IgA vasculitis (Henoch-Schönlein purpura): an update on treatment

Objective: IgA vasculitis (IgAV), previously named as Henoch-Schönlein purpura, is the most frequent systemic vasculitis in children. In adults, IgAV is less common although it is associated with more severe disease. In fact, the frequency of glomerulonephritis (referred to as IgAV nephritis) in adu...

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Autores: Castañeda, Santos, Qukiroga-Colina, Patricia, Floranes, Paz, Uriarte-Ecenarro, Miren, Valero-Martínez, Cristina, Vicente-Rabaneda, Esther F., González-Gay Mantecón, Miguel Ángel
Tipo de recurso: artículo
Fecha de publicación:2024
País:España
Institución:Universidad de Cantabria (UC)
Repositorio:UCrea Repositorio Abierto de la Universidad de Cantabria
Idioma:inglés
OAI Identifier:oai:repositorio.unican.es:10902/35844
Acceso en línea:https://hdl.handle.net/10902/35844
Access Level:acceso abierto
Palabra clave:Henoch–Schönlein purpura
IgA vasculitis
IgA vasculitis nephritis
Glucocorticoids
Cyclosporine A
Tacrolimus
Mycophenolate mofetil
Cyclophosphamide
Rituximab
Pmmunoglobulins
Experimental therapies
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spelling IgA vasculitis (Henoch-Schönlein purpura): an update on treatmentCastañeda, SantosQukiroga-Colina, PatriciaFloranes, PazUriarte-Ecenarro, MirenValero-Martínez, CristinaVicente-Rabaneda, Esther F.González-Gay Mantecón, Miguel ÁngelHenoch–Schönlein purpuraIgA vasculitisIgA vasculitis nephritisGlucocorticoidsCyclosporine ATacrolimusMycophenolate mofetilCyclophosphamideRituximabRituximabPmmunoglobulinsExperimental therapiesObjective: IgA vasculitis (IgAV), previously named as Henoch-Schönlein purpura, is the most frequent systemic vasculitis in children. In adults, IgAV is less common although it is associated with more severe disease. In fact, the frequency of glomerulonephritis (referred to as IgAV nephritis) in adults is higher than in children and tends to present more severely, with around 10-30% of those affected eventually progressing to end-stage renal disease. In this review, we describe the pathophysiology, main clinical features, diagnosis of the disease, and latest clinical data regarding IgAV therapy. Methods: A narrative literature review, primarily based on articles published in PubMed, was conducted. In addition to discussing the main aspects of glucocorticoids and conventional disease-modifying drugs used in the management of IgAV, this review focuses on the latest information reported regarding biologics and potential future therapies. Results: Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. Colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations. Several immunomodulatory agents, such as cyclosporine A, tacrolimus, and mycophenolate mofetil, have shown favorable results as glucocorticoid-sparing agents. Leflunomide has shown promising results but requires further study. The use of rituximab has demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease in children and adults with IgAV. Immunoglobulins and plasma exchange therapy can also be useful in difficult and life-threatening situations. Other potential therapies with encouraging results include TRF-budesonide, B-cell-directed therapy, B-cell-depleting agents, sodium-glucose cotransporter-2 inhibitors, endothelin receptor antagonists, and complement pathway inhibitors. Conclusions: Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. The role of various immunomodulatory therapies, such as calcineurin inhibitors and mycophenolate mofetil, remains promising, while rituximab reduces the long-term side effects of glucocorticoids and can help achieve disease remission. Other potential therapies with encouraging results require further research.This line of research has been partially supported by FOREUM (Program Foundation for Research in Rheumatology) for the “START Project”, granted to Dr. Nicolò Pipitone (Reggio Emilia, Italy) (https://www.foreum.org/projects.cfm?projectid=142 accessed on 3 October 2024), in which SC and MAG-G are the main Spanish collaborators Moreover, this line of research has also been supported in part by RETICS Programs, RD08/0075 (REI), RD12/0009/0013 and RD16/0012 from “Instituto de Salud Carlos III” (ISCIII) (Spain). Nonetheless, this study did not receive any specific grant from funding agencies in the commercial or not-for-profit sectors.MDPIUniversidad de Cantabria20242024-01-01journal articlehttp://purl.org/coar/resource_type/c_6501NAhttp://purl.org/coar/version/c_be7fb7dd8ff6fe43info:eu-repo/semantics/articlehttps://hdl.handle.net/10902/35844Journal of Clinical Medicine, 2024, 13, 6621reponame:UCrea Repositorio Abierto de la Universidad de Cantabriainstname:Universidad de Cantabria (UC)Inglésengopen accesshttp://purl.org/coar/access_right/c_abf2Attribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:repositorio.unican.es:10902/358442026-06-02T12:39:31Z
dc.title.none.fl_str_mv IgA vasculitis (Henoch-Schönlein purpura): an update on treatment
title IgA vasculitis (Henoch-Schönlein purpura): an update on treatment
spellingShingle IgA vasculitis (Henoch-Schönlein purpura): an update on treatment
Castañeda, Santos
Henoch–Schönlein purpura
IgA vasculitis
IgA vasculitis nephritis
Glucocorticoids
Cyclosporine A
Tacrolimus
Mycophenolate mofetil
Cyclophosphamide
Rituximab
Rituximab
Pmmunoglobulins
Experimental therapies
title_short IgA vasculitis (Henoch-Schönlein purpura): an update on treatment
title_full IgA vasculitis (Henoch-Schönlein purpura): an update on treatment
title_fullStr IgA vasculitis (Henoch-Schönlein purpura): an update on treatment
title_full_unstemmed IgA vasculitis (Henoch-Schönlein purpura): an update on treatment
title_sort IgA vasculitis (Henoch-Schönlein purpura): an update on treatment
dc.creator.none.fl_str_mv Castañeda, Santos
Qukiroga-Colina, Patricia
Floranes, Paz
Uriarte-Ecenarro, Miren
Valero-Martínez, Cristina
Vicente-Rabaneda, Esther F.
González-Gay Mantecón, Miguel Ángel
author Castañeda, Santos
author_facet Castañeda, Santos
Qukiroga-Colina, Patricia
Floranes, Paz
Uriarte-Ecenarro, Miren
Valero-Martínez, Cristina
Vicente-Rabaneda, Esther F.
González-Gay Mantecón, Miguel Ángel
author_role author
author2 Qukiroga-Colina, Patricia
Floranes, Paz
Uriarte-Ecenarro, Miren
Valero-Martínez, Cristina
Vicente-Rabaneda, Esther F.
González-Gay Mantecón, Miguel Ángel
author2_role author
author
author
author
author
author
dc.contributor.none.fl_str_mv Universidad de Cantabria
dc.subject.none.fl_str_mv Henoch–Schönlein purpura
IgA vasculitis
IgA vasculitis nephritis
Glucocorticoids
Cyclosporine A
Tacrolimus
Mycophenolate mofetil
Cyclophosphamide
Rituximab
Rituximab
Pmmunoglobulins
Experimental therapies
topic Henoch–Schönlein purpura
IgA vasculitis
IgA vasculitis nephritis
Glucocorticoids
Cyclosporine A
Tacrolimus
Mycophenolate mofetil
Cyclophosphamide
Rituximab
Rituximab
Pmmunoglobulins
Experimental therapies
description Objective: IgA vasculitis (IgAV), previously named as Henoch-Schönlein purpura, is the most frequent systemic vasculitis in children. In adults, IgAV is less common although it is associated with more severe disease. In fact, the frequency of glomerulonephritis (referred to as IgAV nephritis) in adults is higher than in children and tends to present more severely, with around 10-30% of those affected eventually progressing to end-stage renal disease. In this review, we describe the pathophysiology, main clinical features, diagnosis of the disease, and latest clinical data regarding IgAV therapy. Methods: A narrative literature review, primarily based on articles published in PubMed, was conducted. In addition to discussing the main aspects of glucocorticoids and conventional disease-modifying drugs used in the management of IgAV, this review focuses on the latest information reported regarding biologics and potential future therapies. Results: Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. Colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations. Several immunomodulatory agents, such as cyclosporine A, tacrolimus, and mycophenolate mofetil, have shown favorable results as glucocorticoid-sparing agents. Leflunomide has shown promising results but requires further study. The use of rituximab has demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease in children and adults with IgAV. Immunoglobulins and plasma exchange therapy can also be useful in difficult and life-threatening situations. Other potential therapies with encouraging results include TRF-budesonide, B-cell-directed therapy, B-cell-depleting agents, sodium-glucose cotransporter-2 inhibitors, endothelin receptor antagonists, and complement pathway inhibitors. Conclusions: Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. The role of various immunomodulatory therapies, such as calcineurin inhibitors and mycophenolate mofetil, remains promising, while rituximab reduces the long-term side effects of glucocorticoids and can help achieve disease remission. Other potential therapies with encouraging results require further research.
publishDate 2024
dc.date.none.fl_str_mv 2024
2024-01-01
dc.type.none.fl_str_mv journal article
http://purl.org/coar/resource_type/c_6501
NA
http://purl.org/coar/version/c_be7fb7dd8ff6fe43
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://hdl.handle.net/10902/35844
url https://hdl.handle.net/10902/35844
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv MDPI
publisher.none.fl_str_mv MDPI
dc.source.none.fl_str_mv Journal of Clinical Medicine, 2024, 13, 6621
reponame:UCrea Repositorio Abierto de la Universidad de Cantabria
instname:Universidad de Cantabria (UC)
instname_str Universidad de Cantabria (UC)
reponame_str UCrea Repositorio Abierto de la Universidad de Cantabria
collection UCrea Repositorio Abierto de la Universidad de Cantabria
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15.81155

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