Study of Sirolimus in IgG4-related Disease

Status Not yet recruiting

Clinical Trial Summary

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.

Clinical Trial Description

IgG4-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. IgG4-RD is characterized by elevated serum IgG4 levels, tumefactive lesions with a dense lymphoplasmacytic infiltration rich in IgG4 positive plasma cells and storiform fibrosis of related organs. 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. For some mild IgG4-RD patients without internal organ damage, long-term glucocorticoids therapy may have a low benefit/risk ratio. Further, a substantial proportion of patients cannot tolerate glucocorticoids. Sirolimus, also known as rapamycin, is a macrolide compound that inhibits its mechanistic target (mTOR), which regulates cell growth and metabolism in response to environmental cues. mTOR is also essential in driving abnormal lineage specification within the immune system in various rheumatic diseases. We discovered that mTOR was highly activated in IgG4RD tissues, and its inhibitor sirolimus appeared as a good treatment candidate. ;

Study Design

Related Conditions & MeSH terms

IgG4-related Disease

Clinical Trial Details

NCT number	NCT05746689
Study type	Interventional
Source	Peking University International Hospital
Contact	Yuying WANG, Master
Phone	8615210976309
Email	wangyuying028@126.com
Status	Not yet recruiting
Phase	N/A
Start date	March 1, 2023
Completion date	December 31, 2028

View Details

See also
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Completed	`NCT03669861` - Safety and Efficacy of Abatacept in IgG4-Related Disease	Phase 2
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Recruiting	`NCT04125511` - Characterizing IgG4-RD With 68Ga-FAPI PET/CT	Early Phase 1
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Completed	`NCT03690908` - Infraorbital Nerve Involvement on Magnetic Resonance Imaging in IgG4-related Ophthalmic Disease
Completed	`NCT02899039` - Follicular Helper T Cells: Biological Marker and Involvement in the Physiopathology of the IgG4-related Disease	N/A
Recruiting	`NCT04660565` - Belimumab Treatment for IgG4-related Disease	Phase 4
Recruiting	`NCT01670695` - A Prospective Cohort Study of IgG4RD in China	N/A
Recruiting	`NCT03023371` - National Registry of IgG4-RD in China	N/A
Unknown status	`NCT01758393` - Glucocorticoids in Patients With IgG4-RD	Phase 2/Phase 3
Completed	`NCT02705638` - Treatment of IgG4-Related Disease With Revlimid and Rituximab	Phase 1
Recruiting	`NCT03473912` - Meir Medical Center Rheumatologic Biobank
Not yet recruiting	`NCT03466970` - Plasmablast Detection From IgG4-Related Disease Patients	N/A

Clinical trials are conducted in a series of steps, called phases - each phase is designed to answer a separate research question.

Phase 1: Researchers test a new drug or treatment in a small group of people for the first time to evaluate its safety, determine a safe dosage range, and identify side effects.
Phase 2: The drug or treatment is given to a larger group of people to see if it is effective and to further evaluate its safety.
Phase 3: The drug or treatment is given to large groups of people to confirm its effectiveness, monitor side effects, compare it to commonly used treatments, and collect information that will allow the drug or treatment to be used safely.
Phase 4: Studies are done after the drug or treatment has been marketed to gather information on the drug's effect in various populations and any side effects associated with long-term use.