| Title: |
Immunological Biomarkers to Assess Activity and Treatment Response in IgG4-Related Disease |
| Authors: |
Moya, Patricia; Lopez-Gomez, Marta; Martinez-Martinez, Laura; Park, Hye S.; Franco Leyva, Teresa; Concepción, Mar; Codes Méndez, Helena; Calvet Lacruz, Anna; Calleja, Sara; Magallares López, Berta; Castellvi, Ivan; Barros-Membrilla, Antonio J.; Bernárdez, Julia; Corominas, Hèctor; Universitat Autònoma de Barcelona. Departament de Medicina |
| Publication Year: |
2026 |
| Collection: |
Universitat Autònoma de Barcelona: Dipòsit Digital de Documents de la UAB |
| Subject Terms: |
IgG4-related disease; Disease activity; Responder Index; IgG4; Serology; Biomarkers; Cytokines; Immune profiling; PET/CT; Treatment |
| Description: |
Background and Objectives: IgG4-related disease is a chronic fibro-inflammatory condition. Despite the development of classification and responder indexes, reliable biomarkers for disease activity and therapeutic monitoring remain limited. We evaluate the performance of a panel of biomarkers, including cytokine profiles, plasmablasts and conventional markers. Materials and Methods: We conducted a cross-sectional, single-center study, involving 35 patients diagnosed with IgG4-RD. Disease activity was evaluated using the IgG4-RD Responder Index (RI), Damage Index (DI) and clinical assessment. Laboratory evaluation included serum IgG4, total IgG, CRP, ESR, eosinophils, IgE, complement levels, and cytokine profiling via multiplex immunoassay. B cell subpopulations were analyzed by flow cytometry. Statistical analyses were performed using STATA/BE 17.0. Results: Patients with active disease (RI > 4 or clinical judgment) exhibited significantly higher levels of total IgG (p = 0.02), IgG4 (p = 0.01), and IL-5 (p = 0.03). PET-positive patients showed a Th1-skewed immune profile, with elevated IFN-γ/IL-4 (p < 0.001), reduced IL-21/IFN-γ (p = 0.03), and increased eosinophils (p = 0.03). Clinician-assessed active disease was associated with higher total IgG levels (p = 0.01). Treatment-specific effects were observed: prednisone was associated with lower IgG4 and C3 levels. Notably, plasmablasts did not consistently correlate with clinical or imaging activity scores, possibly reflecting treatment status or B cell dynamics. Conclusions: This study demonstrates that cytokine ratios, particularly those involving IL-5, IL-13, IL-21, and IFN-γ, offer complementary information to traditional serological markers for IgG4-RD activity. While PET/CT-defined activity was best reflected by biomarkers of an IFN-γ-mediated pathway, the IgG4-RD RI demonstrated a stronger association with conventional humoral markers like serum IgG4 and total IgG. None of these biomarkers correlated with organ damage. |
| Document Type: |
article in journal/newspaper |
| File Description: |
application/pdf |
| Language: |
English |
| ISSN: |
62020323; 16489144 |
| Relation: |
Medicina; Vol. 62, Num. 2 (February 2026), art. 323; https://ddd.uab.cat/record/326471; urn:10.3390/medicina62020323; urn:oai:ddd.uab.cat:326471; urn:articleid:16489144v62n2e323 |
| Availability: |
https://ddd.uab.cat/record/326471 |
| Rights: |
open access ; Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original. ; https://creativecommons.org/licenses/by/4.0/ |
| Accession Number: |
edsbas.6B0C086D |
| Database: |
BASE |