Immunologic Research

, Volume 65, Issue 1, pp 186–192 | Cite as

Rituximab therapy for IgA-vasculitis with nephritis: a case series and review of the literature

  • Roberta Fenoglio
  • Carla Naretto
  • Bruno Basolo
  • Giacomo Quattrocchio
  • Michela Ferro
  • Paola Mesiano
  • Giulietta Beltrame
  • Dario Roccatello
Therapeutic Aspects in Autoimmunity


Henoch–Schonlein purpura, also called IgA-vasculitis, is a systemic small vessels vasculitis with immunoglobulin A1-dominant immune deposits. The optimal treatment remains controversial. Because IgA-vasculitis is characterized by leukocyte infiltration of the blood vessel walls along with immunoglobulin A deposition, and because glucocorticosteroids inhibit inflammatory processes, early administration of glucocorticosteroids has been postulated to be effective, but this indication remains controversial. Immunosuppressive agents (azathioprine, cyclophosphamide, cyclosporine, mycophenolate) have been used in combination with glucocorticosteroids without definitive evidence of effectiveness. The efficacy of rituximab in adult IgA-vasculitis has been reported in few cases. We described a monocentric experience on the use of rituximab in adult IgA-vasculitis with biopsy-proven nephritis. The patients achieved a complete remission of nephritis and syndromic manifestations, and no patients experienced adverse reactions. These data have been compared with the limited literature nowadays available.


IgA-vasculitis Henoch–Schonlein purpura nephritis Henoch–Schonlein treatment Rituximab B cell depletion therapy ANCA-associated vasculitis 


Compliance with ethical standards

Conflict of interest

The authors declare no conflict of interest, nor any financial interest with regard to the present paper.


  1. 1.
    Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187–92.PubMedCrossRefGoogle Scholar
  2. 2.
    Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 Revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11.PubMedCrossRefGoogle Scholar
  3. 3.
    Pillebout E, Nochy D, Thervet E. Henoch–Shönlein purpura. Nephrol Ther. 2009;5(7):663–75.PubMedCrossRefGoogle Scholar
  4. 4.
    Yang YH, Yu HH, Chiang BL. The diagnosis and classification of Henoch–Schönlein purpura: an updated review. Autoimmun Rev. 2014;13(4–5):355–8.PubMedCrossRefGoogle Scholar
  5. 5.
    Saulsbury FT. Henoch–Schönlein purpura. Curr Opin Rheumatol. 2010;22(5):598–602.PubMedCrossRefGoogle Scholar
  6. 6.
    Pillebout E, Verine J. Henoch–Schönlein purpura in the adult. Rev Med Interne. 2014;35(6):372–81.PubMedCrossRefGoogle Scholar
  7. 7.
    Calvo-Río V, Loricera J, Mata C, Martín L, Ortiz-Sanjuán F, Alvarez L, et al. Henoch–Schönlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center. Medicine (Baltimore). 2014;93(2):106–13.CrossRefGoogle Scholar
  8. 8.
    Michel BA, Hunder GG, Bloch DA, Calabrese LH. Hypersensitivity vasculitis and Henoch–Schönlein purpura: a comparison between the 2 disorders. J Rheumatol. 1992;19(5):721–8.PubMedGoogle Scholar
  9. 9.
    Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T, et al. Outcome of Henoch–Schönlein purpura 8 years after treatment with a placebo or prednisone at disease onset. Pediatr Nephrol. 2012;27(6):933–9.PubMedCrossRefGoogle Scholar
  10. 10.
    Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B. IgA vasculitis (Henoch–Shönlein purpura) in adults: diagnostic and therapeutic aspects. Autoimmun Rev. 2015;14(7):579–85.PubMedCrossRefGoogle Scholar
  11. 11.
    KDIGO clinical practice guideline for glomerulonephritis. Chapter 11: Henoch–Schönlein purpura nephritis. Kidney Int Suppl 2012;2:218–20.Google Scholar
  12. 12.
    Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M, Merenmies J, Rajantie J, Ormälä T, Turtinen J, Nuutinen M. Early prednisone therapy in Henoch–Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2006;149(2):241–7.PubMedCrossRefGoogle Scholar
  13. 13.
    Dudley J, Smith G, Llewellyn-Edwards A, Tizard E. Randomized placebo controlled trial to assess the role of early prednisone on the development and progression of Henoch–Schonlein purpura nephritis. Pediatr Nephrol. 2007;22:1457.Google Scholar
  14. 14.
    Huber AM, King J, McLaine P, Klassen T, Pothos M. A randomized, placebo-controlled trial of prednisone in early Henoch Schönlein Purpura [ISRCTN85109383]. BMC Med. 2004;2:7.PubMedPubMedCentralCrossRefGoogle Scholar
  15. 15.
    Nikibakhsh AA, Mahmoodzadeh H, Karamyyar M, Hejazi S, Noroozi M, Macooie AA. Treatment of severe Henoch–Schonlein purpura nephritis with mycophenolate mofetil. Saudi J Kidney Dis Transplant. 2014;25(4):858–63.CrossRefGoogle Scholar
  16. 16.
    Ohara S, Kawasaki Y, Miyazaki K, Ono A, Suzuki Y, Suyama K, et al. Efficacy of cyclosporine A for steroid-resistant severe Henoch–Schönlein purpura nephritis. Fukushima J Med Sci. 2013;59(2):102–7.PubMedCrossRefGoogle Scholar
  17. 17.
    Floege J, Feehally J. Treatment of IgA nephropathy and Henoch–Schönlein nephritis. Nat Rev Nephrol. 2013;9(6):320–7.PubMedCrossRefGoogle Scholar
  18. 18.
    Iaccarino L, Rampudda M, Canova M, Della Libera S, Sarzi-Puttinic P, Doria A. Mycophenolate mofetil: What is its place in the treatment of autoimmune rheumatic diseases? Autoimmun Rev. 2007;6(3):190–5.PubMedCrossRefGoogle Scholar
  19. 19.
    Halling SE, Söderberg MP, Berg UB. Treatment of severe Henoch-Schönlein and immunoglobulin A nephritis. A single center experience. Pediatr Nephrol. 2009;24(1):91–7.CrossRefGoogle Scholar
  20. 20.
    Modena V, Bianchi G, Roccatello D. Cost-effectiveness of biologic treatment for rheumatoid arthritis in clinical practice: an achievable target? Autoimmun Rev. 2013;12(8):793–5.CrossRefGoogle Scholar
  21. 21.
    Kattah AG, Fervenza FC, Roccatello D. Rituximab-based novel strategies for the treatment of immune-mediated glomerular diseases. Autoimmun Rev. 2013;12(8):854–9.PubMedCrossRefGoogle Scholar
  22. 22.
    Isenberg DA. Rituximab—it was the best of times, it was the worst of times. Autoimmun Rev. 2012;11(11):790–1.PubMedCrossRefGoogle Scholar
  23. 23.
    Kamal A, Khamashta M. The efficacy of novel B cell biologics as the future of SLE treatment: a review. Autoimmun Rev. 2014;13(11):1094–101.PubMedCrossRefGoogle Scholar
  24. 24.
    Roccatello D, Sciascia S, Baldovino S, Rossi D, Alpa M, Naretto C, Di Simone D, Simoncini M, Menegatti E. A 4-year observation in lupus nephritis patients treated with an intensified B-lymphocyte depletion without immunosuppressive maintenance treatment—clinical response compared to literature and immunological re-assessment. Autoimmun Rev. 2015;14(12):1123–30.PubMedCrossRefGoogle Scholar
  25. 25.
    Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein–Henoch purpura nephritis. Pediatr Nephrol. 1998;12(3):238–43.PubMedCrossRefGoogle Scholar
  26. 26.
    Geetha D, Specks U, Stone JH, Merkel PA, Seo P, Spiera R, et al. Rituximab for ANCA-Associated Vasculitis Immune Tolerance Network Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis with renal involvement. J Am Soc Nephrol. 2015;26(4):976–85.PubMedCrossRefGoogle Scholar
  27. 27.
    Sinico RA, Di Toma L, Radice A. Renal involvement in anti-neutrophil cytoplasmic autoantibody associated vasculitis. Autoimmun Rev. 2013;12(4):477–82.PubMedCrossRefGoogle Scholar
  28. 28.
    Gómez-Puerta JA, Quintana LF, Stone JH, Ramos-Casals M, Bosch X. B-cell depleting agents for ANCA vasculitides: a new therapeutic approach. Autoimmun Rev. 2012;11(9):646–52.PubMedCrossRefGoogle Scholar
  29. 29.
    Rutgers A, Kallenberg CG. Refractory vasculitis. Autoimmun Rev. 2011;10(11):702–6.PubMedCrossRefGoogle Scholar
  30. 30.
    Dumoitier N, Terrier B, London J, Lofek S, Mouthon L. Implication of B lymphocytes in the pathogenesis of ANCA-associated vasculitides. Autoimmun Rev. 2015;14(11):996–1004.PubMedCrossRefGoogle Scholar
  31. 31.
    Pietrogrande M, De Vita S, Zignego AL, Pioltelli P, Sansonno D, Roccatello D, et al. Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients. Autoimmun Rev. 2011;10(8):444–54.PubMedCrossRefGoogle Scholar
  32. 32.
    Pani A, Roccatello D, Fervenza F, Lin J, Sethi S. IgA nephropathy and Schonlein–Henoch purpura nephritis. In: Singh A, editor. Core concepts in parenchymal kidney disease. New York: Springer; 2014.Google Scholar
  33. 33.
    Eisenberg R, Looney RJ. The therapeutic potential of anti-CD20 “what do B-cells do?”. Clin Immunol (Orlando, FL). 2005;117(3):207–13.CrossRefGoogle Scholar
  34. 34.
    Di Gaetano N, Cittera E, Nota R, et al. Complement activation determines the therapeutic activity of rituximab in vivo. J Immunol (Baltimore, MD: 1950). 2003;171(3):1581–7.CrossRefGoogle Scholar
  35. 35.
    Ozen S, Pistorio A, Lusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Paediatric Rheumatology International Trials Organisation (PRINTO). Ann Rheum Dis. 2010;69(5):798–806.PubMedCrossRefGoogle Scholar
  36. 36.
    Bluman J, Goldman RD. Henoch–Schönlein purpura in children: limited benefit of corticosteroids. Can Fam Physician. 2014;60(11):1007–10.PubMedPubMedCentralGoogle Scholar
  37. 37.
    Kato-Okada S, Suzuki H, Inoue T, Kikuta T, Okada H. Successful prednisolone therapy in elderly patients with severe forms of Henoch–Schönlein purpura nephritis. Jpn Clin Med. 2015;6(6):5–7.PubMedPubMedCentralGoogle Scholar
  38. 38.
    Chartapisak W, Opastiraku S, Willis NS, Craig JC, Hodson EM. Prevention and treatment of renal disease in Henoch–Schönlein purpura: a systematic review. Arch Dis Child. 2009;94(2):132–7.PubMedCrossRefGoogle Scholar
  39. 39.
    Zaffanello M, Fanos V. Treatment-based literature of Henoch–Schönlein purpura nephritis in childhood. Pediatr Nephrol. 2009;24(10):1901–11.PubMedCrossRefGoogle Scholar
  40. 40.
    Bogdanović R. Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment. Acta Paediatr. 2009;98(12):1882–9.PubMedCrossRefGoogle Scholar
  41. 41.
    Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T, Jahnukainen T, Rajantie J, Ormälä T, Nuutinen M. Clinical course of extrarenal symptoms in Henoch–Schonlein purpura: a 6-month prospective study. Arch Dis Child. 2010;95(11):871–6.PubMedCrossRefGoogle Scholar
  42. 42.
    Jauhola O, Ronkainen J, Autio-Harmainen H, Koskimies O, Ala-Houhala M, Arikoski P, et al. Cyclosporine A vs. methylprednisolone for Henoch–Schönlein nephritis: a randomized trial. Pediatr Nephrol. 2011;26(12):2159–66.PubMedCrossRefGoogle Scholar
  43. 43.
    Roccatello D, Ferro M, Coppo R, Giraudo G, Quattrocchio G, Piccoli G. Report on intensive treatment of extracapillary glomerulonephritis with focus on crescentic IgA nephropathy. Nephrol Dial Transplant. 1995;10(11):2054–9.PubMedGoogle Scholar
  44. 44.
    Shenoy M, Ognjanovic MV, Coulthard MG. Treating severe Henoch–Schönlein and IgA nephritis with plasmapheresis alone. Pediatr Nephrol. 2007;22(8):1167–71.PubMedCrossRefGoogle Scholar
  45. 45.
    Gianviti A, Trompeter RS, Barratt TM, Lythgoe MF, Dillon MJ. Retrospective study of plasma exchange in patients with idiopathic rapidly progressive glomerulonephritis and vasculitis. Arch Dis Child. 1996;75(3):186–90.PubMedPubMedCentralCrossRefGoogle Scholar
  46. 46.
    Han F, Chen LL, Ren PP, Le JY, Choong PJ, Wang HJ, et al. Mycophenolate mofetil plus prednisone for inducing remission of Henoch–Schönlein purpura nephritis: a retrospective study. J Zhejiang Univ Sci B. 2015;16(9):772–9.PubMedPubMedCentralCrossRefGoogle Scholar
  47. 47.
    Quartuccio L, Zuliani F, Corazza L, Scaini P, Zani R, Lenzi M, et al. Retreatment regimen of rituximab monotherapy given at the relapse of severe HCV-related cryoglobulinemic vasculitis: long-term follow up data of a randomized controlled multicentre study. J Autoimmun. 2015;63:88–93.PubMedCrossRefGoogle Scholar
  48. 48.
    Ferri C, Cacoub P, Mazzaro C, Roccatello D, Scaini P, Sebastiani M, et al. Treatment with rituximab in patients with mixed cryoglobulinemia syndrome: results of multicenter cohort study and review of the literature. Autoimmun Rev. 2011;11(1):48–55.PubMedCrossRefGoogle Scholar
  49. 49.
    Yang YH, Chuang YH, Wang LC, Huang HY, Gershwin ME, Chiang BL. The immunobiology of Henoch–Schönlein purpura. Autoimmun Rev. 2008;7(3):179–84.PubMedCrossRefGoogle Scholar
  50. 50.
    Wierciński R, Zoch-Zwierz W, Wasilewska A, Tomaszewska B, Winiecka W, Stasiak-Barmuta A, et al. Lymphocyte subpopulations of peripheral blood in children with Schönlein–Henoch purpura and IgA nephropathy. Pol Merkur Lekarski. 2001;10(58):244–6.PubMedGoogle Scholar
  51. 51.
    Sun XC, Chen MY, Cheng AS, Xu DL. Immunologic changes in children with Henoch–Schonlein purpura in the acute stage. Chin Med J (Engl). 1989;102(7):533–6.Google Scholar
  52. 52.
    Bellan M, Pirisi M, Sainaghi PP. Long-term remission of corticosteroid- and cyclophosphamide-resistant Henoch-Schönlein purpura with rituximab. Scand J Rheumatol. 2015;27:1–2.Google Scholar
  53. 53.
    Ishiguro H, Hashimoto T, Akata M, Suzuki S, Azushima K, Kobayashi Y, et al. Rituximab treatment for adult purpura nephritis with nephrotic syndrome. Intern Med. 2013;52(10):1079–83.PubMedCrossRefGoogle Scholar
  54. 54.
    Pindi Sala T, Michot JM, Snanoudj R, Dollat M, Estève E, Marie B, et al. Successful outcome of a corticodependent Henoch–Schönlein purpura adult with rituximab. Case Rep Med. 2014;2014:619218.PubMedPubMedCentralGoogle Scholar
  55. 55.
    Pillebout E, Rocha F, Fardet L, Rybojad M, Verine J, Glotz D. Successful outcome using rituximab as the only immunomodulation in Henoch–Schonlein purpura: case report. Nephrol Dial Transplant. 2011;26(6):2044–6.PubMedCrossRefGoogle Scholar
  56. 56.
    Donnithorne KJ, Atkinson TP, Hinze CH, Nogueira JB, Saeed SA, Askenazi DJ, et al. Rituximab therapy for severe refractory chronic Henoch–Schönlein purpura. J Pediatr. 2009;155(1):136–9.PubMedCrossRefGoogle Scholar
  57. 57.
    Hinze CH, Colbert RA. B-cell depletion in Wegener’s granulomatosis. Clin Rev Allergy Immunol. 2008;34(3):372–9.PubMedCrossRefGoogle Scholar
  58. 58.
    Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363(3):211–20.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Roberta Fenoglio
    • 1
  • Carla Naretto
    • 1
  • Bruno Basolo
    • 1
  • Giacomo Quattrocchio
    • 1
  • Michela Ferro
    • 1
  • Paola Mesiano
    • 1
  • Giulietta Beltrame
    • 1
  • Dario Roccatello
    • 1
  1. 1.Nephrology and Dialysis Unit, Center of Research of Immunopathology and Rare Diseases, Department of Rare, Immunologic, Hematologic and Immunohematologic DiseasesGiovanni Bosco Hospital and University of TurinTurinItaly

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