Autoinflammatory & rare systemic diseases
Effectiveness of tofacitinib monotherapy for patients with immunoglobulin G4-related disease (IgG4-RD) or idiopathic retroperitoneal fibrosis
Authors' conclusion
Tofacitinib is a potentially effective treatment for IgG4-RD and IRF and it is reasonable to conduct clinical trial to validate its efficacy.
Study context
- Population
- Patients with IgG4-RD or idiopathic retroperitoneal fibrosis (IRF) administered tofacitinib monotherapy
- Participants
- 2 vs 2
- Exposure
- IRF vs IgG4-RD
- IRFn = 2
- IgG4-RDn = 2
- Outcome
- Response to tofacitinib monotherapy (IgG4-RD RI)
Methodological detailsretrospective clinical data collection, immunohistochemistry
- Retrospective clinical data collection
- Immunohistochemistry
Main finding
As reported in the abstract
- IRF (both patients): complete remission with diminished retroperitoneal mass and decreased CRP; IgG4-RD RI decreased from 6 to 1
- 2
Observational data: associations, not effects.
Further findings
JAK expression in lesions (immunohistochemistry; 3 IgG4-RD salivary glands, 1 IRF retroperitoneal tissue)
- JAK1, JAK2, JAK3 and TYK2 expression detected in biopsy tissues
- JAK family staining intensity in IRF lesion similar to IgG4-RD lesions
Original publication
Effectiveness of tofacitinib monotherapy for patients with IgG4-RD or idiopathic retroperitoneal fibrosis. Clin Exp Rheumatol. Published 7 December 2023. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →