非典型抗肾小球基膜病
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摘要
抗肾小球基膜(GBM)病是一种较少见但严重的自身免疫性疾病。其特点为血液循环及肾小球基膜存在抗GBM抗体,多数患者有肾脏损害,伴(或)不伴肺累及。既往认为该病临床表现典型,进展迅速,预后极差。现随着诊断技术的提高,临床工作中发现部分患者临床表现或实验室检查结果不完全符合典型的抗GBM病,给临床诊断及治疗带来困难。本文就非典型抗GBM病的发病机制、临床表现及实验室检查等作一综述,旨在提高对非典型抗GBM病的进一步认识,减少误诊及漏诊。
Anti-glomerular basement membrane(GBM) disease is a rare but severe autoimmune disease.It is characterized by the presence of anti-GBM antibodies in circulatory and glomerular basement membrane,and most of the patients have renal involvement,with or without alveolar hemorrhage.It was considered that the clinical manifestations of this disease were typical,the progress was rapid and prognosis was extremely poor.Along with the continuous improvement of diagnostic techniques,it is found that some patients' clinical manifestation or laboratory assay results are not completely consistent with typical anti-GBM disease,which brings difficulties to clinical diagnosis and treatment.This paper has discussed about atypical anti-GBM disease in pathogenic mechanism,clinical manifestation,and treatments in order to improve the diagnosis and treatment of atypical anti-GBM disease.
Anti-glomerular basement membrane(GBM) disease is a rare but severe autoimmune disease.It is characterized by the presence of anti-GBM antibodies in circulatory and glomerular basement membrane,and most of the patients have renal involvement,with or without alveolar hemorrhage.It was considered that the clinical manifestations of this disease were typical,the progress was rapid and prognosis was extremely poor.Along with the continuous improvement of diagnostic techniques,it is found that some patients' clinical manifestation or laboratory assay results are not completely consistent with typical anti-GBM disease,which brings difficulties to clinical diagnosis and treatment.This paper has discussed about atypical anti-GBM disease in pathogenic mechanism,clinical manifestation,and treatments in order to improve the diagnosis and treatment of atypical anti-GBM disease.
引文
1 Tang Z,Wu Y,Wang Q,et al.Clinical spectrum of diffuse crescentic glomerulonephritis in Chinese patients.Chin Med J (Engl),2003,116(11):1737-1740.
2 Chen S,Tang Z,Xiang H,et al.Etiology and Outcome of Crescentic Glomerulonephritis From a Single Center in China:A 10-Year Review.Am J Kidney Dis,2016,67(3):376-383.
3 Salama AD,Dougan T,Levy JB,et al.Goodpasture′s disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard techniques.Am J Kidney Dis,2002,39(6):1162-1167.
4 Nasr SH,Collins AB,Alexander MP,et al.The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis.Kidney Int,2016,89(4):897-908.
5 Sinico RA,Radice A,Corace C,et al.Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome:a comparison of different assays.Nephrol Dial Transplant,2006,21(2):397-401.
6 Glassock RJ.Atypical anti-glomerular basement membrane disease:lessons learned.Clin Kidney J,2016,9(5):653-656.
7 Jia XY,Qu Z,Cui Z,et al.Circulating anti-glomerular basement membrane autoantibodies against α3(IV)NC1 undetectable by commercially available enzyme-linked immunosorbent assays.Nephrology (Carlton),2012,17(2):160-166.
8 Ohlsson S,Herlitz H,Lundberg S,et al.Circulating anti-glomerular basement membrane antibodies with predominance of subclass IgG4 and false-negative immunoassay test results in anti-glomerular basement membrane disease.Am J Kidney Dis,2014,63(2):289-293.
9 Hu SY,Gu QH,Wang J,et al.The pathogenicity of T cell epitopes on human Goodpasture antigen and its critical amino acid motif.J Cell Mol Med,2017,21(9):2117-2128.
10 Arends J,Wu J,Borillo J,et al.T cell epitope mimicry in antiglomerular basement membrane disease.J Immunol,2006,176(2):1252-1258.
11 陈骏良,崔昭,曲贞,等.肾组织中缺乏IgG线样沉积的抗肾小球基底膜病患者的临床病理特点.中华肾脏病杂志,2011,27(12):871-876.Jun-liang C,Zhao C,Zhen QU,et al.Clinical and pathological features of patients with anti-GBM disease lacking linear IgG deposition along GBM in kidney.Chinese Journal of Nephrology,2011,27(12):871-876.
12 Sheerin NS,Springall T,Abe K,et al.Protection and injury:the differing roles of complement in the development of glomerular injury.Eur J Immunol,2001,31(4):1255-1260.
13 Moulis G,Huart A,Guitard J,et al.IgA-mediated anti-glomerular basement membrane disease:an uncommon mechanism of Goodpasture′s syndrome.Clin Kidney J,2012,5(6):545-548.
14 Cui Z,Zhao MH,Singh AK,et al.Antiglomerular basement membrane disease with normal renal function.Kidney Int,2007,72(11):1403-1408.
15 牟利军,陈丽萌,左来孟,等.肾功能正常的抗肾小球基底膜病临床分析.中国医学科学院学报,2011,33(4):432-435.Li-jun M,Li-meng C,Lai-meng Z,et al.Clinical Analysis of Anti-glomerular Basement Membrane Disease with Normal Renal Function.Acta Academiae Medicinae Sinicae,2011,33(4):432-435.
16 Ang C,Savige J,Dawborn J,et al.Anti-glomerular basement membrane (GBM)-antibody-mediated disease with normal renal function.Nephrol Dial Transplant,1998,13(4):935-939.
17 Cui Z,Zhao MH,Wang SX,et al.Concurrent antiglomerular basement membrane disease and immune complex glomerulonephritis.Ren Fail,2006,28(1):7-14.
18 Patel D,Nivera N,Tunkel AR.Anti-glomerular basement membrane disease superimposed on membranous nephropathy:a case report and review of the literature.J Med Case Rep,2010,4:237.
19 Gao B,Li M,Xia W,et al.Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy:a case report.Clin Nephrol,2014,81(2):138-141.
20 Jia XY,Hu SY,Chen JL,et al.The clinical and immunological features of patients with combined anti-glomerular basement membrane disease and membranous nephropathy.Kidney Int,2014,85(4):945-952.
21 McAdoo SP,Tanna A,Hru?ková Z,et al.Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival,frequency of relapse,and outcomes compared to single-seropositive patients.Kidney Int,2017,92(3):693-702.
22 Zhao J,Yang R,Cui Z,et al.Characteristics and outcome of Chinese patients with both antineutrophil cytoplasmic antibody and antiglomerular basement membrane antibodies.Nephron Clin Pract,2007,107(2):c56-62.
23 Srivastava A,Rao GK,Segal PE,et al.Characteristics and outcome of crescentic glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Clin Rheumatol,2013,32(9):1317-1322.
24 Segelmark M,Hellmark T,Wieslander J.The prognostic significance in Goodpasture′s disease of specificity,titre and affinity of anti-glomerular-basement-membrane antibodies.Nephron Clin Pract,2003,94(3):c59-68.
25 Olson SW,Arbogast CB,Baker TP,et al.Asymptomatic autoantibodies associate with future anti-glomerular basement membrane disease.J Am Soc Nephrol,2011,22(10):1946-1952.
26 Jia XY,Yu JT,Hu SY,et al.Antibodies against linear epitopes on Goodpasture autoantigen in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.Clin Rheumatol,2017,36(9):2087-2094.
27 Li JN,Cui Z,Wang J,et al.Autoantibodies against Linear Epitopes of Myeloperoxidase in Anti-Glomerular Basement Membrane Disease.Clin J Am Soc Nephrol,2016,11(4):568-575.
28 Kumar SV,Kulkarni OP,Mulay SR,et al.Neutrophil Extracellular Trap-Related Extracellular Histones Cause Vascular Necrosis in Severe GN.J Am Soc Nephrol,2015,26(10):2399-2413.
29 Nakazawa D,Tomaru U,Suzuki A,et al.Abnormal conformation and impaired degradation of propylthiouracil-induced neutrophil extracellular traps:implications of disordered neutrophil extracellular traps in a rat model of myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis.Arthritis Rheum,2012,64(11):3779-3787.
2 Chen S,Tang Z,Xiang H,et al.Etiology and Outcome of Crescentic Glomerulonephritis From a Single Center in China:A 10-Year Review.Am J Kidney Dis,2016,67(3):376-383.
3 Salama AD,Dougan T,Levy JB,et al.Goodpasture′s disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard techniques.Am J Kidney Dis,2002,39(6):1162-1167.
4 Nasr SH,Collins AB,Alexander MP,et al.The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis.Kidney Int,2016,89(4):897-908.
5 Sinico RA,Radice A,Corace C,et al.Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome:a comparison of different assays.Nephrol Dial Transplant,2006,21(2):397-401.
6 Glassock RJ.Atypical anti-glomerular basement membrane disease:lessons learned.Clin Kidney J,2016,9(5):653-656.
7 Jia XY,Qu Z,Cui Z,et al.Circulating anti-glomerular basement membrane autoantibodies against α3(IV)NC1 undetectable by commercially available enzyme-linked immunosorbent assays.Nephrology (Carlton),2012,17(2):160-166.
8 Ohlsson S,Herlitz H,Lundberg S,et al.Circulating anti-glomerular basement membrane antibodies with predominance of subclass IgG4 and false-negative immunoassay test results in anti-glomerular basement membrane disease.Am J Kidney Dis,2014,63(2):289-293.
9 Hu SY,Gu QH,Wang J,et al.The pathogenicity of T cell epitopes on human Goodpasture antigen and its critical amino acid motif.J Cell Mol Med,2017,21(9):2117-2128.
10 Arends J,Wu J,Borillo J,et al.T cell epitope mimicry in antiglomerular basement membrane disease.J Immunol,2006,176(2):1252-1258.
11 陈骏良,崔昭,曲贞,等.肾组织中缺乏IgG线样沉积的抗肾小球基底膜病患者的临床病理特点.中华肾脏病杂志,2011,27(12):871-876.Jun-liang C,Zhao C,Zhen QU,et al.Clinical and pathological features of patients with anti-GBM disease lacking linear IgG deposition along GBM in kidney.Chinese Journal of Nephrology,2011,27(12):871-876.
12 Sheerin NS,Springall T,Abe K,et al.Protection and injury:the differing roles of complement in the development of glomerular injury.Eur J Immunol,2001,31(4):1255-1260.
13 Moulis G,Huart A,Guitard J,et al.IgA-mediated anti-glomerular basement membrane disease:an uncommon mechanism of Goodpasture′s syndrome.Clin Kidney J,2012,5(6):545-548.
14 Cui Z,Zhao MH,Singh AK,et al.Antiglomerular basement membrane disease with normal renal function.Kidney Int,2007,72(11):1403-1408.
15 牟利军,陈丽萌,左来孟,等.肾功能正常的抗肾小球基底膜病临床分析.中国医学科学院学报,2011,33(4):432-435.Li-jun M,Li-meng C,Lai-meng Z,et al.Clinical Analysis of Anti-glomerular Basement Membrane Disease with Normal Renal Function.Acta Academiae Medicinae Sinicae,2011,33(4):432-435.
16 Ang C,Savige J,Dawborn J,et al.Anti-glomerular basement membrane (GBM)-antibody-mediated disease with normal renal function.Nephrol Dial Transplant,1998,13(4):935-939.
17 Cui Z,Zhao MH,Wang SX,et al.Concurrent antiglomerular basement membrane disease and immune complex glomerulonephritis.Ren Fail,2006,28(1):7-14.
18 Patel D,Nivera N,Tunkel AR.Anti-glomerular basement membrane disease superimposed on membranous nephropathy:a case report and review of the literature.J Med Case Rep,2010,4:237.
19 Gao B,Li M,Xia W,et al.Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy:a case report.Clin Nephrol,2014,81(2):138-141.
20 Jia XY,Hu SY,Chen JL,et al.The clinical and immunological features of patients with combined anti-glomerular basement membrane disease and membranous nephropathy.Kidney Int,2014,85(4):945-952.
21 McAdoo SP,Tanna A,Hru?ková Z,et al.Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival,frequency of relapse,and outcomes compared to single-seropositive patients.Kidney Int,2017,92(3):693-702.
22 Zhao J,Yang R,Cui Z,et al.Characteristics and outcome of Chinese patients with both antineutrophil cytoplasmic antibody and antiglomerular basement membrane antibodies.Nephron Clin Pract,2007,107(2):c56-62.
23 Srivastava A,Rao GK,Segal PE,et al.Characteristics and outcome of crescentic glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Clin Rheumatol,2013,32(9):1317-1322.
24 Segelmark M,Hellmark T,Wieslander J.The prognostic significance in Goodpasture′s disease of specificity,titre and affinity of anti-glomerular-basement-membrane antibodies.Nephron Clin Pract,2003,94(3):c59-68.
25 Olson SW,Arbogast CB,Baker TP,et al.Asymptomatic autoantibodies associate with future anti-glomerular basement membrane disease.J Am Soc Nephrol,2011,22(10):1946-1952.
26 Jia XY,Yu JT,Hu SY,et al.Antibodies against linear epitopes on Goodpasture autoantigen in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.Clin Rheumatol,2017,36(9):2087-2094.
27 Li JN,Cui Z,Wang J,et al.Autoantibodies against Linear Epitopes of Myeloperoxidase in Anti-Glomerular Basement Membrane Disease.Clin J Am Soc Nephrol,2016,11(4):568-575.
28 Kumar SV,Kulkarni OP,Mulay SR,et al.Neutrophil Extracellular Trap-Related Extracellular Histones Cause Vascular Necrosis in Severe GN.J Am Soc Nephrol,2015,26(10):2399-2413.
29 Nakazawa D,Tomaru U,Suzuki A,et al.Abnormal conformation and impaired degradation of propylthiouracil-induced neutrophil extracellular traps:implications of disordered neutrophil extracellular traps in a rat model of myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis.Arthritis Rheum,2012,64(11):3779-3787.