High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease

The immunomodulatory potential and low incidence of severe side effects of high-dose intravenous immunoglobulin (IVIg) treatment led to its successful application in a variety of dermatological autoimmune diseases over the last two decades. IVIg is usually administered at a dose of 2 g per kg body w...

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Main Authors: Jochen H. O. Hoffmann, Alexander H. Enk
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-06-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fimmu.2019.01090/full
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author Jochen H. O. Hoffmann
Alexander H. Enk
author_facet Jochen H. O. Hoffmann
Alexander H. Enk
author_sort Jochen H. O. Hoffmann
collection DOAJ
description The immunomodulatory potential and low incidence of severe side effects of high-dose intravenous immunoglobulin (IVIg) treatment led to its successful application in a variety of dermatological autoimmune diseases over the last two decades. IVIg is usually administered at a dose of 2 g per kg body weight distributed over 2–5 days every 4 weeks. They are most commonly used as a second- or third-line treatment in dermatological autoimmune disease (pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, dermatomyositis, systemic vasculitis, and systemic lupus erythematosus). However, first-line treatment may be warranted in special circumstances like concomitant malignancy, a foudroyant clinical course, and contraindications against alternative treatments. Furthermore, IVIg can be considered first line in scleromyxedema. Production of IVIg for medical use is strictly regulated to ensure a low risk of pathogen transmission and comparable quality of individual batches. More common side effects include nausea, headache, fatigue, and febrile infusion reactions. Serious side effects are rare and include thrombosis and embolism, pulmonary edema, renal failure, aseptic meningitis, and severe anaphylactic reactions. Regarding the mechanism of action, one can discriminate between functions of the Fcγ region and the F(ab)2 region and their effects on a cellular level. These functions are not mutually exclusive, and more than one pathway may contribute to the beneficial effects. Here, we present a historical background, details on manufacturing, hypotheses on the mechanisms of action, information on the clinical application in the abovementioned conditions, and a brief outlook on future directions of IVIg treatment in dermatology.
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spelling doaj.art-28d67bfb46694f0096582b0bb5a6ab042022-12-21T19:03:58ZengFrontiers Media S.A.Frontiers in Immunology1664-32242019-06-011010.3389/fimmu.2019.01090454125High-Dose Intravenous Immunoglobulin in Skin Autoimmune DiseaseJochen H. O. HoffmannAlexander H. EnkThe immunomodulatory potential and low incidence of severe side effects of high-dose intravenous immunoglobulin (IVIg) treatment led to its successful application in a variety of dermatological autoimmune diseases over the last two decades. IVIg is usually administered at a dose of 2 g per kg body weight distributed over 2–5 days every 4 weeks. They are most commonly used as a second- or third-line treatment in dermatological autoimmune disease (pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, dermatomyositis, systemic vasculitis, and systemic lupus erythematosus). However, first-line treatment may be warranted in special circumstances like concomitant malignancy, a foudroyant clinical course, and contraindications against alternative treatments. Furthermore, IVIg can be considered first line in scleromyxedema. Production of IVIg for medical use is strictly regulated to ensure a low risk of pathogen transmission and comparable quality of individual batches. More common side effects include nausea, headache, fatigue, and febrile infusion reactions. Serious side effects are rare and include thrombosis and embolism, pulmonary edema, renal failure, aseptic meningitis, and severe anaphylactic reactions. Regarding the mechanism of action, one can discriminate between functions of the Fcγ region and the F(ab)2 region and their effects on a cellular level. These functions are not mutually exclusive, and more than one pathway may contribute to the beneficial effects. Here, we present a historical background, details on manufacturing, hypotheses on the mechanisms of action, information on the clinical application in the abovementioned conditions, and a brief outlook on future directions of IVIg treatment in dermatology.https://www.frontiersin.org/article/10.3389/fimmu.2019.01090/fullscleromyxedemavasculitisIVIgbullous pemhigoidpemphigus vulgarisepidermolysis bullosa acquisita
spellingShingle Jochen H. O. Hoffmann
Alexander H. Enk
High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease
Frontiers in Immunology
scleromyxedema
vasculitis
IVIg
bullous pemhigoid
pemphigus vulgaris
epidermolysis bullosa acquisita
title High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease
title_full High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease
title_fullStr High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease
title_full_unstemmed High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease
title_short High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease
title_sort high dose intravenous immunoglobulin in skin autoimmune disease
topic scleromyxedema
vasculitis
IVIg
bullous pemhigoid
pemphigus vulgaris
epidermolysis bullosa acquisita
url https://www.frontiersin.org/article/10.3389/fimmu.2019.01090/full
work_keys_str_mv AT jochenhohoffmann highdoseintravenousimmunoglobulininskinautoimmunedisease
AT alexanderhenk highdoseintravenousimmunoglobulininskinautoimmunedisease