ArticleBMJ open gastroenterology2023
Pancreaticobiliary versus head and neck presentation of immunoglobulin G4-related disease: different sides of the same coin?
Article in BMJ open gastroenterology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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1 citing paper in PubMed, 1 citations in OpenAlex.
- 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
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6 authors at 2 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND STUDY
aimImmunoglobulin G4-related disease (IgG4-RD) is a rare immune mediated fibroinflammatory condition. Pancreaticobiliary (PB) and head and neck (HN) are two of the most commonly involved anatomical sites. It has been postulated that PB IgG4-RD and HN IgG4-RD have distinct clinical phenotypes. Whether the optimum treatment regimen or response to therapy differs between them is unknown. We aimed to assess differences between PB and HN IgG4-RD in a cohort of IgG4 disease managed by an IgG4-RD multispecialty team.
methodsWe performed a retrospective study of a prospectively maintained multidisciplinary IgG4-RD database to identify patients diagnosed with PB and HN IgG4-RD (based on initial presentation) between 2005 and 2019. The electronic patient records were reviewed. Use of immunosuppressive agents and clinical course was analysed.
results60 patients with PB IgG4-RD and 14 with HN IgG4-RD were included in the study. PB IgG4-RD was associated with older age at diagnosis 64 versus 51 years (p<0.001), higher serum IgG4 level as a multiple of upper limit of normal median (IQR) 2 (1-3.75) vs 1 (1-2), (p=0.04) and greater proportion with more than one organ involved 68% vs 33% (p=0.03). HN IgG4-RD was more likely to receive second-line therapy 71% versus 36% (p=0.03). Persistent elevation of serum IgG4 after therapy was more common in PB IgG4-RD 84% versus 43% (p=0.03).
conclusionThese findings support the contention that PB IgG4-RD and HN IgG4-RD have different clinical profiles and represent distinct subtypes of IgG4-RD.
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