IgA Vasculitis (Henoch-Schönlein Purpura): An Update on Treatment

被引:1
|
作者
Castaneda, Santos [1 ,2 ]
Quiroga-Colina, Patricia [1 ]
Floranes, Paz [1 ]
Uriarte-Ecenarro, Miren [1 ]
Valero-Martinez, Cristina [1 ]
Vicente-Rabaneda, Esther F. [1 ,2 ]
Gonzalez-Gay, Miguel A. [3 ,4 ,5 ]
机构
[1] H Univ Princesa, Rheumatol Div, IIS Princesa, Madrid 28006, Spain
[2] Univ Autonoma Madrid UAM, Dept Med, Madrid 28049, Spain
[3] Univ Cantabria, Sch Med, Dept Med & Psychiat, Santander 39011, Spain
[4] IIS Fdn Jimenez Diaz, Rheumatol Div, Madrid 28040, Spain
[5] Univ Witwatersrand, Fac Hlth Sci, Sch Physiol, Cardiovasc Pathophysiol & Genom Res Unit, ZA-2000 Johannesburg, South Africa
关键词
Henoch-Sch & ouml; nlein purpura; IgA vasculitis; IgA vasculitis nephritis; glucocorticoids; cyclosporine A; tacrolimus; mycophenolate mofetil; cyclophosphamide; rituximab; plasma exchange; immunoglobulins; experimental therapies; HENOCH-SCHONLEIN PURPURA; IMMUNOGLOBULIN-A VASCULITIS; ADULT PATIENTS; PLASMA-EXCHANGE; MYCOPHENOLATE-MOFETIL; CLINICAL SPECTRUM; CONTROLLED-TRIAL; CYCLOSPORINE-A; PULSE THERAPY; DOUBLE-BLIND;
D O I
10.3390/jcm13216621
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Objective: IgA vasculitis (IgAV), previously named as Henoch-Sch & ouml;nlein 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.
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页数:21
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