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Treatment of minimal change disease in adults

INTRODUCTION

Glucocorticoid therapy is the treatment of choice in minimal change disease (MCD), leading to a complete remission of proteinuria in over 85 to 90 percent of cases. This approach is based upon the beneficial results reported in several large prospective randomized trials in children, extensive observational studies in children and adults, and a very limited number of small randomized controlled trials in adults that compared prednisone with no specific therapy or that compared various prednisone regimens [1-6]. Due to their small size, the results of these controlled trials are not definitive.

MCD accounts for 90 percent of cases of the idiopathic nephrotic syndrome in young children (under age six years) and 65 percent of cases in older children [7-9]. As a result, glucocorticoid therapy is usually begun empirically in children, and a kidney biopsy is performed only for glucocorticoid-resistant disease. (See "Etiology, clinical manifestations, and diagnosis of nephrotic syndrome in children" and "Treatment of idiopathic nephrotic syndrome in children".)

In comparison, MCD accounts for only 10 to 25 percent of cases of nephrotic syndrome in adults [10-12]. Thus, patients over age 16 years are generally treated after kidney biopsy that shows MCD. (See "Etiology, clinical features, and diagnosis of minimal change disease in adults".)

Children tend to remit rapidly, with 50 percent responding within two weeks and almost all within eight weeks [7,13]. Adults tend to respond more slowly, with more than 25 percent of responders taking three to four months or longer to undergo complete remission (figure 1) [13-17]. Therefore, the definition of steroid resistance is not the same for patients younger than about 16 years of age and for those over about 17 years of age.

The treatment of MCD in adults will be reviewed here. The treatment of other frequent causes of the nephrotic syndrome occurring in adults, such as focal segmental glomerulosclerosis (FSGS), membranous nephropathy, and primary amyloidosis, are discussed separately. (See "Treatment of primary focal segmental glomerulosclerosis" and "Treatment of idiopathic membranous nephropathy" and "Prognosis and treatment of immunoglobulin light chain (AL) amyloidosis and light and heavy chain deposition diseases".)

                            

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Literature review current through: Sep 2014. | This topic last updated: Jul 11, 2013.
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References
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  1. Hogan J, Radhakrishnan J. The treatment of minimal change disease in adults. J Am Soc Nephrol 2013; 24:702.
  2. Black DA, Rose G, Brewer DB. Controlled trial of prednisone in adult patients with the nephrotic syndrome. Br Med J 1970; 3:421.
  3. Coggins CH. Adult minimal change nephropathy: experience of the collaborative study of glomerular disease. Trans Am Clin Climatol Assoc 1986; 97:18.
  4. Imbasciati E, Gusmano R, Edefonti A, et al. Controlled trial of methylprednisolone pulses and low dose oral prednisone for the minimal change nephrotic syndrome. Br Med J (Clin Res Ed) 1985; 291:1305.
  5. Yeung CK, Wong KL, Ng WL. Intravenous methylprednisolone pulse therapy in minimal change nephrotic syndrome. Aust N Z J Med 1983; 13:349.
  6. KDIGO. KDIGO Clinical Practice Guideline for Glomerulonephritis. Kidney Int Suppl 2012; 2:209. http://www.kdigo.org/clinical_practice_guidelines/pdf/KDIGO-GN-Guideline.pdf (Accessed on December 23, 2013).
  7. The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children. J Pediatr 1981; 98:561.
  8. White RH, Glasgow EF, Mills RJ. Clinicopathological study of nephrotic syndrome in childhood. Lancet 1970; 1:1353.
  9. Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children. Kidney Int 1978; 13:159.
  10. Haas M, Meehan SM, Karrison TG, Spargo BH. Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976-1979 and 1995-1997. Am J Kidney Dis 1997; 30:621.
  11. Gesualdo L, Di Palma AM, Morrone LF, et al. The Italian experience of the national registry of renal biopsies. Kidney Int 2004; 66:890.
  12. Tune BM, Mendoza SA. Treatment of the idiopathic nephrotic syndrome: regimens and outcomes in children and adults. J Am Soc Nephrol 1997; 8:824.
  13. Nolasco F, Cameron JS, Heywood EF, et al. Adult-onset minimal change nephrotic syndrome: a long-term follow-up. Kidney Int 1986; 29:1215.
  14. Fujimoto S, Yamamoto Y, Hisanaga S, et al. Minimal change nephrotic syndrome in adults: response to corticosteroid therapy and frequency of relapse. Am J Kidney Dis 1991; 17:687.
  15. Nakayama M, Katafuchi R, Yanase T, et al. Steroid responsiveness and frequency of relapse in adult-onset minimal change nephrotic syndrome. Am J Kidney Dis 2002; 39:503.
  16. Korbet SM, Schwartz MM, Lewis EJ. Minimal-change glomerulopathy of adulthood. Am J Nephrol 1988; 8:291.
  17. Mak SK, Short CD, Mallick NP. Long-term outcome of adult-onset minimal-change nephropathy. Nephrol Dial Transplant 1996; 11:2192.
  18. Arneil GC, Lam CN. Long-term assessment of steroid therapy in childhood nephrosis. Lancet 1966; 2:819.
  19. Tse KC, Lam MF, Yip PS, et al. Idiopathic minimal change nephrotic syndrome in older adults: steroid responsiveness and pattern of relapses. Nephrol Dial Transplant 2003; 18:1316.
  20. Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr. Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. J Am Soc Nephrol 1997; 8:769.
  21. Meyrier, A. Management of idiopathic nephrotic syndrome in adults: Minimal change disease and focal segmental glomerulosclerosis. In: Evidence-Based Nephrology, Molony, DA, Craig, JC (Eds), Wiley-Blackwell, Oxford 2009, p.149.
  22. Waldman M, Crew RJ, Valeri A, et al. Adult minimal-change disease: clinical characteristics, treatment, and outcomes. Clin J Am Soc Nephrol 2007; 2:445.
  23. Ahmad H, Tejani A. Predictive value of repeat renal biopsies in children with nephrotic syndrome. Nephron 2000; 84:342.
  24. Pru C, Kjellstrand CM, Cohn RA, Vernier RL. Late recurrence of minimal lesion nephrotic syndrome. Ann Intern Med 1984; 100:69.
  25. Galle, J. Reduction of proteinuria with angiotensin receptor blockers. Nature Clin Pract Cardiovascular Medicine 2008; 5: (Suppl. 1) S36.
  26. Mathieson PW. Proteinuria and immunity--an overstated relationship? N Engl J Med 2008; 359:2492.
  27. Meyrier AY. Treatment of focal segmental glomerulosclerosis with immunophilin modulation: when did we stop thinking about pathogenesis? Kidney Int 2009; 76:487.
  28. Huang JJ, Hsu SC, Chen FF, et al. Adult-onset minimal change disease among Taiwanese: clinical features, therapeutic response, and prognosis. Am J Nephrol 2001; 21:28.
  29. Nair RB, Date A, Kirubakaran MG, Shastry JC. Minimal-change nephrotic syndrome in adults treated with alternate-day steroids. Nephron 1987; 47:209.
  30. Wang F, Looi LM, Chua CT. Minimal change glomerular disease in Malaysian adults and use of alternate day steroid therapy. Q J Med 1982; 51:312.
  31. Vande Walle JG, Donckerwolcke RA, van Isselt JW, et al. Volume regulation in children with early relapse of minimal-change nephrosis with or without hypovolaemic symptoms. Lancet 1995; 346:148.
  32. Koomans HA, Boer WH, Dorhout Mees EJ. Renal function during recovery from minimal lesions nephrotic syndrome. Nephron 1987; 47:173.
  33. Salant, D, Dember, LM. Minimal change disease. In: Therapy in Nephrology and Hypertension, 2d ed, Brady, HR, Wilcox, CS (Eds), Saunders, Philadelphia, 2003.
  34. Krasner AS. Glucocorticoid-induced adrenal insufficiency. JAMA 1999; 282:671.
  35. Leisti S, Koskimies O. Risk of relapse in steroid-sensitive nephrotic syndrome: effect of stage of post-prednisone adrenocortical suppression. J Pediatr 1983; 103:553.
  36. Matsumoto H, Nakao T, Okada T, et al. Initial remission-inducing effect of very low-dose cyclosporin monotherapy for minimal-change nephrotic syndrome in Japanese adults. Clin Nephrol 2001; 55:143.
  37. Matsumoto H, Nakao T, Okada T, et al. Favorable outcome of low-dose cyclosporine after pulse methylprednisolone in Japanese adult minimal-change nephrotic syndrome. Intern Med 2004; 43:668.
  38. Meyrier A. Treatment of idiopathic nephrosis by immunophillin modulation. Nephrol Dial Transplant 2003; 18 Suppl 6:vi79.
  39. Ponticelli C, Edefonti A, Ghio L, et al. Cyclosporin versus cyclophosphamide for patients with steroid-dependent and frequently relapsing idiopathic nephrotic syndrome: a multicentre randomized controlled trial. Nephrol Dial Transplant 1993; 8:1326.
  40. Cyclophosphamide treatment of steroid dependent nephrotic syndrome: comparison of eight week with 12 week course. Report of Arbeitsgemeinschaft für Pädiatrische Nephrologie. Arch Dis Child 1987; 62:1102.
  41. Cattran DC, Alexopoulos E, Heering P, et al. Cyclosporin in idiopathic glomerular disease associated with the nephrotic syndrome : workshop recommendations. Kidney Int 2007; 72:1429.
  42. Westhoff TH, Schmidt S, Zidek W, et al. Tacrolimus in steroid-resistant and steroid-dependent nephrotic syndrome. Clin Nephrol 2006; 65:393.
  43. Sinha MD, MacLeod R, Rigby E, Clark AG. Treatment of severe steroid-dependent nephrotic syndrome (SDNS) in children with tacrolimus. Nephrol Dial Transplant 2006; 21:1848.
  44. Li X, Li H, Chen J, et al. Tacrolimus as a steroid-sparing agent for adults with steroid-dependent minimal change nephrotic syndrome. Nephrol Dial Transplant 2008; 23:1919.
  45. Li X, Li H, Ye H, et al. Tacrolimus therapy in adults with steroid- and cyclophosphamide-resistant nephrotic syndrome and normal or mildly reduced GFR. Am J Kidney Dis 2009; 54:51.
  46. Meyrier A, Noël LH, Auriche P, Callard P. Long-term renal tolerance of cyclosporin A treatment in adult idiopathic nephrotic syndrome. Collaborative Group of the Société de Néphrologie. Kidney Int 1994; 45:1446.
  47. Goumenos DS, Kalliakmani P, Tsakas S, et al. Cyclosporin-A in the treatment of nephrotic syndrome: the importance of monitoring C0 (trough) and C2 (two hours after its administration) blood levels. Med Chem 2006; 2:391.
  48. Koike M, Honda K, Tsukada M, et al. [Low-dose cyclosporin therapy combined with prednisolone for relapsing minimal change nephrotic syndrome in adults]. Nihon Jinzo Gakkai Shi 2002; 44:447.
  49. Meyrier A, Condamin MC, Broneer D. Treatment of adult idiopathic nephrotic syndrome with cyclosporin A: minimal-change disease and focal-segmental glomerulosclerosis. Collaborative Group of the French Society of Nephrology. Clin Nephrol 1991; 35 Suppl 1:S37.
  50. Lee HY, Kim HS, Kang CM, et al. The efficacy of cyclosporine A in adult nephrotic syndrome with minimal change disease and focal-segmental glomerulosclerosis: a multicenter study in Korea. Clin Nephrol 1995; 43:375.
  51. Melocoton TL, Kamil ES, Cohen AH, Fine RN. Long-term cyclosporine A treatment of steroid-resistant and steroid-dependent nephrotic syndrome. Am J Kidney Dis 1991; 18:583.
  52. Guigonis V, Dallocchio A, Baudouin V, et al. Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases. Pediatr Nephrol 2008; 23:1269.
  53. Ravani P, Magnasco A, Edefonti A, et al. Short-term effects of rituximab in children with steroid- and calcineurin-dependent nephrotic syndrome: a randomized controlled trial. Clin J Am Soc Nephrol 2011; 6:1308.
  54. Sinha A, Bagga A, Gulati A, Hari P. Short-term efficacy of rituximab versus tacrolimus in steroid-dependent nephrotic syndrome. Pediatr Nephrol 2012; 27:235.
  55. Prytuła A, Iijima K, Kamei K, et al. Rituximab in refractory nephrotic syndrome. Pediatr Nephrol 2010; 25:461.
  56. Takei T, Itabashi M, Moriyama T, et al. Effect of single-dose rituximab on steroid-dependent minimal-change nephrotic syndrome in adults. Nephrol Dial Transplant 2013; 28:1225.
  57. Ravani P, Ponticelli A, Siciliano C, et al. Rituximab is a safe and effective long-term treatment for children with steroid and calcineurin inhibitor-dependent idiopathic nephrotic syndrome. Kidney Int 2013; 84:1025.
  58. Munyentwali H, Bouachi K, Audard V, et al. Rituximab is an efficient and safe treatment in adults with steroid-dependent minimal change disease. Kidney Int 2013; 83:511.
  59. Hoxha E, Stahl RA, Harendza S. Rituximab in adult patients with immunosuppressive-dependent minimal change disease. Clin Nephrol 2011; 76:151.
  60. Sinha A, Bagga A. Rituximab therapy in nephrotic syndrome: implications for patients' management. Nat Rev Nephrol 2013; 9:154.
  61. Choi MJ, Eustace JA, Gimenez LF, et al. Mycophenolate mofetil treatment for primary glomerular diseases. Kidney Int 2002; 61:1098.
  62. Briggs WA, Choi MJ, Scheel PJ Jr. Successful mycophenolate mofetil treatment of glomerular disease. Am J Kidney Dis 1998; 31:213.
  63. Day CJ, Cockwell P, Lipkin GW, et al. Mycophenolate mofetil in the treatment of resistant idiopathic nephrotic syndrome. Nephrol Dial Transplant 2002; 17:2011.
  64. Cade R, Mars D, Privette M, et al. Effect of long-term azathioprine administration in adults with minimal-change glomerulonephritis and nephrotic syndrome resistant to corticosteroids. Arch Intern Med 1986; 146:737.
  65. Remuzzi G, Cravedi P, Costantini M, et al. Mycophenolate mofetil versus azathioprine for prevention of chronic allograft dysfunction in renal transplantation: the MYSS follow-up randomized, controlled clinical trial. J Am Soc Nephrol 2007; 18:1973.
  66. Niaudet P. Treatment of childhood steroid-resistant idiopathic nephrosis with a combination of cyclosporine and prednisone. French Society of Pediatric Nephrology. J Pediatr 1994; 125:981.
  67. Ponticelli C, Rizzoni G, Edefonti A, et al. A randomized trial of cyclosporine in steroid-resistant idiopathic nephrotic syndrome. Kidney Int 1993; 43:1377.
  68. Cattran DC, Appel GB, Hebert LA, et al. A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America Nephrotic Syndrome Study Group. Kidney Int 1999; 56:2220.
  69. Meyrier A. Treatment of idiopathic nephrotic syndrome with cyclosporine A. J Nephrol 1997; 10:14.