THU0639 Chinese experience with methotrexate as maintenance therapy in igg4-related disease
Authors: F. Lian, J. Zhou, Y. Wang, H. Xu, L. Liang, X. Yang
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> So far, for IgG4-related disease, no randomised clinical trials concerning therapy are published. Corticosteroids are considered the first-line treatment and most patients respond promptly to steroids. However, recurrent or refractory cases are common. Various immunosuppressive agents such as azathioprine (AZA), methotrexate or mycophenolate mofetil (MMF) have been introduced as corticosteroid-sparing treatment on small case series.<sup>1</sup> High cost of MMF and occasional severe bone marrow toxicity of Aza have limited their use in Chinese patients. Efficacious therapies with less toxicity and more cost-effectiveness were required to be identified and thus modify the immunotherapeutic strategies. Methotrexate (MTX) was commonly used in many other autoimmune disease. In the present study, we retrospectively reviewed 84 IgG4-RD patients who received MMF, Aza or MTX as maintenance regimens, compared the efficacy among these three groups and observed side effects of the medication. <h3>Objectives</h3> To assess the efficacy, safety and tolerability of methotrexate as maintenance therapy in the treatment of IgG4-related disease. <h3>Methods</h3> We retrospectively reviewed 84 IgG4-RD patients refractory or recurrent to steroids in south China from January 2012 to December 2015 who received MMF, Aza or MTX as maintenance regimen. MMF was administrated daily to a maximum of 3 g/day. Aza was given at a dose maximum of 200 mg/day. MTX was given at a dose of 10–20 mg/week. Steroids were continued at the lowest effective dose. Details of the clinical presentation, serological, immunological variables and side effects were collected. <h3>Results</h3> Of the 84 patients, no significance of demographic variables was found among MMF group (22 cases), Aza group (29 cases), and MTX group (33 cases). The 2 year overall, the three groups had similar rates of remaining remission, which was 87.9% (29/33) in MTX group, 86.2% (25/29) in Aza group and 86.3 (19/22) in MMF group. Of the 11 recurrent cases despite immunosuppressive therapies, 2 cases were related to thyroid gland, 4 cases were related to pancreas, 3 cases were related to nose, and 2 cases were related to brain. All cases but the 2 patients with brain lesions achieved remission again after we changed the immunosuppressives. One case on Aza had severe bone marrow toxicity and 2 cases on MMF had severe infection. None of the cases on MTX had side effects affecting the treatment course. It's only associated with reversible liver dysfunction in our study. <h3>Conclusions</h3> MTX was as efficacious as MMF and Aza in maintaining remission in IgG4-related diseases, but with lower price and less severe side effects. IgG4-related brain lesions may be refractory and probably need to be treated with more aggressive agents. <h3>Reference</h3> [1] Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet2015; 385(9976):1460–71. <h3>Acknowledgements</h3> Project supported by National Natural Science Foundation of China (No. 81102270) and Guangdong Natural Science Foundation No. 2016 A030313217) <h3>Disclosure of Interest</h3> None declared