Disease · MONDO_0005019

diffuse scleroderma

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. diffuse scleroderma. Diseases, record MONDO_0005019. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0005019

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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: diffuse scleroderma; Open Targets
Open Targets ID: MONDO_0005019; Open Targets
Other names: Diffuse cutaneous scleroderma, Diffuse cutaneous systemic sclerosis, Diffuse systemic sclerosis, Progressive Scleroderma, Progressive Systemic Sclerosis, Scleroderma, Diffuse, Scleroderma, Progressive, Scleroderma, Sudden Onset, Sclerodermas, Sudden Onset, Sclerosis, Progressive Systemic, Sudden Onset Scleroderma, Sudden Onset Sclerodermas, Systemic Sclerosis, Progressive, Systemic sclerosis, diffuse, systemic sclerosis, diffuse (disorder); Open Targets
Disease Ontology: DOID:1580; Disease Ontology
MeSH: D045743; MeSH
Highest stage of its drugs: Phase 3, for 3 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
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 3	ClinicalTrials.gov	3	3	3	Drugs and targets
HUMAN IMMUNOGLOBULIN G	CHEMBL1201599		Phase 3	ClinicalTrials.gov	2	2	0	Drugs and targets
LENABASUM	CHEMBL456341	Small molecule	Phase 3	TTD	1	0	0	Drugs and targets
BRENTUXIMAB VEDOTIN	CHEMBL1742994	Antibody drug conjugate	Phase 2	ClinicalTrials.gov	3	3	3	Drugs and targets
BELIMUMAB	CHEMBL1789843	Antibody	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
MYCOPHENOLATE MOFETIL	CHEMBL1456	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
RITUXIMAB	CHEMBL1201576	Antibody	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
BELUMOSUDIL	CHEMBL2005186	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
IFETROBAN	CHEMBL3301673	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
PREDNISOLONE	CHEMBL131	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

1 to 10 of 21

See all 21 drugs

Targets36

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
DHFR	Dihydrofolate reductase	1	Dihydrofolate reductase inhibitor	Unknown	https://conductscience.org/library/genes/1719	Drugs and targets
EDNRA	Endothelin receptor, ET-A/ET-B	1	Endothelin receptor, ET-A/ET-B antagonist	Unknown	https://conductscience.org/library/genes/1909	Drugs and targets
EDNRB	Endothelin receptor, ET-A/ET-B	1	Endothelin receptor, ET-A/ET-B antagonist	Unknown	https://conductscience.org/library/genes/1910	Drugs and targets
GUCY1A1	Soluble guanylate cyclase	1	Soluble guanylate cyclase positive allosteric modulator	Phase 2	https://conductscience.org/library/genes/2982	Drugs and targets
GUCY1A2	Soluble guanylate cyclase	1	Soluble guanylate cyclase positive allosteric modulator	Phase 2	https://conductscience.org/library/genes/2977	Drugs and targets
GUCY1B1	Soluble guanylate cyclase	1	Soluble guanylate cyclase positive allosteric modulator	Phase 2	https://conductscience.org/library/genes/2983	Drugs and targets
IGF1R	Insulin-like growth factor 1 receptor	1	Insulin-like growth factor I receptor antagonist	Phase 1	https://conductscience.org/library/genes/3480	Drugs and targets
IL13	Interleukin-13	1	Interleukin-13 inhibitor	Phase 2	https://conductscience.org/library/genes/3596	Drugs and targets
IL4	Interleukin-4	1	Interleukin-4 inhibitor	Phase 2	https://conductscience.org/library/genes/3565	Drugs and targets
IMPDH1	Inosine-5'-monophosphate dehydrogenase (IMPDH)	1	Inosine-5'-monophosphate dehydrogenase (IMPDH) inhibitor	Phase 2	https://conductscience.org/library/genes/3614	Drugs and targets

1 to 10 of 36

See all 36 targets

Trials20

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. 2 more are not shown, since the checks do not confirm them.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT02283762	Efficacy and Safety of Riociguat in Patients With Systemic Sclerosis	Phase 2	121	2019-01-25	Scleroderma, Systemic	RIOCIGUAT	Its record names the disease	ClinicalTrials.gov
NCT02921971	Effectiveness and Safety of SAR156597 in Treating Diffuse Systemic Sclerosis	Phase 2	97	2022-02-02	Systemic Sclerosis	ROMILKIMAB	Its record names the disease	ClinicalTrials.gov
NCT03919799	KD025 in Subjects With Diffuse Cutaneous Systemic Sclerosis	Phase 2	36	2023-08-30	System; Sclerosis, Diffuse Cutaneous Systemic Sclerosis	BELUMOSUDIL	Its record names the disease	ClinicalTrials.gov
NCT01670565	Belimumab for the Treatment of Diffuse Cutaneous Systemic Sclerosis	Phase 2	20	2022-05-24	Systemic Sclerosis	BELIMUMAB, MYCOPHENOLATE MOFETIL	Its record names the disease	ClinicalTrials.gov
NCT03222492	Brentuximab Vedotin for Systemic Sclerosis	Phase 1/2	17	2024-05-17	Diffuse Cutaneous Systemic Sclerosis, Scleroderma, dcSSc	BRENTUXIMAB VEDOTIN	Its record names the disease	ClinicalTrials.gov
NCT01093885	Study of Ambrisentan With Antifibrotic Agent Combination Therapy in Diffuse Systemic Sclerosis	Not applicable	15	2018-11-29	Systemic Sclerosis, Scleroderma	AMBRISENTAN	Its record names the disease	ClinicalTrials.gov
NCT03274076	Evaluation of Tofacitinib in Early Diffuse Cutaneous Systemic Sclerosis (dcSSc)	Phase 1/2	15	2020-05-08	Systemic Sclerosis, Scleroderma	TOFACITINIB	Its record names the disease	ClinicalTrials.gov
NCT00501995	High Dose Cyclophosphamide for Treatment of Scleroderma	Phase 3	6	2014-07-02	Scleroderma	CYCLOPHOSPHAMIDE	Its record names the disease	ClinicalTrials.gov
NCT04478994	A Study With TEPEZZA in Patients With Diffuse Cutaneous Systemic Sclerosis (dcSSc)	Phase 1	3	2024-04-19	Diffuse Cutaneous Systemic Sclerosis	TEPROTUMUMAB	Its record names the disease	ClinicalTrials.gov
NCT02339441	Treatment Outcome in Early Diffuse Cutaneous Systemic Sclerosis		320		Early Diffuse Cutaneous Systemic Sclerosis	CYCLOPHOSPHAMIDE, METHOTREXATE, MYCOPHENOLATE MOFETIL	Its record names the disease	ClinicalTrials.gov

1 to 10 of 20

See all 20 trials

Comparisons in its trials8

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
SAR156597 vs Placebo: deaths from any cause	1 of 48	1 of 49	97	NCT02921971	Trial comparisons
SAR156597 vs Placebo: serious adverse events	4 of 48	5 of 49	97	NCT02921971	Trial comparisons
Mycophenolate Mofetil + Belimumab vs Mycophenolate Mofetil + Saline (Placebo): deaths from any cause	0 of 10	0 of 10	20	NCT01670565	Trial comparisons
Mycophenolate Mofetil + Belimumab vs Mycophenolate Mofetil + Saline (Placebo): serious adverse events	0 of 10	2 of 10	20	NCT01670565	Trial comparisons
Tofacitinib Double Blind 0-24 Weeks vs Placebo Double Blind 0-24 Weeks: Number of Participants Who Experience Grade 3 or Higher Adverse Events That Occur at or Before Week 24	0 of 10	0 of 5	15	NCT03274076	Trial comparisons
TEPEZZA 20 mg/kg vs Placebo: Number of Participants Experiencing a Treatment Emergent Adverse Event (TEAE) Through Week 24	0 of 1	2 of 2	3	NCT04478994	Trial comparisons
TEPEZZA 20 mg/kg vs Placebo: deaths from any cause	0 of 1	0 of 2	3	NCT04478994	Trial comparisons
TEPEZZA 20 mg/kg vs Placebo: serious adverse events	0 of 1	1 of 2	3	NCT04478994	Trial comparisons

Papers from its trials12

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
17974598	RESULT	1	PubMed
40088930	DERIVED	1	PubMed
38652570	DERIVED	1	PubMed
38460548	DERIVED	1	PubMed
38251533	DERIVED	1	PubMed
36637209	DERIVED	1	PubMed
35943798	DERIVED	1	PubMed
32963047	DERIVED	1	PubMed
30886964	DERIVED	1	PubMed
29306872	DERIVED	1	PubMed

1 to 10 of 12

See all 12 papers from its trials

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_0005019

GET https://conductscience.org/api/diseases/v1/MONDO_0005019/drugs?page=2

API reference For AI assistants