IgG4-related sclerosing cholangitis
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Summary
IgG4-related sclerosing cholangitis (MONDO:0018645) is a disease and 4 clinical trials. Top therapeutic interventions include rituximab. A subtype of autoimmune disorder of endocrine system — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Clinical trials: 4
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | IgG4-related sclerosing cholangitis |
| Mondo ID | MONDO:0018645 |
| Orphanet | 447764 |
| ICD-11 | 1676971795 |
| SNOMED CT | 722870008 |
| UMLS | C4302109 |
| MedGen | 927778 |
| GARD | 0021867 |
| Is cancer (heuristic) | no |
Disease family
This is a subtype of autoimmune disorder of endocrine system. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › endocrine system disorder › autoimmune disorder of endocrine system › IgG4-related sclerosing cholangitis
Related subtypes (12): type 1 diabetes mellitus, autoimmune thyroid disease, immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome, autoimmune enteropathy and endocrinopathy - susceptibility to chronic infections syndrome, autoimmune pancreatitis, autoimmune hepatitis, autoimmune polyendocrinopathy, autoimmune hypoparathyroidism, insulin autoimmune syndrome, lymphocytic hypophysitis, autoimmune oophoritis, autoimmune primary ovarian failure
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 4.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 3 |
| PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT02705638 | PHASE1 | COMPLETED | Treatment of IgG4-Related Disease With Revlimid and Rituximab |
| NCT02616705 | Not specified | COMPLETED | Bile Usefulness for Detecting IgG4-related Sclerosing Cholangitis |
| NCT02797665 | Not specified | UNKNOWN | Treatment of Obstructive Jaundice in Autoimmune Pancreatitis and/or Immunoglobulin G4-related Sclerosing Cholangitis by Corticosteroids |
| NCT05463445 | Not specified | UNKNOWN | Multicenter Observational Study of PSC and IgG4-SC in China |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| RITUXIMAB | 4 | 1 |
Related Atlas pages
- Drugs: Rituximab