期刊论文详细信息
BMC Nephrology
Unexpected recovery from longterm renal failure in severe diffuse proliferative lupus nephritis
Katalin Dittrich1  Jörg Dötsch2  Kerstin Amann3  Katja Sauerstein4  Kerstin Benz4  Sophia Ross4 
[1] Hospital for Children and Adolescents, University of Leipzig, Leipzig, Germany;Department of Pediatrics, University of Köln, Köln, Germany;Department of Nephropathology, University of Erlangen-Nürnberg, Erlangen-Nürnberg, Germany;Department of Pediatric Nephrology, University of Erlangen-Nürnberg, Loschgestr. 15, Erlangen, D-91054, Germany
关键词: Remission;    Mycophenolate mofetil;    Immunosuppressive treatment;    Acute renal failure;    Proliferative lupus nephritis;   
Others  :  1083132
DOI  :  10.1186/1471-2369-13-81
 received in 2012-01-23, accepted in 2012-07-28,  发布年份 2012
PDF
【 摘 要 】

Background

Severe renal manifestation of systemic lupus erythematosus (SLE) is not uncommon and is associated with an indeterminate prognosis. Complete remission can be obtained, however, at least in the young when chronic lesions are absent and adequate anti-inflammatory therapy is immediately initiated.

Case presentation

We report the unusual case of a 12-year-old girl who presented with severe oliguric renal failure, macrohematuria and skin rash. Renal biopsy revealed the diagnosis of severe diffuse proliferative glomerulonephritis (GN) with cellular crescents in 15 out of 18 glomeruli and full-house pattern in immunofluorescence indicating lupus nephritis IVB according to WHO, IV-G(A) according to ISN/RPS classification. The serological parameters confirmed the diagnosis of SLE and the patient was immediately treated with methylprednisolone, cyclophosphamide and immunoadsorption. Initially, despite rapid amelioration of her general condition, no substantial improvement of renal function could be achieved and the patient needed hemodialysis treatment for 12 weeks. Unexpectedly, in the further follow-up at first diuresis increased and thereafter also creatinine levels substantially declined so that hemodialysis could be discontinued. Today, 6 years after the initial presentation, the patient has normal renal function and a SLEDAI score of 0 under a continuous immunosuppressive therapy with Mycophenolate mofetil (MMF) and low dose steroid.

Conclusion

Despite the severity of the initial renal injury and the unfavourable renal prognosis the kidney apparently has a tremendous capacity to recover in young patients when the damage is acute and adequate anti-inflammatory therapy is initiated without delay.

【 授权许可】

   
2012 Ross et al.; licensee BioMed Central Ltd.

【 预 览 】
附件列表
Files Size Format View
20150101010120948.pdf 1341KB PDF download
Figure 3. 17KB Image download
Figure 2. 75KB Image download
Figure 1. 418KB Image download
【 图 表 】

Figure 1.

Figure 2.

Figure 3.

【 参考文献 】
  • [1]Suría S, Checa MD: Mycophenolate mofetil in the treatment of lupus nephritis, in patients with failure, intolerance or relapses after treatment with steroids and cyclophosphamide. Nefrologia 2007, 27:459-65.
  • [2]Nezhad ST, Sepaskhah R: Correlation of clinical and pathological findings in patients with lupus nephritis: a five-year experience in iran. Saudi J Kidney Dis Transpl 2008, 19:32-40.
  • [3]Houssiau FA, Vasconcelos C, D’Cruz D, Sebastiani GD, Garrido Ed Ede R, Danieli MG, Abramovicz D, Blockmans D, Mathieu A, Direskeneli H, Galeazzi M, Gül A, Levy Y, Petera P, Popovic R, Petrovic R, Sinico RA, Cattaneo R, Font J, Depresseux G, Cosyns JP, Cervera R: Immunosuppressive therapy in lupus nephritis: The Euro-Lupus Nephritis Trial, a randomized trial of low-dose versus high-dose intravenous cyclophosphamide. Arthritis Rheum 2002, 46:2121-31.
  • [4]Hauer HA, Bajema IM, Van Houwelingen HC, Ferrario F, Noël LH, Waldherr R, Jayne DR, Rasmussen N, Bruijn JA, Hagen EC, European Vasculitis Study Group (EUVAS), European Vasculitis Study Group (EUVAS): European Vasculitis Study Group (EUVAS). Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int 2002, 62:1732-1742.
  • [5]Austin HA, Boumpas DT, Vaughan EM, Balow JE: High-risk features of lupus nephritis: importance of race and clinical and histological factors in 166 patients. Nephrol Dial Transplant 1995, 10:1620-8.
  • [6]Walsh M, Jayne D: Rituximab in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis and systemic lupus erythematodes: past, present and future. Kidney Int 2007, 72:676-682.
  • [7]Chan TM, Tse KC, Tang CS, Mok MY, Li FK, Hong Kong Nephrology Study Group, Hong Kong Nephrology Study Group: Long-term study of mycophenolate mofetil as continuous induction and maintenance treatment for diffuse proliferative lupus nephritis. J Am Soc Nephrol 2005, 16:1076-84.
  • [8]Stummvoll GH, Aringer M, Smolen JS, Schmaldienst S, Jiménez-Boj E, Hörl WH, Graninger WB, Derfler K: IgG immunoadsorption reduces systemic lupus erythematodes activity and proteinuria: a long term observational study. Ann Rheum Dis 2005, 64:1015-21.
  • [9]Gourley MF, Austin HA, Scott D, Yarboro CH, Vaughan EM, Muir J, Boumpas DT, Klippel JH, Balow JE, Steinberg AD: Methylprednisolone and cyclophosphamide, alone or in combination, in patients with lupus nephritis: a randomized, controlled trial. Ann Intern Med 1996, 125:549-557.
  • [10]Benz K, Amann K, Dittrich K, Hugo C, Schnur K, Dötsch J: Patient with antibody-negative relapse of Goodpasture syndrome. Clin Nephrol 2007, 67:240-4.
  • [11]Benseler SM, Bargman JM, Feldman BM, Tyrrell PN, Harvey E, Hebert D, Silverman ED: Acute renal failure in paediatric systemic lupus erythematodes: treatment and outcome. Rheumatology 2009, 48:176-82.
  • [12]Chu JK, Folkert VW: Renal function recovery in chronic dialysis patients. Semin Dial 2010, 23:606-13.
  • [13]Coplon NS, Diskin CJD, Petersen J, Swenson RS: The long-term clinical course of systemic lupus erythematosus in end-stage renal disease. N Engl J Med 1983, 308:186-190.
  • [14]Kimberly RP, Lockshin MD, Sherman RL, Beary JF, Mouradian J, Cheigh JS: “End-stage” lupus nephritis: clinical course to and outcome on dialysis. Experience with 39 patients. Medicine (Baltimore) 1981, 60:277-287.
  • [15]Kimberly RP, Lockshin MD, Sherman RL, Mouradian J, Saal S: Reversible “end-stage” lupus nephritis. Analysis of patients able to discontinue dialysis. Am J Med 1983, 74:361-368.
  • [16]Ziff M, Helderman JH: Dialysis and transplantation in end-stage lupus nephritis. N Engl J Med 1983, 308:218-219.
  • [17]Correia P, Cameron JS, Ogg CS, Williams DG, Bewick M, Hicks JA: End-stage renal failure in systemic lupus erythematosus with nephritis. Clin Nephrol 1984, 22:293-302.
  文献评价指标  
  下载次数:36次 浏览次数:6次