Evidence map›Paper›PMID 40456554›Full record

ReviewPediatric dermatology

Blau Syndrome (Juvenile Systemic Granulomatosis): State-Of-The-Art Review.

Ou Jia Emilie Wang, Marianna Jacob, Richard I Crawford, Joseph M Lam

Abstract readReview
In one paragraph

Review in Pediatric dermatology. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

  1. Review
  2. Article
  3. Article
  4. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

4 authors.

Ou Jia Emilie WangFaculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.ORCID https://orcid.org/0009-0001-2217-0161
Marianna JacobDepartment Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada.
Richard I CrawfordDepartment Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada.
Joseph M LamDepartment Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada.ORCID https://orcid.org/0000-0001-7808-3369

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Blau syndrome (BlauS) is a rare pediatric autoinflammatory disorder due to NOD2 gain-of-function pathogenic variants characterized by a triad of granulomatous dermatitis, arthritis, and uveitis, which can progress to systemic complications if untreated. Skin and joint findings typically emerge by age 2 years, with ocular involvement appearing around age 4 years. Early biologic intervention, particularly with anti-TNF-α therapies, can prevent severe complications like joint destruction and blindness. Systemic corticosteroids serve as bridging therapy, while methotrexate and thalidomide may help but are often insufficient as monotherapy. IL-1 and IL-6 inhibitors, as well as tofacitinib, are options for refractory cases.

Indexed as

ArthritisDermatitisSynovitisUveitisChildChild, PreschoolHereditary Autoinflammatory DiseasesHumansNod2 Signaling Adaptor ProteinSarcoidosisNOD2 protein, humanNod2 Signaling Adaptor ProteinarthritisBlau syndromegranulomatous diseasesNOD2sarcoidosisuveitis

Identifiers

PMID40456554
PMCPMC12285560

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.