Disease · MONDO_0017287

immunoglobulin G4-related sclerosing disease

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. immunoglobulin G4-related sclerosing disease. Diseases, record MONDO_0017287. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0017287

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Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: immunoglobulin G4-related sclerosing disease; Open Targets
Open Targets ID: MONDO_0017287; Open Targets
Other names: IgG4-RD, IgG4-related disease, IgG4-related sclerosing disease, IgG4-related systemic disease; Open Targets
Disease Ontology: DOID:0080356; Disease Ontology
NCI Thesaurus: C95992; NCI Thesaurus
Orphanet: ORPHA:284264; Orphanet
Orphanet: ORPHA:596448; Orphanet
Highest stage of its drugs: Phase 3, for 2 of its 21 drugs, from ClinicalTrials.gov and TTD; Open Targets

Drugs21

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
RILZABRUTINIB	CHEMBL3702854	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
OBEXELIMAB	CHEMBL4298134	Antibody	Phase 3	TTD	3	1	1	Drugs and targets
PREDNISONE	CHEMBL635	Small molecule	Phase 2/3	ClinicalTrials.gov	7	7	6	Drugs and targets
LENALIDOMIDE	CHEMBL848	Small molecule	Phase 2/3	ClinicalTrials.gov	1	1	1	Drugs and targets
MYCOPHENOLATE MOFETIL	CHEMBL1456	Small molecule	Phase 2	ClinicalTrials.gov	4	4	4	Drugs and targets
EFGARTIGIMOD ALFA	CHEMBL4297551	Protein	Phase 2	ClinicalTrials.gov	2	1	1	Drugs and targets
ABATACEPT	CHEMBL1201823	Protein	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
FILGOTINIB	CHEMBL3301607	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
FLUDARABINE	CHEMBL1568	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

1 to 10 of 21

See all 21 drugs

Targets23

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
JAK1	Janus Kinase (JAK)	3	Janus Kinase (JAK) inhibitor; Tyrosine-protein kinase JAK1 inhibitor	Phase 2	https://conductscience.org/library/genes/3716	Drugs and targets
JAK2	Janus Kinase (JAK)	3	Janus Kinase (JAK) inhibitor; Tyrosine-protein kinase JAK2 inhibitor	Phase 2	https://conductscience.org/library/genes/3717	Drugs and targets
NR3C1	Glucocorticoid receptor	3	Glucocorticoid receptor agonist	Phase 2/3	https://conductscience.org/library/genes/2908	Drugs and targets
BTK	Tyrosine-protein kinase BTK	2	Tyrosine-protein kinase BTK inhibitor	Phase 3	https://conductscience.org/library/genes/695	Drugs and targets
CD19	B-lymphocyte antigen CD19	2	B-lymphocyte antigen CD19 binding agent; B-lymphocyte antigen CD19 inhibitor	Phase 3	https://conductscience.org/library/genes/930	Drugs and targets
FCGRT	IgG receptor FcRn large subunit p51	2	IgG receptor FcRn large subunit p51 antagonist	Phase 2	https://conductscience.org/library/genes/2217	Drugs and targets
JAK3	Janus Kinase (JAK)	2	Janus Kinase (JAK) inhibitor; Tyrosine-protein kinase JAK3 inhibitor	Phase 2	https://conductscience.org/library/genes/3718	Drugs and targets
TYK2	Janus Kinase (JAK)	2	Janus Kinase (JAK) inhibitor; Tyrosine-protein kinase TYK2 inhibitor	Phase 2	https://conductscience.org/library/genes/7297	Drugs and targets
CD80	T-lymphocyte activation antigen CD80	1	T-lymphocyte activation antigen CD80 inhibitor	Phase 2	https://conductscience.org/library/genes/941	Drugs and targets
CD86	T-lymphocyte activation antigen CD86	1	T-lymphocyte activation antigen CD86 inhibitor	Phase 2	https://conductscience.org/library/genes/942	Drugs and targets

1 to 10 of 23

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Trials21

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT01584388	Rituximab in IgG4-RD: A Phase 1-2 Trial	Phase 1/2	30	2017-07-02	Retroperitoneal Fibrosis, Autoimmune Pancreatitis, Sialadenitis, Pseudotumor	RITUXIMAB	Its record names the disease	ClinicalTrials.gov
NCT02725476	Study to Evaluate the Effect of XmAb®5871 on Disease Activity in Patients With IgG4-Related Disease (RD)	Phase 2	20	2018-12-07	IgG4-RD	OBEXELIMAB	Its record names the disease	ClinicalTrials.gov
NCT03669861	Safety and Efficacy of Abatacept in IgG4-Related Disease	Phase 2	10	2021-08-24	IgG4-related Disease	ABATACEPT	Its record names the disease	ClinicalTrials.gov
NCT07068165	A Randomized Controlled Study of the Efficacy and Safety of Lenalidomide in the Treatment of Active IgG4-related Disease	Phase 2/3	198		IgG4 Related Disease	LENALIDOMIDE, PREDNISONE	Its record names the disease	ClinicalTrials.gov
NCT07190196	A 52-week Study of Rilzabrutinib Efficacy and Safety Compared to Placebo in Adults Diagnosed With IgG4-related Disease	Phase 3	124		Immunoglobulin G4 Related Disease	RILZABRUTINIB	Its record names the disease	ClinicalTrials.gov
NCT05974683	Treatment Strategies in IgG4-RD Patients With Re-elevation of Serum IgG4 Level During Maintenance Remission Period	Not applicable	108		Autoimmune Diseases	MYCOPHENOLATE MOFETIL	Its record names the disease	ClinicalTrials.gov
NCT05781516	Baricitinib Plus Glucocorticoid for Eosinophilia in IgG4-RD	Not applicable	74		IgG4-related Disease With Eosinophilia	BARICITINIB, PREDNISOLONE	Its record names the disease	ClinicalTrials.gov
NCT03715699	Leflunomide Treatment for IgG4-RD	Not applicable	70		Autoimmune Disease	LEFLUNOMIDE, PREDNISONE	Its record names the disease	ClinicalTrials.gov
NCT06663618	Short-term Glucocorticoid Combined with MMF for IgG4-RD	Not applicable	63		IgG4-related Disease	MYCOPHENOLATE MOFETIL, PREDNISONE	Its record names the disease	ClinicalTrials.gov
NCT02458196	Study of Treatment Response on IgG4 Related Disease (IgG4RD)	Phase 2	60		Autoimmune Disease	MYCOPHENOLATE MOFETIL, PREDNISONE	Its record names the disease	ClinicalTrials.gov

1 to 10 of 21

See all 21 trials

Comparisons in its trials3

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

Comparison	Group 1, with the outcome	Group 2, with the outcome	Participants	Trial	Source
XmAb5871 5 mg/kg vs XmAb5871 Fixed Dose: Number of Patients Experiencing a Treatment-emergent Adverse Event as Assessed by CTCAE v4.3	13 of 15	5 of 5	20	NCT02725476	Trial comparisons
XmAb5871 5 mg/kg vs XmAb5871 Fixed Dose: deaths from any cause	0 of 15	0 of 5	20	NCT02725476	Trial comparisons
XmAb5871 5 mg/kg vs XmAb5871 Fixed Dose: serious adverse events	1 of 15	1 of 5	20	NCT02725476	Trial comparisons

Papers from its trials9

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
39672025	RESULT	1	PubMed
38643278	RESULT	1	PubMed
38602546	RESULT	1	PubMed
35333600	RESULT	1	PubMed
42731845	DERIVED	1	PubMed
39915014	DERIVED	1	PubMed
38251576	DERIVED	1	PubMed
30124952	DERIVED	1	PubMed
25667206	DERIVED	1	PubMed

Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0017287

GET https://conductscience.org/api/diseases/v1/MONDO_0017287/drugs?page=2

API reference For AI assistants