Disease · MONDO_0011776

CINCA syndrome

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. CINCA syndrome. Diseases, record MONDO_0011776. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0011776

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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: CINCA syndrome; Open Targets
Open Targets ID: MONDO_0011776; Open Targets
Other names: CINCA/NOMID, Chronic infantile neurological cutaneous articular syndrome, IOMID syndrome, NOMID, NOMID syndrome, Neonatal-Onset Multisystem Inflammatory Disease, Prieur-Griscelli syndrome, chronic infantile neurological cutaneous and articular syndrome, chronic neurologic cutaneous and articular syndrome, cryopyrin-associated periodic syndrome 3, infantile-onset multisystem inflammatory disease; Open Targets
Disease Ontology: DOID:0090029; Disease Ontology
NCI Thesaurus: C116380; NCI Thesaurus
Orphanet: ORPHA:1451; Orphanet
Highest stage of its drugs: Approval, for 1 of its 3 drugs, from DailyMed; Open Targets

Drugs3

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
ANAKINRA	CHEMBL1201570	Protein	Approval	DailyMed	2	1	1	Drugs and targets
CANAKINUMAB	CHEMBL1201834	Antibody	Phase 3	ClinicalTrials.gov	4	4	4	Drugs and targets
RILONACEPT	CHEMBL1201830	Protein	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

Targets2

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
IL1B	Interleukin-1 beta	2	Interleukin-1 beta inhibitor	Phase 3	https://conductscience.org/library/genes/3553	Drugs and targets
IL1R1	Interleukin-1 receptor type 1	1	Interleukin-1 receptor antagonist	Approval	https://conductscience.org/library/genes/3554	Drugs and targets

Trials6

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
NCT00685373	Efficacy and Safety of ACZ885 in Patients With the Following Cryopyrin-associated Periodic Syndromes: Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, or Neonatal Onset Multisystem Inflammatory Disease	Phase 3	166	2011-05-27	Cryopyrin-Associated Periodic Syndromes, Familial Cold Autoinflammatory Syndrome, Muckle Wells Syndrome, Neonatal Onset Multisystem Inflammatory Disease	CANAKINUMAB	Its record names the disease	ClinicalTrials.gov
NCT00069329	Anakinra to Treat Patients With Neonatal Onset Multisystem Inflammatory Disease	Phase 1/2	43	2016-12-01	Nervous System Malformations, Arthropathy, Neurogenic, Urticaria, Papilledema	ANAKINRA	Its record names the disease	ClinicalTrials.gov
NCT00094900	Interleukin-1 Trap to Treat Autoinflammatory Diseases	Phase 2	11	2013-07-10	Inflammation, Familial Mediterranean Fever, Still's Disease, Adult-Onset	RILONACEPT	Its record names the disease	ClinicalTrials.gov
NCT00770601	Canakinumab to Treat Neonatal-Onset Multisystem Inflammatory Disease	Phase 3	7	2021-06-10	NOMID, CINCA Syndrome	CANAKINUMAB	Its record names the disease	ClinicalTrials.gov
NCT00487708	Safety, Efficacy, Pharmacokinetics, Pharmacodynamics of ACZ885 in Patients With NALP3 Mutations and Clinical Symptoms	Phase 2	34		NALP3 Mutation	CANAKINUMAB	Its record names the disease	ClinicalTrials.gov
NCT00991146	Efficacy and Safety Study of Canakinumab Administered for 6 Months (24 Weeks) in Japanese Patients With Cryopyrin-associated Periodic Syndromes Followed by an Extension Phase	Phase 3	19		Cryopyrin-associated Periodic Syndromes, Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, Neonatal Onset Multisystem Inflammatory Disease	CANAKINUMAB	Its record names the disease	ClinicalTrials.gov

Comparisons in its trials0

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.

None in this release.

Papers from its trials10

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
23380020	RESULT	1	PubMed
28118536	DERIVED	1	PubMed
27143789	DERIVED	1	PubMed
25223501	DERIVED	1	PubMed
24906637	DERIVED	1	PubMed
22294344	DERIVED	1	PubMed
21859692	DERIVED	1	PubMed
21356079	DERIVED	1	PubMed
18668591	DERIVED	1	PubMed
16899778	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_0011776

GET https://conductscience.org/api/diseases/v1/MONDO_0011776/drugs

API reference For AI assistants