Ignite Creation Date:
2024-05-06 @ 6:40 PM
Last Modification Date:
2024-10-26 @ 2:52 PM
Study NCT ID:
NCT05746689
Status:
NOT_YET_RECRUITING
Last Update Posted:
2023-02-28
First Post:
2023-02-13
Brief Title:
Study of Sirolimus in IgG4-related Disease
Sponsor:
Peking University International Hospital
Organization:
Peking University International Hospital
Study Overview
Official Title:
Combination Therapy of Sirolimus and Glucocorticoids for the Maintenance of Remission in Patients With IgG4-related Disease
Status:
NOT_YET_RECRUITING
Status Verified Date:
2023-02
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief Summary:
gG4-related disease IgG4-RD is a newly recognized systemic autoimmune disease that can involve the pan-creatobiliary tract retroperitoneumaorta 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
Detailed Description:
IgG4-related disease IgG4-RD is a newly recognized systemic autoimmune disease that can involve the pan- creatobiliary tract retroperitoneumaorta 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 benefitrisk 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
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 benefitrisk 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 Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
False
Is an FDA AA801 Violation?:
None