Combination therapy of leflunomide and glucocorticoids for the maintenance of remission in patients with IgG4-related disease: a retrospective study and literature review.
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.


OBJECTIVE
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.


CONCLUSIONS
The combination therapy of LEF and glucocoticoids is effective and safe in IgG4-RD.
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Yiwen
http://orcid.org/0000-0003-2495-6552
Y
Wang
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Kunpeng
K
Li
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Dai
D
Gao
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Gui
G
Luo
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Yurong
Y
Zhao
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Xiuru
X
Wang
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Jie
J
Zhang
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Jingyu
J
Jin
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Zheng
Z
Zhao
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Chunhua
C
Yang
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Jian
J
Zhu
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Jianglin
J
Zhang
Department of Rheumatology, Chinese PLA General Hospital, Beijing, China.
Feng
F
Huang
D001327:Autoimmune Diseases / Q000097:blood* / Q000175:diagnosis / Q000188:drug therapy*; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D007555:Isoxazoles / Q000008:administration & dosage*; D000077339:Leflunomide; D008297:Male; D008875:Middle Aged; D012074:Remission Induction / Q000379:methods; D012189:Retrospective Studies; D016896:Treatment Outcome
D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D007555:Isoxazoles; D000077339:Leflunomide

IgG4-related disease; glucocorticoid; immunosuppressive agent; leflunomide; treatment outcome