التفاصيل البيبلوغرافية
| العنوان: |
Pancreaticobiliary versus head and neck presentation of immunoglobulin G4-related disease: different sides of the same coin? |
| المؤلفون: |
Awadelkarim, Bidour, Vila, Josephine, Nayar, Manu K, Leeds, John S, Griffiths, Bridget, Oppong, Kofi W |
| المصدر: |
BMJ Open Gastroenterology ; volume 10, issue 1, page e000961 ; ISSN 2054-4774 |
| بيانات النشر: |
BMJ |
| سنة النشر: |
2023 |
| مصطلحات موضوعية: |
Gastroenterology |
| الوصف: |
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. |
| نوع الوثيقة: |
article in journal/newspaper |
| اللغة: |
English |
| DOI: |
10.1136/bmjgast-2022-000961 |
| الإتاحة: |
https://doi.org/10.1136/bmjgast-2022-000961Test |
| حقوق: |
http://creativecommons.org/licenses/by-nc/4.0Test/ |
| رقم الانضمام: |
edsbas.1FB1EDE2 |
| قاعدة البيانات: |
BASE |
