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.
Similar content being viewed by others
References
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.
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.
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.
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.
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.
Davin JC, Coppo R. Henoch-Schönlein Purpura nephritis in children. Nat Rev Nephrol. 2014;10:563–73.
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.
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.
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.
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.
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.
Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-Schönlein Purpura: a literature review. Acta Derm Venereol. 2017;97:1160–6.
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.
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.
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.
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.
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.
Berquist JB, Bartels CM. Rare association of Henoch-Schönlein Purpura with recurrent endocarditis. WMJ. 2011;110:38–40.
Counahan R, Winterborn MH, White RH, et al. Prognosis of Henoch-Schonlein nephritis in children. Br Med J. 1977;2:11–4.
Rai A, Nast C, Adler S. Henoch-Schönlein Purpura nephritis. J Am Soc Nephrol. 1999;10:2637–44.
Soylemezoglu O, Ozkaya O, Ozen S, et al. Henoch-Schönlein Nephritis: a nationwide study. Nephron Clin Pract. 2009;112:c199–204.
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.
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.
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.
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.
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.
Davin JC, Weening JJ. Diagnosis of Henoch-Schönlein Purpura: renal or skin biopsy? Pediatr Nephrol. 2003;18:1201–3.
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.
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.
Jefferson JA, Alpers CE. Diagnosis: should renal biopsies be performed in the very elderly? Nat Rev Nephrol. 2009;5:561–2.
Moutzouris DA, Herlitz L, Appel GB, et al. Renal biopsy in the very elderly. Clin J Am Soc Nephrol. 2009;4:1073–82.
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.
Stuck AE, Minder CE, Frey FJ. Risk of infectious complications in patients taking glucocorticosteroids. Rev Infect Dis. 1989;11:954–63.
Van Staa TP, Leufkens HGM, Cooper C. The epidemiology of corticosteroid-induced osteoporosis: a meta-analysis. Osteoporos Int. 2002;13:777–87.
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.
Bowyer SL, LaMothe MP, Holiiiter JR. Steroid myopathy: incidence and detection in a population with asthma. J Allergy Clin Immunol. 1985;76:234–42.
Mima A. Efficacy of mizoribine and prednisolone combination therapy in adult patients with IgA vasculitis. Rheumatol Int. 2017;37:1387–93.
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.
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.
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.
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.
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.
Liu L, Ren B, Zhang H, et al. Population pharmacokinetic analysis of mizoribine in chinese renal transplant recipients. Transpl Proc. 2018;50:2392–7.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors have declared that no conflicts of interest exists.
Informed consent
Informed consent was obtained from all individual participants included in the study.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
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
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13730-020-00513-6