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.
引用
收藏
页数:21
相关论文
共 50 条
  • [21] Eosinophil cationic protein in Henoch-Schönlein purpura
    Castellazzi Massimo Luca
    Emanuela Anna Laicini
    Emilio F. Fossali
    World Journal of Pediatrics, 2014, 10 : 90 - 91
  • [22] Henoch-Schönlein purpura: a case with atypical presentation
    Mukaddes Kalyoncu
    Murat Cakir
    Erol Erduran
    Aysenur Okten
    Rheumatology International, 2006, 26 : 669 - 671
  • [23] Erratum to: Pathogenesis of Henoch-Schönlein purpura nephritis
    Keith K. Lau
    Hitoshi Suzuki
    Jan Novak
    Robert J. Wyatt
    Pediatric Nephrology, 2010, 25 (1) : 179 - 179
  • [24] Treatment of children with Henoch-Schönlein purpura nephritis with mycophenolate mofetil
    Yue Du
    Ling Hou
    Chengguang Zhao
    Mei Han
    Yubin Wu
    Pediatric Nephrology, 2012, 27 : 765 - 771
  • [25] Intestinal dysbiosis featuring abundance of Streptococcus associates with Henoch-Schönlein purpura nephritis (IgA vasculitis with nephritis) in adult
    Jiaxing Tan
    Zhengxia Zhong
    Yi Tang
    Wei Qin
    BMC Nephrology, 23
  • [26] Periumbilical Purpura Prior to Gastrointestinal Involvement in Henoch-Schönlein Purpura
    Patrick J. Brown
    Justin M. Haught
    Joseph C. English
    American Journal of Clinical Dermatology, 2009, 10 : 127 - 130
  • [27] Old and New Treatment Options in IgA Nephropathy and Henoch Schönlein Purpura Nephritis/IgA Vasculitis in Children
    Peruzzi L.
    Cocchi E.
    Tarizzo F.
    Current Treatment Options in Pediatrics, 2019, 5 (3) : 236 - 254
  • [28] Serum levels of galactose-deficient IgA in children with IgA nephropathy and Henoch-Schönlein purpura
    Keith K. Lau
    Robert J. Wyatt
    Zina Moldoveanu
    Milan Tomana
    Bruce A. Julian
    Ronald J. Hogg
    Jeannette Y. Lee
    Wen-Qiang Huang
    Jiri Mestecky
    Jan Novak
    Pediatric Nephrology, 2007, 22 : 2067 - 2072
  • [29] A case of Henoch-Schönlein purpura associated with scrub typhus
    Jae Hyoung Im
    Suk Jin Choi
    Moon-Hyun Chung
    Seung Yun Lee
    Young Kyoung Park
    Hea Yoon Kwon
    Ji Hyeon Baek
    Jin-Soo Lee
    BMC Infectious Diseases, 20
  • [30] Henoch-Schönlein purpura in Wiskott-Aldrich syndrome
    A. Duzova
    Rezan Topaloglu
    Ozden Sanal
    Sebnem Kılıc
    Cinzia Mazza
    Nesrin Besbas
    Aysin Bakkaloglu
    Pediatric Nephrology, 2001, 16 : 500 - 502