamide vs rituximab for the treatment of early diffuse scleroderma lung disease: open label, randomized, controlled trial","volume":"57","author":"Sircar","year":"2018","journal-title":"Rheumatology"},{"key":"10.1016\/j.berh.2020.101485_bib8","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1111\/1346-8138.15079","article-title":"Rituximab therapy is more effective than cyclophosphamide therapy for Japanese patients with anti-topoisomerase I-positive systemic sclerosis-associated interstitial lung disease","volume":"46","author":"Ebata","year":"2019","journal-title":"J\u00a0Dermatol"},{"key":"10.1016\/j.berh.2020.101485_bib9","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1186\/s13075-015-0669-3","article-title":"Gene expression changes reflect clinical response in a placebo-controlled randomized trial of abatacept in patients with diffuse cutaneous systemic sclerosis","volume":"17","author":"Chakravarty","year":"2015","journal-title":"Arthritis Res Ther"},{"key":"10.1016\/j.berh.2020.101485_bib10","doi-asserted-by":"crossref","first-page":"2142","DOI":"10.1136\/annrheumdis-2015-208213","article-title":"Treatment with abatacept prevents experimental dermal fibrosis and induces regression of established inflammation-driven fibrosis","volume":"75","author":"Ponsoye","year":"2016","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib11","article-title":"Abatacept in early diffuse cutaneous systemic sclerosis \u2013 results of a phase 2 investigator-initiated, multicenter, double-blind randomized placebo-controlled trial","author":"Khanna","year":"2019","journal-title":"Arthritis Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib12","doi-asserted-by":"crossref","first-page":"1217","DOI":"10.1136\/annrheumdis-2012-202657","article-title":"Outcomes of patients with systemic sclerosis-associated polyarthritis and myopathy treated with tocilizumab or abatacept: a EUSTAR observational study","volume":"72","author":"Elhai","year":"2013","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib13","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1002\/art.40358","article-title":"Belimumab for the treatment of early diffuse systemic sclerosis: results of a randomized, double-blind, placebo-controlled, pilot trial","volume":"70","author":"Gordon","year":"2018","journal-title":"Arthritis Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib14","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1016\/j.berh.2019.01.011","article-title":"Outcomes of patients with systemic sclerosis treated with tocilizumab: case series and review of the literature","volume":"32","author":"Zacay","year":"2018","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib15","doi-asserted-by":"crossref","first-page":"2630","DOI":"10.1016\/S0140-6736(16)00232-4","article-title":"Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial","volume":"387","author":"Khanna","year":"2016","journal-title":"Lancet"},{"key":"10.1016\/j.berh.2020.101485_bib16","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1136\/annrheumdis-2017-211682","article-title":"Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate)","volume":"77","author":"Khanna","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib17","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2018-214449","article-title":"Effectiveness and safety of tocilizumab for the treatment of refractory systemic sclerosis associated interstitial lung disease: a case series","volume":"78","author":"Narv\u00e1ez","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib18","doi-asserted-by":"crossref","first-page":"1362","DOI":"10.1136\/annrheumdis-2018-213031","article-title":"Therapeutic interleukin-6 blockade reverses transforming growth factor-beta pathway activation in dermal fibroblasts: insights from the faSScinate clinical trial in systemic sclerosis","volume":"77","author":"Denton","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib19","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1186\/s13075-019-1979-7","article-title":"A\u00a0multicenter randomized, double-blind, placebo-controlled pilot study to assess the efficacy and safety of riociguat in systemic sclerosis-associated digital ulcers","volume":"21","author":"Nagaraja","year":"2019","journal-title":"Arthritis Res Ther"},{"key":"10.1016\/j.berh.2020.101485_bib20","article-title":"Riociguat in patients with early diffuse cutaneous systemic sclerosis: a randomized, double-blind, placebo-controlled phase IIb study (RISE-SSc) [abstract]","volume":"70","author":"Distler","year":"2018","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib21","doi-asserted-by":"crossref","first-page":"2518","DOI":"10.1056\/NEJMoa1903076","article-title":"Nintedanib for systemic sclerosis-associated interstitial lung disease","volume":"380","author":"Distler","year":"2019","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.berh.2020.101485_bib22","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.02493-2016","article-title":"Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension","volume":"50","author":"Gaine","year":"2017","journal-title":"Eur Respir J"},{"key":"10.1016\/j.berh.2020.101485_bib23","doi-asserted-by":"crossref","first-page":"2370","DOI":"10.1002\/art.40242","article-title":"Efficacy and safety of selexipag in adults with Raynaud's phenomenon secondary to systemic sclerosis: a randomized, placebo-controlled, phase II study","volume":"69","author":"Denton","year":"2017","journal-title":"Arthritis Rheumatol (Hoboken, NJ)"},{"key":"10.1016\/j.berh.2020.101485_bib24","doi-asserted-by":"crossref","first-page":"1672","DOI":"10.3899\/jrheum.151322","article-title":"An open-label, phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial","volume":"43","author":"Khanna","year":"2016","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib25","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1016\/S1474-4422(18)30254-0","article-title":"Classification and management of adult inflammatory myopathies","volume":"17","author":"Selva-O\u2019Callaghan","year":"2018","journal-title":"Lancet Neurol"},{"key":"10.1016\/j.berh.2020.101485_bib26","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1002\/art.37754","article-title":"Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial","volume":"65","author":"Oddis","year":"2013","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib27","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1159\/000070852","article-title":"Successful treatment of dermatomyositis and polymyositis with anti-tumor-necrosis-factor-alpha: preliminary observations","volume":"50","author":"Hengstman","year":"2003","journal-title":"Eur Neurol"},{"key":"10.1016\/j.berh.2020.101485_bib28","doi-asserted-by":"crossref","first-page":"1670","DOI":"10.1136\/ard.2007.077974","article-title":"A\u00a0high incidence of disease flares in an open pilot study of infliximab in patients with refractory inflammatory myopathies","volume":"67","author":"Dastmalchi","year":"2008","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib29","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1159\/000114036","article-title":"Open-label trial of anti-TNF-\u03b1 in dermato- and polymyositis treated concomitantly with methotrexate","volume":"59","author":"Hengstman","year":"2008","journal-title":"Eur Neurol"},{"key":"10.1016\/j.berh.2020.101485_bib30","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1002\/ana.22477","article-title":"A\u00a0Randomized, pilot trial of etanercept in dermatomyositis","volume":"70","author":"Group","year":"2011","journal-title":"Ann Neurol"},{"key":"10.1016\/j.berh.2020.101485_bib31","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1016\/j.semarthrit.2017.10.010","article-title":"A\u00a0randomized, double-blind, placebo-controlled trial of infliximab in refractory polymyositis and dermatomyositis","volume":"47","author":"Schiffenbauer","year":"2018","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib32","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1056\/NEJMc1900045","article-title":"Tofacitinib in amyopathic dermatomyositis\u2013associated interstitial lung disease","volume":"381","author":"Chen","year":"2019","journal-title":"NEJM"},{"key":"10.1016\/j.berh.2020.101485_bib33","doi-asserted-by":"crossref","DOI":"10.1016\/j.semarthrit.2016.08.009","article-title":"A\u00a0case of refractory dermatomyositis responsive to tofacitinib","volume":"46","author":"Paik","year":"2017","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib34","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/RHU.0000000000000807","article-title":"Tofacitinib for the treatment of refractory polymyositis","volume":"25","author":"Babaoglu","year":"2018","journal-title":"JCR J Clin Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib35","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1093\/rheumatology\/key366","article-title":"Management of refractory cutaneous dermatomyositis: potential role of Janus kinase inhibition with tofacitinib","volume":"58","author":"Moghadam-Kia","year":"2019","journal-title":"Rheumatology"},{"key":"10.1016\/j.berh.2020.101485_bib36","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1136\/annrheumdis-2017-211751","article-title":"Abatacept in the treatment of adult dermatomyositis and polymyositis: a randomised, phase IIb treatment delayed-start trial","volume":"77","author":"Tj\u00e4rnlund","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib37","doi-asserted-by":"crossref","first-page":"1344","DOI":"10.1093\/rheumatology\/ker152","article-title":"Therapeutic effect of tocilizumab on two patients with polymyositis","volume":"50","author":"Narazaki","year":"2011","journal-title":"Rheumatology"},{"key":"10.1016\/j.berh.2020.101485_bib38","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/j.jdcr.2018.11.019","article-title":"Apremilast as a potential treatment for moderate to severe dermatomyositis: a retrospective study of 3 patients","volume":"5","author":"Bitar","year":"2019","journal-title":"JAAD Case Rep"},{"key":"10.1016\/j.berh.2020.101485_bib39","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1136\/annrheumdis-2012-202794","article-title":"A\u00a0phase 1b clinical trial evaluating sifalimumab, an anti-IFN-\u03b1 monoclonal antibody, shows target neutralisation of a type I IFN signature in blood of dermatomyositis and polymyositis patients","volume":"73","author":"Higgs","year":"2014","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib40","doi-asserted-by":"crossref","first-page":"2029","DOI":"10.1136\/ard.2011.150326","article-title":"Patients with systemic lupus erythematosus, myositis, rheumatoid arthritis and scleroderma share activation of a common type I interferon pathway","volume":"70","author":"Higgs","year":"2011","journal-title":"Ann Rheum Dis"},{"issue":"suppl 10","key":"10.1016\/j.berh.2020.101485_bib41","article-title":"Efficacy and safety of anifrolumab in patients with moderate to severe systemic lupus erythematosus: results of the second phase 3 randomized controlled trial","volume":"71","author":"Morand","year":"2019","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib42","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1111\/j.1538-7836.2006.01753.x","article-title":"International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS)","volume":"4","author":"Miyakis","year":"2006","journal-title":"J\u00a0Thromb Haemost"},{"key":"10.1016\/j.berh.2020.101485_bib43","first-page":"1","article-title":"EULAR recommendations for the management of antiphospholipid syndrome in adults","author":"Tektonidou","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib44","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1016\/j.autrev.2018.04.009","article-title":"Increased risk of thrombosis in antiphospholipid syndrome patients treated with direct oral anticoagulants. Results from an international patient-level data meta-analysis","volume":"17","author":"Dufrost","year":"2018","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.berh.2020.101485_bib45","doi-asserted-by":"crossref","first-page":"e426","DOI":"10.1016\/S2352-3026(16)30079-5","article-title":"Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome, with or without systemic lupus erythematosus (RAPS): a randomised, controlled, open-label, phase 2\/3, non-inferiority trial","volume":"3","author":"Cohen","year":"2016","journal-title":"Lancet Haematol"},{"key":"10.1016\/j.berh.2020.101485_bib46","doi-asserted-by":"crossref","first-page":"1365","DOI":"10.1182\/blood-2018-04-848333","article-title":"Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome","volume":"132","author":"Pengo","year":"2018","journal-title":"Blood"},{"key":"10.1016\/j.berh.2020.101485_bib47","doi-asserted-by":"crossref","DOI":"10.7326\/M19-0291","article-title":"Rivaroxaban versus vitamin K antagonist in antiphospholipid syndrome: a randomized noninferiority trial","author":"Ordi-Ros","year":"2019","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.berh.2020.101485_bib48","first-page":"2","article-title":"Protocol modification of apixaban for the secondary prevention of thrombosis among patients with antiphospholipid syndrome study","author":"Woller","year":"2017","journal-title":"Clin Appl Thromb"},{"key":"10.1016\/j.berh.2020.101485_bib49","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.autrev.2015.08.011","article-title":"Delineating the deranged immune system in the antiphospholipid syndrome","volume":"15","author":"van den Hoogen","year":"2016","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.berh.2020.101485_bib50","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1136\/ard.2007.078402","article-title":"B\u00a0cell depletion therapy for patients with systemic lupus erythematosus results in a significant drop in anticardiolipin antibody titres","volume":"67","author":"Ioannou","year":"2008","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib51","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1002\/art.37759","article-title":"A\u00a0pilot open-label phase II trial of rituximab for non-criteria manifestations of antiphospholipid syndrome","volume":"65","author":"Erkan","year":"2013","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib52","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.clim.2018.03.001","article-title":"Treatment of antiphospholipid syndrome beyond anticoagulation","volume":"206","author":"Dobrowolski","year":"2019","journal-title":"Clin Immunol"},{"key":"10.1016\/j.berh.2020.101485_bib53","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1016\/j.autrev.2013.05.004","article-title":"Rituximab use in the catastrophic antiphospholipid syndrome: descriptive analysis of the CAPS registry patients receiving rituximab","volume":"12","author":"Berman","year":"2013","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.berh.2020.101485_bib54","doi-asserted-by":"crossref","first-page":"1123","DOI":"10.1177\/0961203316682102","article-title":"Belimumab in primary antiphospholipid syndrome","author":"Yazici","year":"2017","journal-title":"Lupus"},{"key":"10.1016\/j.berh.2020.101485_bib55","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/j.autrev.2018.11.002","article-title":"Belimumab reduces antiphospholipid antibodies in SLE patients independently of hydroxychloroquine treatment","volume":"18","author":"Emmi","year":"2019","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.berh.2020.101485_bib56","doi-asserted-by":"crossref","first-page":"2328","DOI":"10.1002\/art.34400","article-title":"Belimumab reduces autoantibodies, normalizes low complement levels, and reduces select B cell populations in patients with systemic lupus erythematosus","volume":"64","author":"Stohl","year":"2012","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib57","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2018-000693","article-title":"Increased B-cell activating factor BAFF\/B-lymphocyte stimulator BLyS in primary antiphospholipid syndrome is associated with higher adjusted global antiphospholipid syndrome scores","volume":"4","author":"van den Hoogen","year":"2018","journal-title":"RMD Open"},{"key":"10.1016\/j.berh.2020.101485_bib58","doi-asserted-by":"crossref","first-page":"1744","DOI":"10.1056\/NEJMc0910965","article-title":"Eculizumab and renal transplantation in a patient with CAPS","volume":"362","author":"Lonze","year":"2010","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.berh.2020.101485_bib59","first-page":"2","article-title":"Effect on mother and child of eculizumab given before caesarean section in a patient with severe antiphospholipid syndrome","volume":"96","author":"Gustavsen","year":"2017","journal-title":"Med (United States)"},{"key":"10.1016\/j.berh.2020.101485_bib60","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1182\/blood-2015-09-672139","article-title":"Complement activation in antiphospholipid syndrome and its inhibition to prevent rethrombosis after arterial surgery","volume":"127","author":"Meroni","year":"2016","journal-title":"Blood"},{"key":"10.1016\/j.berh.2020.101485_bib61","doi-asserted-by":"crossref","first-page":"2179","DOI":"10.1111\/ajt.12319","article-title":"Eculizumab improves posttransplant thrombotic microangiopathy due to antiphospholipid syndrome recurrence but fails to prevent chronic vascular changes","volume":"13","author":"Canaud","year":"2013","journal-title":"Am J Transplant"},{"key":"10.1016\/j.berh.2020.101485_bib62","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1136\/annrheumdis-2017-212224","article-title":"Complement activation predicts adverse pregnancy outcome in patients with systemic lupus erythematosus and\/or antiphospholipid antibodies","volume":"77","author":"Kim","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib63","doi-asserted-by":"crossref","first-page":"1222","DOI":"10.1038\/nm1121","article-title":"Heparin prevents antiphospholipid antibody-induced fetal loss by inhibiting complement activation","volume":"10","author":"Girardi","year":"2004","journal-title":"Nat Med"},{"key":"10.1016\/j.berh.2020.101485_bib64","doi-asserted-by":"crossref","first-page":"2177","DOI":"10.1111\/jth.13475","article-title":"Rivaroxaban limits complement activation compared with warfarin in antiphospholipid syndrome patients with venous thromboembolism","volume":"14","author":"Arachchillage","year":"2016","journal-title":"J\u00a0Thromb Haemost"},{"key":"10.1016\/j.berh.2020.101485_bib65","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1056\/NEJMoa1312890","article-title":"Inhibition of the mTORC pathway in the antiphospholipid syndrome","volume":"371","author":"Canaud","year":"2014","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.berh.2020.101485_bib66","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1097\/RHU.0000000000000409","article-title":"Stents coated with mammalian target of rapamycin inhibitors (mTOR) appear to Be the best choice in patients with antiphospholipid syndrome and myocardial infarction","volume":"22","author":"Mora-Ramirez","year":"2016","journal-title":"J\u00a0Clin Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib67","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1016\/j.semarthrit.2019.02.006","article-title":"Treatment of refractory poor aPL-related obstetric outcomes with TNF-alpha blockers: maternal-fetal outcomes in a series of 18 cases","volume":"49","author":"Alijotas-Reig","year":"2019","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib68","first-page":"1","article-title":"Sj\u00f6gren syndrome","volume":"2","author":"Brito-Zer\u00f3n","year":"2016","journal-title":"Nat Rev Dis Prim"},{"key":"10.1016\/j.berh.2020.101485_bib69","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2019-eular.8570","article-title":"EULAR recommendations for the management of Sj\u00f6gren\u2019s syndrome with topical and systemic therapies","author":"Ramos-Casals","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib70","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1038\/nrrheum.2018.1","article-title":"B\u00a0cells in the pathogenesis of primary Sj\u00f6gren syndrome","volume":"14","author":"Nocturne","year":"2018","journal-title":"Nat Rev Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib71","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1002\/art.27314","article-title":"Effectiveness of rituximab treatment in primary sj\u00f6gren\u2019s syndrome: a randomized, double-blind, placebo-controlled trial","volume":"62","author":"Meijer","year":"2010","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib72","doi-asserted-by":"crossref","first-page":"1541","DOI":"10.1136\/ard.2007.083865","article-title":"Reduction of fatigue in Sj\u00f6gren syndrome with rituximab: results of a randomised, double-blind, placebo-controlled pilot study","volume":"67","author":"Dass","year":"2008","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib73","doi-asserted-by":"crossref","first-page":"376","DOI":"10.7326\/L14-5017-3","article-title":"Treatment of primary sj\u00f6gren syndrome with rituximab","volume":"161","author":"Devauchelle-Pensec","year":"2014","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.berh.2020.101485_bib74","doi-asserted-by":"crossref","first-page":"1440","DOI":"10.1002\/art.40093","article-title":"Randomized controlled trial of rituximab and cost-effectiveness analysis in treating fatigue and oral dryness in primary sj\u00f6gren\u2019s syndrome","volume":"69","author":"Bowman","year":"2017","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib75","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1136\/annrheumdis-2017-212268","article-title":"Effect of rituximab on a salivary gland ultrasound score in primary Sj\u00f6gren\u2019s syndrome: results of the TRACTISS randomised double-blind multicentre substudy","volume":"77","author":"Fisher","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib76","doi-asserted-by":"crossref","first-page":"R129","DOI":"10.1186\/ar2018","article-title":"Epratuzumab (humanised anti-CD22 antibody) in primary Sj\u00f6gren\u2019s syndrome: an open-label phase I\/II study","volume":"8","author":"Steinfeld","year":"2006","journal-title":"Arthritis Res Ther"},{"key":"10.1016\/j.berh.2020.101485_bib77","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1002\/art.40425","article-title":"Efficacy of epratuzumab, an anti-CD22 monoclonal IgG antibody, in systemic lupus erythematosus patients with associated sj\u00f6gren\u2019s syndrome","volume":"70","author":"Gottenberg","year":"2018","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib78","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1136\/annrheumdis-2013-203991","article-title":"Efficacy and safety of belimumab in primary Sj\u00f6gren\u2019s syndrome: results of the BELISS open-label phase II study","volume":"74","author":"Mariette","year":"2015","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib79","first-page":"2249","article-title":"Efficacy and safety of belimumab given for 12 months in primary Sj\u00f6gren\u2019s syndrome: the BELISS open-label phase II study","volume":"54","author":"De Vita","year":"2015","journal-title":"Rheumatology (United Kingdom)"},{"key":"10.1016\/j.berh.2020.101485_bib80","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1136\/annrheumdis-2018-214720","article-title":"Treatment of primary Sj\u00f6gren\u2019s syndrome with ianalumab (vay736) targeting B cells by BAFF-receptor blockade coupled with enhanced, antibody-dependent cellular cytotoxicity","volume":"78","author":"D\u00f6rner","year":"2019","journal-title":"Ann Rheum Dis"},{"issue":"suppl 10","key":"10.1016\/j.berh.2020.101485_bib81","article-title":"Ianalumab (VAY736), a dual mode of action biologic combining BAFF receptor inhibition with B cell depletion, for treatment of primary Sj\u00f6gren\u2019s syndrome: results of an international randomized, placebo controlled dose range finding study in 190 patients","volume":"71","author":"Bowman","year":"2019","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib82","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1136\/annrheumdis-2012-202071","article-title":"Serum levels of BAFF, but not APRIL, are increased after rituximab treatment in patients with primary Sj\u00f6gren\u2019s syndrome: data from a placebo-controlled clinical trial","volume":"72","author":"Pollard","year":"2013","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib83","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/j.jaut.2015.11.002","article-title":"Blood and salivary-gland BAFF-driven B-cell hyperactivity is associated to rituximab inefficacy in primary Sj\u00f6gren\u2019s syndrome","volume":"67","author":"Cornec","year":"2016","journal-title":"J\u00a0Autoimmun"},{"key":"10.1016\/j.berh.2020.101485_bib84","doi-asserted-by":"crossref","first-page":"1270","DOI":"10.1002\/art.20146","article-title":"Inefficacy of infliximab in primary Sj\u00f6gren\u2019s syndrome: results of the randomized, controlled trial of remicade in primary Sj\u00f6gren\u2019s syndrome (TRIPSS)","volume":"50","author":"Mariette","year":"2004","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib85","first-page":"814","article-title":"Notice of retraction of two articles (\u201cInfliximab in patients with primary Sj\u00f6gren\u2019s syndrome: a pilot study\u201d and \u201cInfliximab in patients with primary Sj\u00f6gren\u2019s syndrome: one-year followup\u201d)","volume":"65","author":"Steinfeld","year":"2013","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib86","doi-asserted-by":"crossref","first-page":"2240","DOI":"10.1002\/art.20299","article-title":"Etanercept in Sj\u00f6gren\u2019s syndrome: a twelve-week randomized, double-blind, placebo-controlled pilot clinical trial","volume":"50","author":"Sankar","year":"2004","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib87","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0030123","article-title":"Interleukin-1 inhibition and fatigue in primary sj\u00f6gren\u2019s syndrome \u2013 a double blind, randomised clinical trial","volume":"7","author":"Norheim","year":"2012","journal-title":"PloS One"},{"key":"10.1016\/j.berh.2020.101485_bib88","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1111\/bcp.13748","article-title":"Anti-IL-7 receptor \u03b1 monoclonal antibody (GSK2618960) in healthy subjects - a randomized, double-blind, placebo-controlled study","volume":"85","author":"Ellis","year":"2019","journal-title":"Br J Clin Pharmacol"},{"key":"10.1016\/j.berh.2020.101485_bib89","doi-asserted-by":"crossref","first-page":"1838","DOI":"10.1136\/annrheumdis-2018-213036","article-title":"Short-term and low-dose IL-2 therapy restores the Th17\/Treg balance in the peripheral blood of patients with primary Sj\u00f6gren\u2019s syndrome","volume":"77","author":"Miao","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib90","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2016-210485","article-title":"Monocyte type I interferon signature in antiphospholipid syndrome is related to proinflammatory monocyte subsets, hydroxychloroquine and statin use","volume":"75","author":"van den Hoogen","year":"2016","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib91","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1136\/annrheumdis-2019-eular.3098","article-title":"Effect of rslv-132 on fatigue in patients with primary Sj\u00f6gren\u2019s syndrome \u2013 results of a phase II randomised, double-blind, placebo-controlled, proof of concept study","volume":"78","author":"Fisher","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib92","doi-asserted-by":"crossref","first-page":"1862","DOI":"10.1002\/acr.22052","article-title":"Evaluation of histologic, serologic, and clinical changes in response to abatacept treatment of primary Sj\u00f6gren\u2019s syndrome: a pilot study","volume":"65","author":"Adler","year":"2013","journal-title":"Arthritis Care Res"},{"key":"10.1016\/j.berh.2020.101485_bib93","doi-asserted-by":"crossref","first-page":"1393","DOI":"10.1136\/annrheumdis-2013-204653","article-title":"Abatacept treatment reduces disease activity in early primary Sj\u00f6gren\u2019s syndrome (open-label proof of concept ASAP study)","volume":"73","author":"Meiners","year":"2014","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib94","first-page":"317","article-title":"Abatacept treatment of patients with primary Sj\u00f6gren\u2019s syndrome results in a decrease of germinal centres in salivary gland tissue","volume":"35","author":"Haacke","year":"2017","journal-title":"Clin Exp Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib95","article-title":"Effectiveness and safety of abatacept for the treatment of patients with primary Sj\u00f6gren\u2019s syndrome","author":"Machado","year":"2019","journal-title":"Clin Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib96","doi-asserted-by":"crossref","first-page":"891","DOI":"10.3109\/14397595.2016.1158773","article-title":"Effectiveness of abatacept for patients with Sj\u00f6gren\u2019s syndrome associated with rheumatoid arthritis. An open label, multicenter, one-year, prospective study: ROSE (Rheumatoid Arthritis with Orencia Trial toward Sj\u00f6gren\u2019s syndrome Endocrinopathy) trial","volume":"26","author":"Tsuboi","year":"2016","journal-title":"Mod Rheumatol"},{"key":"10.1016\/j.berh.2020.101485_bib97","doi-asserted-by":"crossref","first-page":"89.2","DOI":"10.1136\/annrheumdis-2019-eular.2090","article-title":"Efficacy and safety of abatacept in active primary Sj\u00f6gren\u2019s syndrome: results of a randomised placebo-controlled phase III trial","volume":"78","author":"Baer","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib98","doi-asserted-by":"crossref","first-page":"760.2","DOI":"10.1136\/annrheumdis-2019-eular.4277","article-title":"Subcutaneous dosing of the novel anti-CD40 antibody iscalimab achieves target drug exposure and clinical efficacy in primary Sj\u00f6gren\u2019s syndrome; results of a phase IIa randomised open label two arm parallel group trial","volume":"78","author":"Fisher","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib99","doi-asserted-by":"crossref","first-page":"1470","DOI":"10.1002\/art.40513","article-title":"Clinical efficacy and safety of baminercept, a lymphotoxin \u03b2 receptor fusion protein, in primary Sj\u00f6gren\u2019s syndrome: results from a phase II randomized, double-blind, placebo-controlled trial","volume":"70","author":"StClair","year":"2018","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib100","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1136\/annrheumdis-2018-eular.3111","article-title":"A randomised, double-blind study to assess the safety, tolerability and preliminary efficacy of leniolisib (CDZ173) in patients with primary Sj\u00f6gren\u2019s syndrome","volume":"77","author":"D\u00f6rner","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2020.101485_bib101","doi-asserted-by":"crossref","first-page":"1692.2","DOI":"10.1136\/annrheumdis-2019-eular.4887","article-title":"A phase II randomised, double-blind, placebo-controlled, proof of concept study of oral seletalisib in patients with primary Sj\u00f6gren\u2019s syndrome (pSS)","volume":"78","author":"Juarez","year":"2019","journal-title":"Ann Rheum Dis"},{"issue":"suppl 10","key":"10.1016\/j.berh.2020.101485_bib102","article-title":"Therapeutic efficacy of iguratimod in patients with primary Sjogren's syndrome","volume":"68","author":"Zhang","year":"2016","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2020.101485_bib103","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1093\/rheumatology\/key151","article-title":"Ongoing clinical trials and treatment options for patients with systemic sclerosis-associated interstitial lung disease","volume":"58","author":"Khanna","year":"2019","journal-title":"Rheumatology"}],"container-title":["Best Practice & Research Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521694220300012?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521694220300012?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T17:19:03Z","timestamp":1761499143000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1521694220300012"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,2]]},"references-count":103,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,2]]}},"alternative-id":["S1521694220300012"],"URL":"https:\/\/doi.org\/10.1016\/j.berh.2020.101485","relation":{},"ISSN":["1521-6942"],"issn-type":[{"value":"1521-6942","type":"print"}],"subject":[],"published":{"date-parts":[[2020,2]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Targeted therapies in systemic sclerosis, myositis, antiphospholipid syndrome, and Sj\u00f6gren's syndrome","name":"articletitle","label":"Article Title"},{"value":"Best Practice & Research Clinical Rheumatology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.berh.2020.101485","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 The Author(s). Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}],"article-number":"101485"}}