{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,8]],"date-time":"2026-05-08T00:23:11Z","timestamp":1778199791639,"version":"3.51.4"},"reference-count":36,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2017,6,4]],"date-time":"2017-06-04T00:00:00Z","timestamp":1496534400000},"content-version":"vor","delay-in-days":3,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007185","name":"University of California, Los Angeles","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007185","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2017,6]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Although glucocorticoids are effective in <jats:styled-content style=\"fixed-case\">IgG4<\/jats:styled-content>\u2010related disease (<jats:styled-content style=\"fixed-case\">IgG4\u2010RD<\/jats:styled-content>), patients may relapse during or after glucocorticoid tapering. Immunosuppressive agents, including leflunomide (<jats:styled-content style=\"fixed-case\">LEF<\/jats:styled-content>), are regarded as steroid\u2010sparing agents in other autoimmune disorders and need to be discussed in the management of <jats:styled-content style=\"fixed-case\">IgG4\u2010RD<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To identify the efficacy and safety of combination therapy of <jats:styled-content style=\"fixed-case\">LEF<\/jats:styled-content> and glucocorticoids in <jats:styled-content style=\"fixed-case\">IgG4\u2010RD<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively summarised data of patients diagnosed with <jats:styled-content style=\"fixed-case\">IgG4\u2010RD<\/jats:styled-content> between November 2012 and November 2015. Only patients treated with <jats:styled-content style=\"fixed-case\">LEF<\/jats:styled-content> plus glucocorticoids and had been followed up for more than three visits and 6\u2009months were analysed with clinical symptoms, laboratory and imaging findings, treatment protocol, <jats:styled-content style=\"fixed-case\">LEF<\/jats:styled-content>\u2010related adverse events and disease activity reflected by <jats:styled-content style=\"fixed-case\">IgG4\u2010RD<\/jats:styled-content> Responder Index (<jats:styled-content style=\"fixed-case\">IgG4\u2010RD RI<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 18 patients, including 14 untreated patients and 4 relapsing patients, was included. The mean (<jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content>) onset age was 54.0 (9.6) years. The mean (<jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content>) follow\u2010up period was 12.1 (7.4) months. All patients had active disease with mean (<jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content>) <jats:styled-content style=\"fixed-case\">IgG4\u2010RD RI<\/jats:styled-content> of 15.0 (5.6) at baseline and experienced improvements at 1 month. At the last follow up, the mean (<jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content>) <jats:styled-content style=\"fixed-case\">IgG4\u2010RD<\/jats:styled-content> Responder Index declined to 3.1 (1.7) in all patients and to 2.5 (1.2) in patients without relapse. The mean (<jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content>) dose of <jats:styled-content style=\"fixed-case\">GC<\/jats:styled-content> declined to 6.9 (2.7) mg\/day. A total of 12 (66.7%) and 11 (61.1%) patients were in remission at 6\u2009months and the last follow up respectively. Three (16.7%) patients relapsed in clinical course. Two reversible adverse events were observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The combination therapy of <jats:styled-content style=\"fixed-case\">LEF<\/jats:styled-content> and glucocoticoids is effective and safe in <jats:styled-content style=\"fixed-case\">IgG4\u2010RD<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/imj.13430","type":"journal-article","created":{"date-parts":[[2017,3,21]],"date-time":"2017-03-21T10:03:22Z","timestamp":1490090602000},"page":"680-689","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Combination therapy of leflunomide and glucocorticoids for the maintenance of remission in patients with <scp>IgG4<\/scp>\u2010related disease: a retrospective study and literature 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