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Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center

Background and Objectives: Autoimmune bullous diseases (AIBDs) may be treated with intravenous immunoglobulin (IVIG) infusions. This study aimed to evaluate the benefits and safety profiles of high-dose IVIG therapy in AIBD patients, as determined by clinical remission, the glucocorticosteroid-spari...

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Autores principales: Spałek, Maciej Marek, Bowszyc-Dmochowska, Monika, Dmochowski, Marian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10385677/
https://www.ncbi.nlm.nih.gov/pubmed/37512078
http://dx.doi.org/10.3390/medicina59071265
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author Spałek, Maciej Marek
Bowszyc-Dmochowska, Monika
Dmochowski, Marian
author_facet Spałek, Maciej Marek
Bowszyc-Dmochowska, Monika
Dmochowski, Marian
author_sort Spałek, Maciej Marek
collection PubMed
description Background and Objectives: Autoimmune bullous diseases (AIBDs) may be treated with intravenous immunoglobulin (IVIG) infusions. This study aimed to evaluate the benefits and safety profiles of high-dose IVIG therapy in AIBD patients, as determined by clinical remission, the glucocorticosteroid-sparing effect, and adverse events at 12 months follow-up in a Central European university dermatology department setting. Materials and Methods: Our case series included 10 patients: five patients with pemphigus vulgaris, one with pemphigus herpetiformis, one with pemphigus foliaceus, one with bullous pemphigoid, two with epidermolysis bullosa acquisita. They underwent 4–12 monthly cycles of IVIG therapy at a dose of 2 g/kg per cycle. Results: The prednisone dosage reduction after 2, 6, and 12 months following the final IVIG course was 65.45%, 70.91%, and 76.37%, respectively. During the 12-month observation period, disease relapse was observed in 20% of patients, while others achieved complete or partial remission without or with minimal therapy. Side effects were seen in 80% of patients; they were transient and did not necessitate discontinuation of IVIG. Conclusions: IVIG demonstrates effectiveness as a treatment with a favorable safety profile. Nevertheless, its high cost remains a significant drawback, particularly in low-income countries. IVIG should be considered, especially in patients opposed to standard therapies or with contraindications to their use.
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spelling pubmed-103856772023-07-30 Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center Spałek, Maciej Marek Bowszyc-Dmochowska, Monika Dmochowski, Marian Medicina (Kaunas) Article Background and Objectives: Autoimmune bullous diseases (AIBDs) may be treated with intravenous immunoglobulin (IVIG) infusions. This study aimed to evaluate the benefits and safety profiles of high-dose IVIG therapy in AIBD patients, as determined by clinical remission, the glucocorticosteroid-sparing effect, and adverse events at 12 months follow-up in a Central European university dermatology department setting. Materials and Methods: Our case series included 10 patients: five patients with pemphigus vulgaris, one with pemphigus herpetiformis, one with pemphigus foliaceus, one with bullous pemphigoid, two with epidermolysis bullosa acquisita. They underwent 4–12 monthly cycles of IVIG therapy at a dose of 2 g/kg per cycle. Results: The prednisone dosage reduction after 2, 6, and 12 months following the final IVIG course was 65.45%, 70.91%, and 76.37%, respectively. During the 12-month observation period, disease relapse was observed in 20% of patients, while others achieved complete or partial remission without or with minimal therapy. Side effects were seen in 80% of patients; they were transient and did not necessitate discontinuation of IVIG. Conclusions: IVIG demonstrates effectiveness as a treatment with a favorable safety profile. Nevertheless, its high cost remains a significant drawback, particularly in low-income countries. IVIG should be considered, especially in patients opposed to standard therapies or with contraindications to their use. MDPI 2023-07-07 /pmc/articles/PMC10385677/ /pubmed/37512078 http://dx.doi.org/10.3390/medicina59071265 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/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 (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Spałek, Maciej Marek
Bowszyc-Dmochowska, Monika
Dmochowski, Marian
Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center
title Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center
title_full Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center
title_fullStr Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center
title_full_unstemmed Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center
title_short Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center
title_sort intravenous immunoglobulin for autoimmune bullous diseases: a case series from a central european referral center
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10385677/
https://www.ncbi.nlm.nih.gov/pubmed/37512078
http://dx.doi.org/10.3390/medicina59071265
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