SynthesisPediatric rheumatology online journal2016
IgG4-related disease: a systematic review of this unrecognized disease in pediatrics.
Synthesis in Pediatric rheumatology online journal, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 67 papers, 1 of them a synthesis that pooled it.
What it found
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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.
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.
Who cites it
67 citing papers in PubMed, 1 synthesis or guideline pooled it, 165 citations in OpenAlex.
- European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations.United European gastroenterology journal · 2020Guideline
- Trial
- Giant paratesticular immunoglobulin G4-related disease mimicking malignancy: A case report.Urology case reports · 2026Article
- Pediatric orbital IgG4-related disease: A case series.American journal of ophthalmology case reports · 2026Article
- Proptosis Revealing a Neurological Form of IgG4-Related Disease: A Case Report and Literature Review.Cureus · 2026Article
- Immunoglobulin-G4 Related Disease: A Rare Entity with Many Clinical Faces - Literature Review and Case Illustration.Mediterranean journal of rheumatology · 2026Review
- Case report: The masquerading spectrum: a pediatric case series of IgG4-related disease.Frontiers in immunology · 2026Observational
- Histologically confirmed immunoglobulin G4-related hypophysitis in an adolescent girl: a case report with review of literature.Endocrine journal · 2025Review
- IgG4-Related Disease in Childhood: Clinical Presentation, Management, and Diagnostic Challenges.Children (Basel, Switzerland) · 2025Review
- IgG4-Related Disease: Current and Future Insights into Pathological Diagnosis.International journal of molecular sciences · 2025Review
- IgG4-related ophthalmic disease presenting as posterior scleritis in a pediatric patient.Journal of ophthalmic inflammation and infection · 2025Article
- The Management of IgG4-Related Disease in Children: A Systematic Review.Children (Basel, Switzerland) · 2025Review
- Tubulointerstitial nephritis in children and adolescents.Pediatric nephrology (Berlin, Germany) · 2025Review
- The relationship between immunoglobulin G4-related disease and malignancy.Reumatologia · 2025Article
- Prevalence of Elevated IgG4 in Patients with Nasopharynx and Laryngopharynx Diseases: A Systematic Review and Meta-Analysis.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2025Article
- Recurrent Pulmonary Hemorrhage due to Fibrosing Mediastinitis due to Probable IgG4.Indian journal of pediatrics · 2024Article
- Inflammatory Diseases of the Eye, Bowel, and Bone in Children.Indian journal of pediatrics · 2024Review
- Sex as a predictor of clinical phenotype and determinant of immune response in IgG4-related disease: a retrospective study of patients fulfilling the American College of Rheumatology-European League Against Rheumatism classification criteria.The Lancet. Rheumatology · 2024Article
- Article
- IgG4-related disease-rare but you should not forget it.Advances in rheumatology (London, England) · 2024Review
7 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundImmunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with an unclear pathophysiological mechanism affecting different parts of the body. If untreated, the disease can lead to fibrosis and irreversible organ damage. IgG4-RD mostly has been described in adults, hence it is generally unknown among pediatricians. This systematic search of the literature provides an overview of all reports published on IgG4-RD in children in order to create awareness of IgG4-RD in pediatrics and to emphasize the broad clinical presentation of this disease.
methodsA systematic literature search of Embase, Medline, Web-of-Science, PubMed publisher, Cochrane and Google Scholar was performed for case reports on IgG4-RD in children.
resultsOf total 740 articles identified by the search, 22 case reports including 25 cases of IgG4-RD in children were found. The median age of the children was 13 years, of which 64 % were girls. IgG4-related orbital disease (44 %) and autoimmune pancreatitis type 1/IgG4-related pancreatitis (12 %) predominantly occurred. Less frequently, other manifestations as pulmonary manifestation, cholangitis and lymphadenopathy were also found. Almost all cases were histologically proven. Prednisone was the first choice of treatment leading to favorable clinical response in 83 % of the cases. Maintenance therapy with steroid sparing agents was required in 43 % of the cases needing therapy. Rituximab was successful in all 4 cases, whereas, the disease modifying rheumatic drugs (DMARDs) mycophenolate mofetil, azathioprine and methotrexate were effective in almost 50 % of the cases.
conclusionIgG4-RD in children is a generally unknown disease among pediatricians, but several pediatric cases have been described. Prednisone is the first choice of treatment leading to disease remission in the majority of the cases. DMARDs and rituximab are alternative effective steroid sparing agents with more positive evidence for the latter.
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Registered trials
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.