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Low-dose corticosteroid with mizoribine might be an effective therapy for elderly-onset ISKDC grade VI IgA vasculitis

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Abstract

Both the diagnosis of elderly-onset IgA vasculitis (IgAV) and its prognosis can be difficult because of its rarity and the likely presence of comorbidities. Furthermore, the treatment of elderly-onset IgAV remains controversial: the ideal dosages of corticosteroid and/or immunosuppressants have not been determined. In the elderly, corticosteroid adverse effects can lead to severe outcomes, and a consensus regarding its benefit and risk balance has not been reached. We report a case of IgAV in an 89-year-old patient who was admitted to our hospital to investigate a 30-day history of palpable purpura and pitting edema on her leg. A renal biopsy showed membranoproliferative glomerulonephritis with IgA deposits (The International Study of Kidney Disease in Children (ISKDC) grade VI), which is a predictor of a poor prognosis; these findings led to early intervention with low-dose corticosteroid (15 mg/day) and mizoribine. As a result, a complete remission without obvious adverse effects was obtained. Early intervention with low-dose corticosteroid and mizoribine based on renal histopathology results might be an effective treatment for elderly-onset ISKDC grade VI IgAV.

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References

  1. Komatsu H, Fujimoto S, Maruyama S, et al. Distinct characteristics and outcomes in elderly-onset IgA vasculitis (Henoch-Schönlein Purpura) with nephritis: nationwide cohort study of data from the Japan Renal Biopsy Registry (J-RBR). PLoS ONE. 2018;13:e0196955.

    PubMed  PubMed Central  Google Scholar 

  2. Ueda H, Miyazaki Y, Tsuboi N, et al. Clinical and pathological characteristics of elderly japanese patients with IgA vasculitis with nephritis: a case series. Intern Med. 2019;58:31–8.

    CAS  PubMed  Google Scholar 

  3. Hitomi K, Izaki S, Teraki Y, et al. Characterization of adult-type IgA vasculitis (Henoch-Schönlein Purpura): a retrospective study of 122 cases. Open Dermatol J. 2014;8:51–9.

    Google Scholar 

  4. Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D. Henoch-Schonlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002;13:1271–8.

    PubMed  Google Scholar 

  5. 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;14:579–85.

    CAS  PubMed  Google Scholar 

  6. Davin JC, Coppo R. Henoch-Schönlein Purpura nephritis in children. Nat Rev Nephrol. 2014;10:563–73.

    CAS  PubMed  Google Scholar 

  7. Roman C, Dima B, Muyshont L, Schurmans T, Gilliaux O. Indications and efficiency of Dapsone in IgA vasculitis (Henoch-Schonlein Purpura): case series and a review of the literature. Eur J Pediatr. 2019;178:1275–81.

    CAS  PubMed  Google Scholar 

  8. Sasaki E, Shibata M, Kato A, et al. An adult case of severe steroid-resistant Henoch-Schönlein Purpura nephritis treated with intravenous cyclophosphamide and tonsillectomy. CEN Case Rep. 2016;5:212–8.

    PubMed  PubMed Central  Google Scholar 

  9. Maritati F, Fenoglio R, Pillebout E, et al. Brief report: Rituximab for the treatment of adult-Onset IgA vasculitis (Henoch-Schönlein). Arthritis Rheumatol. 2018;70:109–14.

    CAS  PubMed  Google Scholar 

  10. Fenoglio R, Naretto C, Basolo B, et al. Rituximab therapy for IgA-vasculitis With nephritis: a case series and review of the literature. Immunol Res. 2017;65:186–92.

    CAS  PubMed  Google Scholar 

  11. Audemard-Verger A, Terrier B, Dechartres A, et al. Characteristics and management of IgA vasculitis (Henoch-Schönlein) in adults: data from 260 patients included in a french multicenter retrospective survey. Arthritis Rheumatol. 2017;69:1862–70.

    CAS  PubMed  Google Scholar 

  12. Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-Schönlein Purpura: a literature review. Acta Derm Venereol. 2017;97:1160–6.

    CAS  PubMed  Google Scholar 

  13. Hočevar A, Rotar Z, Jurčić V, et al. IgA vasculitis in adults: the performance of the EULAR/PRINTO/PRES classification criteria in adults. Arthritis Res Ther. 2016;18:58.

    PubMed  PubMed Central  Google Scholar 

  14. Yokoyama H, Sugiyama H, Sato H, et al. Renal disease in the elderly and the very elderly Japanese: analysis of the Japan Renal Biopsy Registry (J-RBR). Clin Exp Nephrol. 2012;16:903–20.

    PubMed  Google Scholar 

  15. Schinzel V, Fernandez JD, Clemente G, et al. The profile and clinical outcomes of patients with renal involvement due to IgA vasculitis: is azathioprine a good option for treatment? Adv Rheumatol. 2019;59:21.

    PubMed  Google Scholar 

  16. Nagasaka T, Miyamoto J, Ishibashi M, Chen KR. MPO-ANCA- and IgA-positive systemic vasculitis: a possibly overlapping syndrome of microscopic polyangiitis and Henoch-Schoenlein Purpura. J Cutan Pathol. 2009;36:871–7.

    PubMed  Google Scholar 

  17. Bernardino V, Mendes-Bastos P, Rodrigues A, Riso N. IgA vasculitis (formerly Henoch-Schönlein Purpura) in an adult with systemic lupus erythematosus. BMJ Case Rep. 2015. https://doi.org/10.1136/bcr-2015-210121.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Berquist JB, Bartels CM. Rare association of Henoch-Schönlein Purpura with recurrent endocarditis. WMJ. 2011;110:38–40.

    PubMed  PubMed Central  Google Scholar 

  19. Counahan R, Winterborn MH, White RH, et al. Prognosis of Henoch-Schonlein nephritis in children. Br Med J. 1977;2:11–4.

    CAS  PubMed  PubMed Central  Google Scholar 

  20. Rai A, Nast C, Adler S. Henoch-Schönlein Purpura nephritis. J Am Soc Nephrol. 1999;10:2637–44.

    CAS  PubMed  Google Scholar 

  21. Soylemezoglu O, Ozkaya O, Ozen S, et al. Henoch-Schönlein Nephritis: a nationwide study. Nephron Clin Pract. 2009;112:c199–204.

    CAS  PubMed  Google Scholar 

  22. Huang YJ, Yang XQ, Zhai WS, et al. Clinicopathological features and prognosis of membranoproliferative-like Henoch-Schönlein Purpura nephritis in children. World J Pediatr. 2015;11:338–45.

    PubMed  Google Scholar 

  23. Koskela M, Jahnukainen T, Endén K, et al. Methylprednisolone or cyclosporine a in the treatment of Henoch-Schönlein nephritis: a nationwide study. Pediatr Nephrol. 2019;34:1447–566.

    PubMed  Google Scholar 

  24. Kawasaki Y, Suyama K, Yugeta E, et al. The incidence and severity of Henoch-Schönlein Purpura nephritis over a 22-year period in Fukushima Prefecture. Japan Int Urol Nephrol. 2010;42:1023–9.

    PubMed  Google Scholar 

  25. Komatsu H, Fujimoto S, Yoshikawa N, Kitamura H, Sugiyama H, Yokoyama H. Clinical manifestations of Henoch-Schönlein Purpura nephritis and iga nephropathy: comparative analysis of data from the Japan Renal Biopsy Registry (J-RBR). Clin Exp Nephrol. 2016;20:552–60.

    PubMed  Google Scholar 

  26. Kaneko M, Ikezumi Y, Yamada T, Hasegawa H, Kaneko U, Saitoh A. Local leukocyte proliferation as a target for cyclophosphamide in the treatment of Henoch-Schönlein Purpura nephritis grade VI. Nephrology (Carlton). 2015;21:68–71.

    Google Scholar 

  27. Davin JC, Weening JJ. Diagnosis of Henoch-Schönlein Purpura: renal or skin biopsy? Pediatr Nephrol. 2003;18:1201–3.

    PubMed  Google Scholar 

  28. Navaratnarajah A, Sambasivan K, Cook TH, Pusey C, Roufosse C, Willicombe M. Predicting long-term renal and patient survival by clinicopathological features in elderly patients undergoing a renal biopsy in a UK cohort. Clin Kidney. 2019;J12:512–20.

    Google Scholar 

  29. Verde E, Quiroga B, Rivera F, López-Gómez JM. Renal biopsy in very elderly patients: data from the Spanish registry of glomerulonephritis. Am J Nephrol. 2012;35:230–7.

    PubMed  Google Scholar 

  30. Jefferson JA, Alpers CE. Diagnosis: should renal biopsies be performed in the very elderly? Nat Rev Nephrol. 2009;5:561–2.

    PubMed  Google Scholar 

  31. Moutzouris DA, Herlitz L, Appel GB, et al. Renal biopsy in the very elderly. Clin J Am Soc Nephrol. 2009;4:1073–82.

    CAS  PubMed  PubMed Central  Google Scholar 

  32. Koike M, Takei T, Uchida K, et al. Clinical assessment of low-dose steroid therapy for patients with IgA nephropathy: a prospective study in a single center. Clin Exp Nephrol. 2008;12:250–5.

    CAS  PubMed  Google Scholar 

  33. Stuck AE, Minder CE, Frey FJ. Risk of infectious complications in patients taking glucocorticosteroids. Rev Infect Dis. 1989;11:954–63.

    CAS  PubMed  Google Scholar 

  34. Van Staa TP, Leufkens HGM, Cooper C. The epidemiology of corticosteroid-induced osteoporosis: a meta-analysis. Osteoporos Int. 2002;13:777–87.

    PubMed  Google Scholar 

  35. Van Staa TP, Leufkens HG, Abenhaim L, Zhang B, Cooper C. Oral corticosteroids and fracture risk: relationship to daily and cumulative doses. Rheumatology (Oxford). 2000;39:1383–9.

    Google Scholar 

  36. Bowyer SL, LaMothe MP, Holiiiter JR. Steroid myopathy: incidence and detection in a population with asthma. J Allergy Clin Immunol. 1985;76:234–42.

    CAS  PubMed  Google Scholar 

  37. Mima A. Efficacy of mizoribine and prednisolone combination therapy in adult patients with IgA vasculitis. Rheumatol Int. 2017;37:1387–93.

    CAS  PubMed  Google Scholar 

  38. Kawasaki Y, Suyama K, Matsumoto A, et al. Efficacy of tonsillectomy plus methylprednisolone pulse therapy for a child with Henoch-Schoenlein Purpura nephritis. Tohoku J Exp Med. 2007;211:291–5.

    PubMed  Google Scholar 

  39. Kawakami T, Shirai S, Kimura K, Soma Y. Successful use of mizoribine to treat recurrent corticosteroid-resistant palpable purpura in a patient with Henoch Schonlein Purpura nephritis. Arch Dermatol. 2010;146:212–3.

    PubMed  Google Scholar 

  40. Chishiki M, Kawasaki Y, Kaneko M, et al. A 10-year-old girl with IgA nephropathy who 5 years later developed the characteristic features of Henoch-Schönlein Purpura nephritis. Fukushima J Med Sci. 2010;56:157–61.

    PubMed  Google Scholar 

  41. Ichiyama S, Matayoshi T, Kaneko T. Successful multitarget therapy using prednisolone, mizoribine and tacrolimus for Henoch-Schönlein Purpura nephritis in children. J Dermatol. 2017;44:e56–57.

    PubMed  Google Scholar 

  42. Itoh H, Komatsuda A, Wakui H, Miura AB, Tashima Y. Mammalian HSP60 is a major target for an immunosuppressant mizoribine. J Biol Chem. 1999;274:35147–51.

    CAS  PubMed  Google Scholar 

  43. Liu L, Ren B, Zhang H, et al. Population pharmacokinetic analysis of mizoribine in chinese renal transplant recipients. Transpl Proc. 2018;50:2392–7.

    CAS  Google Scholar 

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Correspondence to Hikaru Sugimoto.

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Sugimoto, H., Matsuno, S., Yamanaka, N. et al. Low-dose corticosteroid with mizoribine might be an effective therapy for elderly-onset ISKDC grade VI IgA vasculitis. CEN Case Rep 10, 46–52 (2021). https://doi.org/10.1007/s13730-020-00513-6

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