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Combination therapy of leflunomide and glucocorticoids for the maintenance of remission in patients with IgG4 ‐related disease: a retrospective study and literature review
Author(s) -
Wang Yiwen,
Li Kunpeng,
Gao Dai,
Luo Gui,
Zhao Yurong,
Wang Xiuru,
Zhang Jie,
Jin Jingyu,
Zhao Zheng,
Yang Chunhua,
Zhu Jian,
Zhang Jianglin,
Huang Feng
Publication year - 2017
Publication title -
internal medicine journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.596
H-Index - 70
eISSN - 1445-5994
pISSN - 1444-0903
DOI - 10.1111/imj.13430
Subject(s) - medicine , leflunomide , igg4 related disease , adverse effect , retrospective cohort study , glucocorticoid , gastroenterology , disease , rheumatoid arthritis
Background Although glucocorticoids are effective in IgG4 ‐related disease ( IgG4‐RD ), patients may relapse during or after glucocorticoid tapering. Immunosuppressive agents, including leflunomide ( LEF ), are regarded as steroid‐sparing agents in other autoimmune disorders and need to be discussed in the management of IgG4‐RD . Aim To identify the efficacy and safety of combination therapy of LEF and glucocorticoids in IgG4‐RD . Methods We retrospectively summarised data of patients diagnosed with IgG4‐RD between November 2012 and November 2015. Only patients treated with LEF plus glucocorticoids and had been followed up for more than three visits and 6 months were analysed with clinical symptoms, laboratory and imaging findings, treatment protocol, LEF ‐related adverse events and disease activity reflected by IgG4‐RD Responder Index ( IgG4‐RD RI ). Results A total of 18 patients, including 14 untreated patients and 4 relapsing patients, was included. The mean ( SD ) onset age was 54.0 (9.6) years. The mean ( SD ) follow‐up period was 12.1 (7.4) months. All patients had active disease with mean ( SD ) IgG4‐RD RI of 15.0 (5.6) at baseline and experienced improvements at 1 month. At the last follow up, the mean ( SD ) IgG4‐RD Responder Index declined to 3.1 (1.7) in all patients and to 2.5 (1.2) in patients without relapse. The mean ( SD ) dose of GC declined to 6.9 (2.7) mg/day. A total of 12 (66.7%) and 11 (61.1%) patients were in remission at 6 months and the last follow up respectively. Three (16.7%) patients relapsed in clinical course. Two reversible adverse events were observed. Conclusion The combination therapy of LEF and glucocoticoids is effective and safe in IgG4‐RD .