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A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab

Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears...

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Autores principales: Backhus, Johanna, Neumann, Christian, Perkhofer, Lukas, Schulte, Lucas A, Mayer, Benjamin, Seufferlein, Thomas, Müller, Martin, Kleger, Alexander
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8004657/
https://www.ncbi.nlm.nih.gov/pubmed/33807051
http://dx.doi.org/10.3390/jcm10061329
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author Backhus, Johanna
Neumann, Christian
Perkhofer, Lukas
Schulte, Lucas A
Mayer, Benjamin
Seufferlein, Thomas
Müller, Martin
Kleger, Alexander
author_facet Backhus, Johanna
Neumann, Christian
Perkhofer, Lukas
Schulte, Lucas A
Mayer, Benjamin
Seufferlein, Thomas
Müller, Martin
Kleger, Alexander
author_sort Backhus, Johanna
collection PubMed
description Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.
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spelling pubmed-80046572021-03-29 A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab Backhus, Johanna Neumann, Christian Perkhofer, Lukas Schulte, Lucas A Mayer, Benjamin Seufferlein, Thomas Müller, Martin Kleger, Alexander J Clin Med Article Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy. MDPI 2021-03-23 /pmc/articles/PMC8004657/ /pubmed/33807051 http://dx.doi.org/10.3390/jcm10061329 Text en © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Backhus, Johanna
Neumann, Christian
Perkhofer, Lukas
Schulte, Lucas A
Mayer, Benjamin
Seufferlein, Thomas
Müller, Martin
Kleger, Alexander
A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab
title A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab
title_full A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab
title_fullStr A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab
title_full_unstemmed A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab
title_short A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab
title_sort follow-up study of a european igg4-related disease cohort treated with rituximab
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8004657/
https://www.ncbi.nlm.nih.gov/pubmed/33807051
http://dx.doi.org/10.3390/jcm10061329
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