IgG4-related disease is a chronic immune-mediated fibroinflammatory condition that can involve multiple organ systems with tumefactive lesions and characteristic tissue pathology. Commonly affected sites include pancreas, salivary glands, bile ducts, kidneys, and retroperitoneum. Many patients respond to glucocorticoids and other immunosuppressive therapies.
January and March 2026 drug pipeline updates included Phase 3 results, Fast Track designations and Priority Review decisions across multiple disease areas. Mavacamten, teplizumab-mzwv, anifrolumab, AXS-05, gedatolisib and AFTX-201 were among the programs with notable milestones.
Sanofi's oral BTK inhibitor rilzabrutinib received orphan drug designation in Japan for IgG4-related disease and warm autoimmune hemolytic anemia, while also securing U.S. FDA breakthrough therapy designation for the rare anemia indication.
Japan's Ministry of Health, Labour and Welfare has granted orphan drug designation to Sanofi's rilzabrutinib for IgG4-related disease, marking the third global orphan designation for this indication based on positive phase 2 study data.
| NCT ID | Title | Status | Phase |
|---|---|---|---|
| NCT07025330 |
A Study of Efgartigimod in Patients With IgG4-Related Disease |
RECRUITING | PHASE2 |
| NCT06663618 |
Short-term Glucocorticoid Combined with MMF for IgG4-RD |
RECRUITING | NA |
| NCT06497387 |
Safety and Efficacy of PRG-1801 for Refractory Lupus Nephritis and IgG4-Related Disease |
RECRUITING | EARLY_PHASE1 |
| NCT06497361 |
Safety and Efficacy of PRG-2311 for Refractory Lupus Nephritis and IgG4-Related Disease |
RECRUITING | EARLY_PHASE1 |
| NCT06285539 |
Drug Rediscovery for Rare Immune Mediated Inflammatory Diseases |
RECRUITING | PHASE2 |
| NCT05746689 |
Study of Sirolimus in IgG4-related Disease |
NOT_YET_RECRUITING | NA |
| NCT05625581 |
Tofatib Treatment for IgG4-related Disease |
RECRUITING | |
| NCT04660565 |
Belimumab Treatment for IgG4-related Disease |
UNKNOWN | PHASE4 |
| NCT04125511 |
Characterizing IgG4-RD With 68Ga-FAPI PET/CT |
UNKNOWN | EARLY_PHASE1 |
| NCT03690908 |
Infraorbital Nerve Involvement on Magnetic Resonance Imaging in IgG4-related Ophthalmic Disease |
COMPLETED |
