{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T16:53:35Z","timestamp":1792601615805,"version":"4.5.0"},"reference-count":128,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2024,11,4]],"date-time":"2024-11-04T00:00:00Z","timestamp":1730678400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"FOREUM (Program Foundation for Research in Rheumatology)","award":["RD08\/0075 (REI)"],"award-info":[{"award-number":["RD08\/0075 (REI)"]}]},{"name":"FOREUM (Program Foundation for Research in Rheumatology)","award":["RD12\/0009\/0013"],"award-info":[{"award-number":["RD12\/0009\/0013"]}]},{"name":"FOREUM (Program Foundation for Research in Rheumatology)","award":["RD16\/0012"],"award-info":[{"award-number":["RD16\/0012"]}]},{"name":"\u201cInstituto de Salud Carlos III\u201d (ISCIII) (Spain)","award":["RD08\/0075 (REI)"],"award-info":[{"award-number":["RD08\/0075 (REI)"]}]},{"name":"\u201cInstituto de Salud Carlos III\u201d (ISCIII) (Spain)","award":["RD12\/0009\/0013"],"award-info":[{"award-number":["RD12\/0009\/0013"]}]},{"name":"\u201cInstituto de Salud Carlos III\u201d (ISCIII) (Spain)","award":["RD16\/0012"],"award-info":[{"award-number":["RD16\/0012"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Objective: IgA vasculitis (IgAV), previously named as Henoch\u2013Sch\u00f6nlein 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\u201330% 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\u2013glucose 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.<\/jats:p>","DOI":"10.3390\/jcm13216621","type":"journal-article","created":{"date-parts":[[2024,11,4]],"date-time":"2024-11-04T10:57:20Z","timestamp":1730717840000},"page":"6621","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":73,"title":["IgA Vasculitis (Henoch\u2013Sch\u00f6nlein Purpura): An Update on Treatment"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7748-853X","authenticated-orcid":false,"given":"Santos","family":"Casta\u00f1eda","sequence":"first","affiliation":[{"name":"Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain"},{"name":"Department of Medicine, Universidad Aut\u00f3noma de Madrid (UAM), 28049 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Quiroga-Colina","sequence":"additional","affiliation":[{"name":"Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paz","family":"Floranes","sequence":"additional","affiliation":[{"name":"Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miren","family":"Uriarte-Ecenarro","sequence":"additional","affiliation":[{"name":"Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Valero-Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esther F.","family":"Vicente-Rabaneda","sequence":"additional","affiliation":[{"name":"Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain"},{"name":"Department of Medicine, Universidad Aut\u00f3noma de Madrid (UAM), 28049 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7924-7406","authenticated-orcid":false,"given":"Miguel A.","family":"Gonz\u00e1lez-Gay","sequence":"additional","affiliation":[{"name":"Department of Medicine and Psychiatry, School of Medicine, University of Cantabria, 39011 Santander, Spain"},{"name":"Rheumatology Division, IIS-Fundaci\u00f3n Jim\u00e9nez D\u00edaz, 28040 Madrid, Spain"},{"name":"Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg 2000, South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,11,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/art.37715","article-title":"2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides","volume":"65","author":"Jennette","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.1097\/01.ASN.0000013883.99976.22","article-title":"Henoch-Sch\u00f6nlein Purpura in adults: Outcome and prognostic factors","volume":"13","author":"Pillebout","year":"2002","journal-title":"J. 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