gG4-related disease (IgG4-RD) is a newly recognized systemic autoimmune disease that can
involve the pan-creatobiliary tract, retroperitoneum/aorta, head and neck region, and
salivary glands, et al. Glucocorticoids are the first-line agents for the treatment of
IgG4-RD, however, in order to maintain long-term disease stability and avoid disease relapse,
glucocorticoids maintenance therapy should last for a long period, which may induce various
glucocorticoid-associated adverse reactions. Sirolimus plays dual roles in inhibiting
lymphocyte activation and fibroblast proliferation. It is inferred from its mechanism that
sirolimus is a good potential treatment option for IgG4-RD. Therefore, we conducted this
single-arm clinical trial on patients with IgG4-RD to determine the efficacy and safety of
sirolimus.