Immunosuppressive Treatment in C3 Glomerulopathy: Is it Really Effective?

被引:37
作者
Caliskan, Yasar [1 ]
Torun, Ege Sinan [1 ]
Tiryaki, Tarik Onur [1 ]
Oruc, Aysegul [4 ]
Ozluk, Yasemin [2 ]
Akgul, Sebahat Usta [3 ]
Temurhan, Sonay [3 ]
Oztop, Nida [1 ]
Kilicaslan, Isin [2 ]
Sever, Mehmet Sukru [1 ]
机构
[1] Istanbul Univ, Istanbul Fac Med, Dept Internal Med, Div Nephrol, Istanbul, Turkey
[2] Istanbul Univ, Istanbul Fac Med, Dept Pathol, Istanbul, Turkey
[3] Istanbul Univ, Istanbul Fac Med, Dept Med Biol, Istanbul, Turkey
[4] Uludag Univ, Fac Med, Dept Internal Med, Div Nephrol, Bursa, Turkey
关键词
Complement system; C3; glomerulopathy; End-stage renal disease; Proteinuria; DENSE DEPOSIT DISEASE; HEMOLYTIC-UREMIC SYNDROME; COMPLEMENT ABNORMALITIES; ECULIZUMAB; GLOMERULONEPHRITIS; ADULT;
D O I
10.1159/000479012
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
Background: C3 glomerulopathy (C3GP) is a recently identified and described disease that has a high risk of progressing into end-stage renal disease. We aimed to evaluate the effects of various immunosuppressive regimens on C3GP progression because there are conflicting data on the treatment modalities. Methods: In this retrospective study of 66 patients with C3GP, 27 patients received mycophenolate mofetil (MMF)-based treatment, 23 received non-MMF-based treatment (prednisolone or cyclophosphamide), and 16 received conservative care. The study groups were compared with each other with specific focus on primary outcomes defined as (1) kidney failure and (2) estimated glomerular filtration rate (eGFR) decline >= 50% from the baseline value. Results: Overall, 17 (25.8%) patients reached the primary outcome after a median period of 28 months. The number of patients who reached the primary outcome were similar among the study groups (MMF-based: 8/27 [29.6%], non-MMF-based: 4/23 [17.4%], and conservative care: 5/16 [31.3%], p = 0.520). In the Cox regression analysis, age (HR 0.912, p = 0.006), eGFR (HR 0.945, p = 0.001), and proteinuria levels (HR 1.418, p = 0.015) at the time of biopsy, percentage of crescentic (HR 1.035, p = 0.001) and sclerotic glomeruli (HR 1.041, p = 0.006), severity of interstitial fibrosis (HR 1.981, p = 0.048), as well as no remission of proteinuria (HR 2.418, p = 0.002) predicted the primary outcome. Conclusion: Although patients receiving immunosuppressive treatments had higher proteinuria and lower serum albumin at baseline, there were no differences between these patients and those receiving conservative care alone in proteinuria remission or in the decline of renal function. Younger age, higher proteinuria, lower eGFR, and the presence of crescentic and sclerotic glomeruli, severity of interstitial fibrosis, and no remission of proteinuria predicted the progression of kidney disease. (C) 2017 S. Karger AG, Basel
引用
收藏
页码:96 / 107
页数:12
相关论文
共 26 条
  • [1] [Anonymous], EMJ
  • [2] [Anonymous], KIDNEY INT S
  • [3] Advances in the Understanding of Complement Mediated Glomerular Disease: Implications for Recurrence in the Transplant Setting
    Barbour, S.
    Gill, J. S.
    [J]. AMERICAN JOURNAL OF TRANSPLANTATION, 2015, 15 (02) : 312 - 319
  • [4] Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis
    Bertsias, George K.
    Tektonidou, Maria
    Amoura, Zahir
    Aringer, Martin
    Bajema, Ingeborg
    Berden, Jo H. M.
    Boletis, John
    Cervera, Ricard
    Doerner, Thomas
    Doria, Andrea
    Ferrario, Franco
    Floege, Juergen
    Houssiau, Frederic A.
    Ioannidis, John P. A.
    Isenberg, David A.
    Kallenberg, Cees G. M.
    Lightstone, Liz
    Marks, Stephen D.
    Martini, Alberto
    Moroni, Gabriela
    Neumann, Irmgard
    Praga, Manuel
    Schneider, Matthias
    Starra, Argyre
    Tesar, Vladimir
    Vasconcelos, Carlos
    van Vollenhoven, Ronald F.
    Zakharova, Helena
    Haubitz, Marion
    Gordon, Caroline
    Jayne, David
    Boumpas, Dimitrios T.
    [J]. ANNALS OF THE RHEUMATIC DISEASES, 2012, 71 (11) : 1771 - 1782
  • [5] Pathogenesis of the C3 glomerulopathies and reclassification of MPGN
    Bomback, Andrew S.
    Appel, Gerald B.
    [J]. NATURE REVIEWS NEPHROLOGY, 2012, 8 (11) : 634 - 642
  • [6] Eculizumab for Dense Deposit Disease and C3 Glomerulonephritis
    Bomback, Andrew S.
    Smith, Richard J.
    Barile, Gaetano R.
    Zhang, Yuzhou
    Heher, Eliot C.
    Herlitz, Leal
    Stokes, M. Barry
    Markowitz, Glen S.
    D'Agati, Vivette D.
    Canetta, Pietro A.
    Radhakrishnan, Jai
    Appel, Gerald B.
    [J]. CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2012, 7 (05): : 748 - 756
  • [7] IDIOPATHIC MESANGIOCAPILLARY GLOMERULONEPHRITIS - COMPARISON OF TYPE-I AND TYPE-II IN CHILDREN AND ADULTS AND LONG-TERM PROGNOSIS
    CAMERON, JS
    TURNER, DR
    HEATON, J
    WILLIAMS, DG
    OGG, CS
    CHANTLER, C
    HAYCOCK, GB
    HICKS, J
    [J]. AMERICAN JOURNAL OF MEDICINE, 1983, 74 (02) : 175 - 192
  • [8] Combined C3b and Factor B Autoantibodies and MPGN Type II
    Chen, Qian
    Mueller, Dominik
    Rudolph, Birgit
    Hartmann, Andrea
    Kuwertz-Broeking, Eberhard
    Wu, Kaiyin
    Kirschfink, Michael
    Skerka, Christine
    Zipfel, Peter F.
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 2011, 365 (24) : 2340 - 2342
  • [9] C3 glomerulopathy: what's in a name?
    D'Agati, Vivette D.
    Bomback, Andrew S.
    [J]. KIDNEY INTERNATIONAL, 2012, 82 (04) : 379 - 381
  • [10] Heterozygous and homozygous factor H deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis:: Report and genetic analysis of 16 cases
    Dragon-Durey, MA
    Frémeaux-Bacchi, V
    Loirat, C
    Blouin, J
    Niaudet, P
    Deschenes, G
    Coppo, P
    Fridman, WH
    Weiss, L
    [J]. JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2004, 15 (03): : 787 - 795