Evidence map›Paper›PMID 33746107›Full record

SynthesisWorld neurosurgery2021

IgG4-Related Disease of the Skull and Skull Base-A Systematic Review and Report of Two Cases.

Samuel J Cler, Nima Sharifai, Brandi Baker, Joshua L Dowling, Patrik Pipkorn, Lauren Yaeger, David B Clifford, Sonika Dahiya, Michael R Chicoine

Open access · greenAbstract readCase ReportsSystematic Review
In one paragraph

Synthesis in World neurosurgery, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed
2.4field-weighted citation impact, top 11% of its field
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

11 citing papers in PubMed, 18 citations in OpenAlex.

  1. Article
  2. IgG4-related disease involving distinctive areas of skull base: our experience.European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery · 2025
    Article
  3. Article
  4. [Analysis of diagnosis and treatment of IgG4-related disease involving the nasal cavity and skull base(with 8 case reports)].Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery · 2025
    Article
  5. 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 · 2025
    Article
  6. Article
  7. Review
  8. Article
  9. Article
  10. Article
  11. Article
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

9 authors at 1 institution in 1 country.

Samuel J ClerDepartment of Neurosurgery, Washington University School of Medicine, Washington, D.C., USA. Electronic address: sjcler@wustl.edu.
Nima SharifaiDepartment of Pathology and Immunology, Washington University School of Medicine, Washington, D.C., USA.
Brandi BakerDepartment of Neurology, Washington University School of Medicine, Washington, D.C., USA.
Joshua L DowlingDepartment of Neurosurgery, Washington University School of Medicine, Washington, D.C., USA.
Patrik PipkornDepartment of Otolaryngology, Washington University School of Medicine, Washington, D.C., USA.
Lauren YaegerBernard Becker Medical Library, Washington University School of Medicine, Washington, D.C., USA.
David B CliffordDepartment of Neurology, Washington University School of Medicine, Washington, D.C., USA; Department of Infectious Disease, Washington University School of Medicine, Washington, D.C., USA.
Sonika DahiyaDepartment of Pathology and Immunology, Washington University School of Medicine, Washington, D.C., USA.
Michael R ChicoineDepartment of Neurosurgery, Washington University School of Medicine, Washington, D.C., USA.
University of Washington · US

Funding

Washington University Center for Cellular ImagingP30CA091842 · NCI · WASHINGTON UNIVERSITY · PI AIMEE S JAMES · 2001 to 2026
$128.0M
NCI NIH HHS P30 CA091842
6 · The paper itself

Abstract

objectiveIgG4-related disease (IgG4-RD) is an inflammatory process that uncommonly can present in the skull base and calvarium and mimic a tumor but the nature of this condition is not well summarized in the neurosurgical literature.

methodsA review was performed of 2 cases of IgG4-RD in the skull base highlighting the diagnostic challenges with assessment of these skull base lesions, and a systematic review of relevant literature was carried out.

resultsA systematic review of the literature conducted in accordance with PRISMA guidelines identified 113 articles, with 184 cases of IgG4-RD in the skull base or calvarium. The most commonly affected locations include the meninges, cavernous sinus, base of the posterior fossa, clivus, and mastoid bone. Headache, visual and auditory disturbances, cranial nerve dysfunction, and seizures were the most common presenting symptoms. Medical treatment was highly successful and most commonly consisted of corticosteroids coadministered with immunosuppressive agents such as rituximab. Prevalence seemed to be equal between sexes, and serum IgG4 levels were increased in 61% of patients. Delayed diagnosis and a need for multiple biopsies were reported in numerous cases. Two cases of skull base IgG4-RD from the authors' institution show the variable presentations of this disease. More invasive surgical biopsies were required in both cases, and corticosteroid treatment led to significant clinical improvement.

conclusionsIgG4-RD is an uncommon condition with an increasing body of reported cases that can affect the skull base and calvarium and should be in the differential diagnosis, because delay in diagnosis and treatment may be common.

Indexed as

Immunoglobulin G4-Related DiseaseBrainFemaleHumansMiddle AgedSkull BaseTreatment OutcomeCalvariumIgG4-related diseaseSkull base

Identifiers

PMID33746107
PMCPMC13012661
OpenAlexW3138924914

What OpenQuestion holds

Textmetadata
LicenceTDM
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.