RT Journal Article
SR Electronic
T1 Pancreaticobiliary versus head and neck presentation of immunoglobulin G4-related disease: different sides of the same coin?
JF BMJ Open Gastroenterology
JO BMJ Open Gastro
FD BMJ Publishing Group Ltd
SP e000961
DO 10.1136/bmjgast-2022-000961
VO 10
IS 1
A1 Bidour Awadelkarim
A1 Josephine Vila
A1 Manu K Nayar
A1 John S Leeds
A1 Bridget Griffiths
A1 Kofi W Oppong
YR 2023
UL http://bmjopengastro.bmj.com//content/10/1/e000961.abstract
AB Background and study aim Immunoglobulin 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.Methods We 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.Results 60 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).Conclusion These findings support the contention that PB IgG4-RD and HN IgG4-RD have different clinical profiles and represent distinct subtypes of IgG4-RD.No data are available.