Anti-Glomerular Basement Membrane Disease With Thrombotic Thrombocytopenic Purpura
Abstract
We report a 25-year-old man who was initially presented with Rapidly Progressive Glomerulonephritis (RPGN) in Shahid Modarress Hospital, Tehran, Iran. His diagnosis was Anti-Glomerular Basement Membrane (Anti-GBM) disease that was confirmed by kidney biopsy, as his serum Anti-GBM antibody was undetectable. Although the patient was on cyclophosphamide and high dose prednisolone, he developed Thrombotic Thrombocytopenic Purpura (TTP). We initiated treatment with a high dose of prednisone and plasmapheresis. Because of his poor response, rituximab was administered. After that, the patient’s hematologic criteria and clinical symptoms improved to an acceptable level. Our case is a unique form of negative serum Anti-GBM antibody disease which was complicated with TTP and responded to treatment with rituximab
[1] Couser WG. Rapidly Progressive Glomerulonephrits: Classifcaton, pathogenetc mechanisms, and therapy. American Journal of Kidney Diseases. 1988; 11(6):449-64. [DOI:10.1016/S0272- 6386(88)80079-9] [PMID]
[2] Atkins RC, Nikolic-Paterson DJ, Song Q, Lan HY. Modulators of crescentc glomerulonephrits. Journal of the American Society of Nephrology. 1996; 7(11):2271-8. [PMID]
[3] Li FK, Tse KC, Lam MF, Yip TP, Lui SL, Chan GS, et al. Incidence and outcome of antglomerular basement membrane disease in Chinese. Nephrology. 2004; 9(2):100-4. [DOI:10.1111/j.1440- 1797.2003.00234.x]
[4] Cui Z, Zhao MH, Xin G, Wang HY. Characteristcs and prognosis of Chinese patents with Ant-Glomerular Basement Membrane disease. Nephron Clinical Practce. 2005; 99(2):c49-55. [DOI:10.1159/000083133]
[5] Yu XJ, Han SS, Wang SX, Jia XY, Guo WY, Cui Z, et al. Ant-Glomerular Basement Membrane glomerulonephrits with thrombotc microangiopathy: A case report. Immunologic Research. 2017; 65(4):769- 73. [DOI:10.1007/s12026-017-8918 y]
[6] George JN, Nester CM. Syndromes of thrombotc microangiopathy. New England Journal of Medicine. 2014; 371(7):654-66. [DOI:10.1056/NEJMra1312353]
[7] Touzot M, Poisson J, Faguer S, Ribes D, Cohen P, Geffray L, et al. Rituximab in Ant-GBM disease: A retrospectve study of 8 patents. Journal of Autoimmunity. 2015; 60:74-9. [DOI:10.1016/j. jaut.2015.04.003]
[8] Stave GM, Croker BP. Thrombotc microangiopathy in Ant-Glomerular Basement Membrane glomerulonephrits. Archives of Pathology & Laboratory Medicine. 1984; 108(9):747-51. [PMID]
[9] Terryn W, Benoit D, Van Loo A, Peeters P, Schepkens H, Cokelaere K, et al. Goodpasture’s syndrome associated with autoimmune thrombotc thrombocytopenic purpura- An unusual case. Nephrology Dialysis Transplantaton. 2007; 22(12):3672-3. [DOI:10.1093/ ndt/gfm483]
[10] Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of ant- glomerular basement membrane antbody disease treated with plasma exchange and immunosuppression. Annals of Internal Medicine. 2001; 134(11):1033-42. [DOI:10.7326/0003-4819-134- 11-200106050-00009]
[11] Salama AD, Levy JB, Lightstone L, Pusey CD. Goodpasture’s disease. The Lancet. 2001; 358(9285):917-20 [DOI:10.1016/S0140- 6736(01)06077-9]
[12] Cameron JS, Davison AM, Grunfeld JP, Kerr DNS, Ritz E, Rees A, et al. Oxford text book of clinical nephrology: Three volume set (Oxford Medical Publicatons). Oxford: University Press Oxford; 1992.
[13] Pusey CD. Ant-Glomerular Basement Membrane disease. Kidney Internatonal. 2003; 64(4):1535-50. [DOI:10.1046/j.1523- 1755.2003.00241.x]
[14] Peto P, Salama AD. Update on antglomerular basement membrane disease. Current Opinion in Rheumatology. 2011; 23(1):32-7. [DOI:10.1097/BOR.0b013e328341009f] [PMID]
[15] Baldwin DS, Neugarten J, Feiner HD, Gluck M, Spinowitz B. The existence of a protracted course in crescentc glomerulonephrits. Kidney Internatonal. 1987; 31(3):790-4. [DOI:10.1038/ki.1987.67]
[16] Nachman PH, Jennete JC, Falk RJ. Primary glomerular disease. In: Brenner BM, Rector FC, editor. Brenner and Rector’s The Kidney. Philadelphia: Saunder; 2007.
[17] Chen L, Hellmark T, Pedcdhenko V, Hudson BG, Pusey CD, Fox JW, et al. A nephritogenic peptde induces intermolecular epitope spreading on collagen IV in experimental autoimmune glomerulonephrits. Journal of the American Society of Nephrology. 2006; 17(11):3076-81. [DOI:10.1681/ASN.2006070688] [PMID] [PMCID]
[18] Salama AD, Dougan T, Levy JB, Cook HT, Morgan SH, Naudeer S, et al. Goodpasture’s disease in the absence of circulatng Ant-Glomerular Basement Membrane antbodies as detected by standard techniques. American Journal of Kidney Diseases. 2002; 39(6):1162-7. [DOI:10.1053/ajkd.2002.33385] [PMID]
[19] Stolk M, Carl D, Massey HD. Antbody-negatve Goodpasture’s disease. NDT Plus. 2010; 3(3):253-6. [DOI:10.1093/ndtplus/sfq003] [PMID] [PMCID]
[20] Borkum M, Calligaro GL, Symons G. A case of Ant-Glomerular Basement Membrane (GBM) negatve Goodpasture’s Disease. Journal of Pulmonary and Respiratory Medicine. 2014; 4(213):213-15. [DOI:10.4172/2161-105X.1000213]
[21] Kidney Disease Improving Global Outcomes. Clinical practce guideline for glomerulonephrits kidney. Ofcial Journal of the Internatonal Society of Nephrology. 2012; 2(2):240-2.
[22] Serisier DJ, Wong RC, Armstrong JG. Alveolar haemorrhage in Ant-Glomerular Basement Membrane disease without detectable antbodies by conventonal assays. Thorax. 2006; 61(7):636-9. [DOI:10.1136/thx.2004.028985] [PMID] [PMCID]
[23] Gu B, Magil AB, Barbour SJ. Frequently relapsing Ant-Glomerular Basement Membrane antbody disease with changing clinical phenotype and antbody characteristcs over tme. Clinical Kidney Journal. 2016; 9(5):661-4. [DOI:10.1093/ckj/sfw048] [PMID] [PMCID]
[24] Singhal P, Ren KY, Curts BM, MacPherson I, Avila-Casado C. Atypical noncrescentc antglomerular basement membrane disease with concurrent thin basement membrane nephropathy. Kidney Internatonal Reports. 2018; 3(4):991 6. [DOI:10.1016/j.ekir.2018.03.012]
[25] Cui Z, Zhao MH. Advances in human antglomerular basement membrane disease. Nature Reviews Nephrology. 2011; 7(12):697- 705. [DOI:10.1038/nrneph.2011.89]
[26] George JN. The remarkable diversity of thrombotc thrombocytopenic purpura: A perspectve. Blood Advances. 2018; 2(12):1510-6. [DOI:10.1182/bloodadvances.2018018432] [PMID] [PMCID]
[27] Franchini M, Mannucci PM. Advantages and limits of ADAMTS13 testng in thrombotc thrombocytopenic purpura. Blood Transfusion. 2008; 6(3):127-35. [Doi:10.2450/2008.0056-07] [PMID] [PMCID]
[28] Saha M, McDaniel JK, Zheng XL. Thrombotc thrombocytopenic purpura: Pathogenesis, diagnosis and potental novel therapeutcs. Journal of Thrombosis and Haemostasis. 2017; 15(10):1889-900. [DOI:10.1111/jth.13764] [PMID] [PMCID]
[29] Scully M, Cataland S, Coppo P, De La Rubia J, Friedman KD, Kremer Hovinga J, et al. Consensus on the standardizaton of terminology in thrombotc thrombocytopenic purpura and related thrombotc microangiopathies. Journal of Thrombosis and Haemostasis. 2017; 15(2):312-22. [DOI:10.1111/jth.13571] [PMID]
[30] Torok N, Niazi M, Al Ahwel Y, Taleb M, Taji J, Assaly R. Thrombotc thrombocytopenic purpura associated with Ant Glomerular Basement Membrane disease. Nephrology Dialysis Transplantaton. 2010; 25(10):3446-9. [DOI:10.1111/j.1440-1797.2006.00584.x]
[PMID]
[31] Moake JL. Thrombotc microangiopathies. The New England Journal of Medicine. 2002; 347(8):589-600. [DOI:10.1056/NEJMra020528] [PMID]
[32] Shin JI, Park SJ, Kim JH. Is there a link between thrombotc thrombocytopenic purpura and Ant-Glomerular Basement Membrane disease? Nephrology Dialysis Transplantaton. 2011; 26(6):2058-9. [DOI:10.1093/ndt/gfr036]
[33] Le Hir M, Ryffel B, Schatzmann U. IL-12-dependent, IFN-independent experimental glomerulonephrits. Kidney and Blood Pressure Research. 2001; 24:27-32. [DOI:10.1159/000054202]
[34] Idorn T, Schejbel L, Rydahl C, Heaf JG, Jølvig KR, Bergstrøm M, et al. Ant-Glomerular Basement Membrane glomerulonephrits and thrombotc microangiopathy in frst degree relatves; A case report. BMC Nephrology. 2012; 13:64. [DOI:10.1186/1471-2369-13-64]
[35] Miki T, Akimoto T, Sugase T, Numata A, Otani N, Iwazu Y, et al. Ant-Glomerular Basement Membrane glomerulonephrits complicated by thrombocytopenia. Internal Medicine Journal. 2012; 51(24):3395-9. [DOI:10.2169/internalmedicine.51.8507] [PMID]
[36] Torok N, Niazi M, Al Ahwel Y, Taleb M, Taji J, Assaly R. Thrombotc thrombocytopenic purpura associated with Ant Glomerular Basement Membrane disease. Nephrology Dialysis Transplantaton. 2010; 25(10):3446-9. [DOI:10.1093/ndt/gfq437]
[37] Gowrishankar S, Patro A, Maitra S. Ant-GBM antbody disease sans crescents with thrombotc microangiopathy. NDT Plus. 2009; 2(4):282-4. [DOI:10.1093/ndtplus/sfp056] [PMID] [PMCID]
[38] Watanabe H, Kitagawa W, Suzuki K, Yoshino M, Aoyama R, Miura N, et al. Thrombotc thrombocytopenic purpura in a patent with Rapidly Progressive Glomerulonephrits with both Ant-Glomerular Basement Membrane antbodies and myeloperoxidase ant-neutrophil cytoplasmic antbodies. Clinical and Experimental Nephrology. 2010; 14(6):598-601. [DOI:10.1007/s10157-010-0312-1]
[39] Allford SL, Hunt BJ, Rose P, Machin SJ. Haemostasis and thrombosis task force of the Britsh commitee for standards in haematology. Guidelines on the diagnosis and management of the thrombotc microangiopathic haemolytc anaemias. Britsh Journal of Haematology. 2003; 120(4):556-73. [DOI:10.1046/j.1365-2141.2003.04049.x] [PMID]
[40] Blombery P, Scully M. Management of thrombotc thrombocytopenic purpura: Current perspectves. Journal of Blood Medicine. 2014; 5:15-23. [DOI:10.2147/JBM.S46458] [PMID] [PMCID]
[41] Chen H, Fu A, Wang J, Wu T, Li Z, Tang J, et al. Rituximab as frstline treatment for acquired thrombotc thrombocytopenic purpura. Journal of Internatonal Medical Research. 2017; 45(3):1253-60. [DOI:10.1177/0300060517695646] [PMID] [PMCID]
[2] Atkins RC, Nikolic-Paterson DJ, Song Q, Lan HY. Modulators of crescentc glomerulonephrits. Journal of the American Society of Nephrology. 1996; 7(11):2271-8. [PMID]
[3] Li FK, Tse KC, Lam MF, Yip TP, Lui SL, Chan GS, et al. Incidence and outcome of antglomerular basement membrane disease in Chinese. Nephrology. 2004; 9(2):100-4. [DOI:10.1111/j.1440- 1797.2003.00234.x]
[4] Cui Z, Zhao MH, Xin G, Wang HY. Characteristcs and prognosis of Chinese patents with Ant-Glomerular Basement Membrane disease. Nephron Clinical Practce. 2005; 99(2):c49-55. [DOI:10.1159/000083133]
[5] Yu XJ, Han SS, Wang SX, Jia XY, Guo WY, Cui Z, et al. Ant-Glomerular Basement Membrane glomerulonephrits with thrombotc microangiopathy: A case report. Immunologic Research. 2017; 65(4):769- 73. [DOI:10.1007/s12026-017-8918 y]
[6] George JN, Nester CM. Syndromes of thrombotc microangiopathy. New England Journal of Medicine. 2014; 371(7):654-66. [DOI:10.1056/NEJMra1312353]
[7] Touzot M, Poisson J, Faguer S, Ribes D, Cohen P, Geffray L, et al. Rituximab in Ant-GBM disease: A retrospectve study of 8 patents. Journal of Autoimmunity. 2015; 60:74-9. [DOI:10.1016/j. jaut.2015.04.003]
[8] Stave GM, Croker BP. Thrombotc microangiopathy in Ant-Glomerular Basement Membrane glomerulonephrits. Archives of Pathology & Laboratory Medicine. 1984; 108(9):747-51. [PMID]
[9] Terryn W, Benoit D, Van Loo A, Peeters P, Schepkens H, Cokelaere K, et al. Goodpasture’s syndrome associated with autoimmune thrombotc thrombocytopenic purpura- An unusual case. Nephrology Dialysis Transplantaton. 2007; 22(12):3672-3. [DOI:10.1093/ ndt/gfm483]
[10] Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of ant- glomerular basement membrane antbody disease treated with plasma exchange and immunosuppression. Annals of Internal Medicine. 2001; 134(11):1033-42. [DOI:10.7326/0003-4819-134- 11-200106050-00009]
[11] Salama AD, Levy JB, Lightstone L, Pusey CD. Goodpasture’s disease. The Lancet. 2001; 358(9285):917-20 [DOI:10.1016/S0140- 6736(01)06077-9]
[12] Cameron JS, Davison AM, Grunfeld JP, Kerr DNS, Ritz E, Rees A, et al. Oxford text book of clinical nephrology: Three volume set (Oxford Medical Publicatons). Oxford: University Press Oxford; 1992.
[13] Pusey CD. Ant-Glomerular Basement Membrane disease. Kidney Internatonal. 2003; 64(4):1535-50. [DOI:10.1046/j.1523- 1755.2003.00241.x]
[14] Peto P, Salama AD. Update on antglomerular basement membrane disease. Current Opinion in Rheumatology. 2011; 23(1):32-7. [DOI:10.1097/BOR.0b013e328341009f] [PMID]
[15] Baldwin DS, Neugarten J, Feiner HD, Gluck M, Spinowitz B. The existence of a protracted course in crescentc glomerulonephrits. Kidney Internatonal. 1987; 31(3):790-4. [DOI:10.1038/ki.1987.67]
[16] Nachman PH, Jennete JC, Falk RJ. Primary glomerular disease. In: Brenner BM, Rector FC, editor. Brenner and Rector’s The Kidney. Philadelphia: Saunder; 2007.
[17] Chen L, Hellmark T, Pedcdhenko V, Hudson BG, Pusey CD, Fox JW, et al. A nephritogenic peptde induces intermolecular epitope spreading on collagen IV in experimental autoimmune glomerulonephrits. Journal of the American Society of Nephrology. 2006; 17(11):3076-81. [DOI:10.1681/ASN.2006070688] [PMID] [PMCID]
[18] Salama AD, Dougan T, Levy JB, Cook HT, Morgan SH, Naudeer S, et al. Goodpasture’s disease in the absence of circulatng Ant-Glomerular Basement Membrane antbodies as detected by standard techniques. American Journal of Kidney Diseases. 2002; 39(6):1162-7. [DOI:10.1053/ajkd.2002.33385] [PMID]
[19] Stolk M, Carl D, Massey HD. Antbody-negatve Goodpasture’s disease. NDT Plus. 2010; 3(3):253-6. [DOI:10.1093/ndtplus/sfq003] [PMID] [PMCID]
[20] Borkum M, Calligaro GL, Symons G. A case of Ant-Glomerular Basement Membrane (GBM) negatve Goodpasture’s Disease. Journal of Pulmonary and Respiratory Medicine. 2014; 4(213):213-15. [DOI:10.4172/2161-105X.1000213]
[21] Kidney Disease Improving Global Outcomes. Clinical practce guideline for glomerulonephrits kidney. Ofcial Journal of the Internatonal Society of Nephrology. 2012; 2(2):240-2.
[22] Serisier DJ, Wong RC, Armstrong JG. Alveolar haemorrhage in Ant-Glomerular Basement Membrane disease without detectable antbodies by conventonal assays. Thorax. 2006; 61(7):636-9. [DOI:10.1136/thx.2004.028985] [PMID] [PMCID]
[23] Gu B, Magil AB, Barbour SJ. Frequently relapsing Ant-Glomerular Basement Membrane antbody disease with changing clinical phenotype and antbody characteristcs over tme. Clinical Kidney Journal. 2016; 9(5):661-4. [DOI:10.1093/ckj/sfw048] [PMID] [PMCID]
[24] Singhal P, Ren KY, Curts BM, MacPherson I, Avila-Casado C. Atypical noncrescentc antglomerular basement membrane disease with concurrent thin basement membrane nephropathy. Kidney Internatonal Reports. 2018; 3(4):991 6. [DOI:10.1016/j.ekir.2018.03.012]
[25] Cui Z, Zhao MH. Advances in human antglomerular basement membrane disease. Nature Reviews Nephrology. 2011; 7(12):697- 705. [DOI:10.1038/nrneph.2011.89]
[26] George JN. The remarkable diversity of thrombotc thrombocytopenic purpura: A perspectve. Blood Advances. 2018; 2(12):1510-6. [DOI:10.1182/bloodadvances.2018018432] [PMID] [PMCID]
[27] Franchini M, Mannucci PM. Advantages and limits of ADAMTS13 testng in thrombotc thrombocytopenic purpura. Blood Transfusion. 2008; 6(3):127-35. [Doi:10.2450/2008.0056-07] [PMID] [PMCID]
[28] Saha M, McDaniel JK, Zheng XL. Thrombotc thrombocytopenic purpura: Pathogenesis, diagnosis and potental novel therapeutcs. Journal of Thrombosis and Haemostasis. 2017; 15(10):1889-900. [DOI:10.1111/jth.13764] [PMID] [PMCID]
[29] Scully M, Cataland S, Coppo P, De La Rubia J, Friedman KD, Kremer Hovinga J, et al. Consensus on the standardizaton of terminology in thrombotc thrombocytopenic purpura and related thrombotc microangiopathies. Journal of Thrombosis and Haemostasis. 2017; 15(2):312-22. [DOI:10.1111/jth.13571] [PMID]
[30] Torok N, Niazi M, Al Ahwel Y, Taleb M, Taji J, Assaly R. Thrombotc thrombocytopenic purpura associated with Ant Glomerular Basement Membrane disease. Nephrology Dialysis Transplantaton. 2010; 25(10):3446-9. [DOI:10.1111/j.1440-1797.2006.00584.x]
[PMID]
[31] Moake JL. Thrombotc microangiopathies. The New England Journal of Medicine. 2002; 347(8):589-600. [DOI:10.1056/NEJMra020528] [PMID]
[32] Shin JI, Park SJ, Kim JH. Is there a link between thrombotc thrombocytopenic purpura and Ant-Glomerular Basement Membrane disease? Nephrology Dialysis Transplantaton. 2011; 26(6):2058-9. [DOI:10.1093/ndt/gfr036]
[33] Le Hir M, Ryffel B, Schatzmann U. IL-12-dependent, IFN-independent experimental glomerulonephrits. Kidney and Blood Pressure Research. 2001; 24:27-32. [DOI:10.1159/000054202]
[34] Idorn T, Schejbel L, Rydahl C, Heaf JG, Jølvig KR, Bergstrøm M, et al. Ant-Glomerular Basement Membrane glomerulonephrits and thrombotc microangiopathy in frst degree relatves; A case report. BMC Nephrology. 2012; 13:64. [DOI:10.1186/1471-2369-13-64]
[35] Miki T, Akimoto T, Sugase T, Numata A, Otani N, Iwazu Y, et al. Ant-Glomerular Basement Membrane glomerulonephrits complicated by thrombocytopenia. Internal Medicine Journal. 2012; 51(24):3395-9. [DOI:10.2169/internalmedicine.51.8507] [PMID]
[36] Torok N, Niazi M, Al Ahwel Y, Taleb M, Taji J, Assaly R. Thrombotc thrombocytopenic purpura associated with Ant Glomerular Basement Membrane disease. Nephrology Dialysis Transplantaton. 2010; 25(10):3446-9. [DOI:10.1093/ndt/gfq437]
[37] Gowrishankar S, Patro A, Maitra S. Ant-GBM antbody disease sans crescents with thrombotc microangiopathy. NDT Plus. 2009; 2(4):282-4. [DOI:10.1093/ndtplus/sfp056] [PMID] [PMCID]
[38] Watanabe H, Kitagawa W, Suzuki K, Yoshino M, Aoyama R, Miura N, et al. Thrombotc thrombocytopenic purpura in a patent with Rapidly Progressive Glomerulonephrits with both Ant-Glomerular Basement Membrane antbodies and myeloperoxidase ant-neutrophil cytoplasmic antbodies. Clinical and Experimental Nephrology. 2010; 14(6):598-601. [DOI:10.1007/s10157-010-0312-1]
[39] Allford SL, Hunt BJ, Rose P, Machin SJ. Haemostasis and thrombosis task force of the Britsh commitee for standards in haematology. Guidelines on the diagnosis and management of the thrombotc microangiopathic haemolytc anaemias. Britsh Journal of Haematology. 2003; 120(4):556-73. [DOI:10.1046/j.1365-2141.2003.04049.x] [PMID]
[40] Blombery P, Scully M. Management of thrombotc thrombocytopenic purpura: Current perspectves. Journal of Blood Medicine. 2014; 5:15-23. [DOI:10.2147/JBM.S46458] [PMID] [PMCID]
[41] Chen H, Fu A, Wang J, Wu T, Li Z, Tang J, et al. Rituximab as frstline treatment for acquired thrombotc thrombocytopenic purpura. Journal of Internatonal Medical Research. 2017; 45(3):1253-60. [DOI:10.1177/0300060517695646] [PMID] [PMCID]
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| Issue | Vol 3 No 4 (2018): Autumn | |
| Section | Case Report(s) | |
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| Ant-glomerular basement Glomerulonephrits TTP | ||
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How to Cite
1.
Alirezaei A, Bakhtiyari M, Zare E. Anti-Glomerular Basement Membrane Disease With Thrombotic Thrombocytopenic Purpura. CRCP. 2019;3(4):106-112.
