НЕФРОПАТИИ, СВЯЗАННЫЕ С ПАТОЛОГИЕЙ СИСТЕМЫ КОМПЛЕМЕНТА


https://doi.org/10.21508/1027-4065-2016-61-6-21-31

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Аннотация

Обобщен научный материал по нефропатиям, связанным с патологией системы комплемента у детей и взрослых. Представлены клинические, иммунологические и морфологические отличия нефропатий, обусловленных патологией системы комплемента, от других заболеваний почек, прежде всего от гломерулонефрита (в том числе мембранопролиферативного варианта) и нефротического синдрома другой природы. Представлен патогенез развития указанных нефропатий, где выделены формы, связанные с генетическими мутациями, и варианты, обусловленные образованием аутоантител к компонентам комплемента. Показаны варианты и эффективность лечения иммуносупрессивными препаратами и экулизумабом в зависимости от патогенетических и клинических особенностей нефропатий, связанных с патологией системы комплемента.


Об авторах

В. В. Длин
ОСП «Научно-исследовательский клинический институт педиатрии имени академика Ю.Е. Вельтищева» ГБОУ ВПО РНИМУ им. Н.И. Пирогова Минздрава России, Москва, Россия
Россия
д.м.н., профессор, руководитель отдела наследственных и приобретенных болезней почек


М. С. Игнатова
ОСП «Научно-исследовательский клинический институт педиатрии имени академика Ю.Е. Вельтищева» ГБОУ ВПО РНИМУ им. Н.И. Пирогова Минздрава России, Москва, Россия
Россия
заслуженный деятель науки РФ, д.м.н., профессор, научный консультант


Список литературы

1. Gunn W.C. The variation in the amount of complement in the blood in some acute infectious diseases and its relation to the clinical features. J Pathol Bacteriol 1915; 19: 155–181.

2. Müller-Eberhard H.J., Nilsson U., Aronssen T. Isolation and characterization of two β1 glycoproteins of human serum. J Exp Med 1960; 111: 201.

3. Seligmann M., Hanau C. Étude immuno-electrophorétique du sérum de malades atteits de lupus érythémateux disséminé. Rev Hémat (Paris) 1958; 13: 239.

4. West C.D., Northway J.D., Davis N.C. Serum Levels of Beta1C Globulin, a Complement Component, in the Nephritides, Lipoid Nephrosis, and Other Conditions. J Clin Invest 1964; 43: 1507–1517.

5. West C.D., McAdams A.J., McConville J.M. et al. Hypo-complementaemic and normocomplementaemic persistent (chronic) glomerulonephritis: clinical and pathologic characteristics. J Pediatr 1965; 67: 1089–9112.

6. Lachmann P.J., Müller-Eberhard H.J., Kunkel H.G. et al. The localization of in vivo bound complement in tissue sections. J Exp Med 1962; 115: 63.

7. Spitzer R.E., Vallota E.H., Forristal J. et al. Serum C’3 lytic system in patients with glomerulonephritis. Science 1969; 164: 436–437.

8. Mathew T.H., Kincaid-Smith P. Membrano-proliferative glomerulonephritis (MPGN) with dense deposits in basement membrane. ASN Abstr 1971; 5: 51.

9. Habib R., Gubler M.C., Loirat C. et al. Dense deposit disease: a variant of membranoproliferative glomerulonephritis. Kidney Int 1975; 7: 204–215.

10. Marder H.K., Coleman T.H., Forristal J. et al. An inherited defect in the C3 convertase, C3b, Bb, associated with glomerulonephritis. Kidney Int 1983; 23: 749–758.

11. Levy M., Halbwachs-Mecarelli L., Gubler MC. et al. H deficiency in two brothers with atypical dense intramembranous deposit disease. Kidney Int 1986; 30: 949–956.

12. Linshaw M.A., Stapleton F.B., Cuppage F.E. et al. Hypocomplementemic glomerulonephritis in an infant and mother. Evidence for an abnormal form of C3. Am J Nephrol 1987; 7: 470–477.

13. Lopez-Larrea C., Dieguez M., Enguix A. et al. A familial deficiency of complement factor H. Biochem Soc Trans 1987; 15: 648–649.

14. Fakhouri F., Frémeaux-Bacchi V., Noël LH. et al. C3 glomerulopathy: a new classification. Nat Rev Nephrol 2010; 6: 8: 494–499.

15. Zipfel PF., Skerka C. Complement regulators and inhibitory proteins. Nat Rev Immunol 2009; 9: 729–740.

16. Walport M.J. Complement. First of two parts. N Engl J Med 2001; 344: 1058–1066.

17. Walport M.J. Complement. Second of two parts. N Engl J Med 2001; 344: 1140–1144.

18. Barbour T.D., Pickering M.C., Cook H.T. Recent insights into C3 glomerulopathy. Nephrol Dial Transplant 2013; 28: 7: 1685–1693.Lachmann P.J. The amplification loop of the complement pathways. Adv Immunol 2009; 104: 115–149.

19. D’Agati V.D., Bomback A.S. C3 glomerulopathy: what’s in a name? Kidney international 2012; 83: 379–381.

20. Deltas C., Gale D.,Cook T. et al. C3 Glomerulonephritis/ CFHR5 Nephropathy Is an Endemic Disease in Cyprus: Clinical and Molecular Findings in 21 Families. Adv Exp Med Biol 2013; 734: 189–196.

21. Deltas C., Gale D., Cook T. et al. The role of molecular genetics in diagnosing familial hematuria(s). Pediatr Nephrol 2012; 27: 8: 1221–1231.

22. Viswanathan G.K., Nada R., Kumar A. et al. Clinico-pathologic spectrum of C3 glomerulopathy an Indian experience. Diagnostic Pathology 2015; 10: 6: doi:10.1186/s13000-015– 0233.

23. Okuda Y., Ishikura K., Hamada R., et al. Membranoproliferative glomerulonephritis and C3 glomerulonephritis: Frequency, clinical features, and outcome in children. Nephrology (Carlton) 2015; 20: 4: 286–292.

24. Bomback A.S., Appel G.B. Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol 2012; 8: 11: 634–642.

25. Sethi S., Fervenza FC., Zhang Y. et al. C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int 2012; 82: 4: 465–473.

26. Servais A., Noël L.H., Roumenina L.T. et al. Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int 2012; 82: 4: 454–464.

27. Daha M.R., Fearon D.T., Austen K.F. C3 nephritic factor (C3NeF): stabilization of fluid phase and cell-bound alternative pathway convertase. J Immunol 1976; 116: 1–7.

28. Cameron J.S., Turner D.R., Heaton J. et al. Idiopathic mesangiocapillary glomerulonephritis. Comparison of types I and II in children and adults and long-term prognosis. Am J Med 1983; 74: 175–192.

29. Schwertz R., Rother U., Anders D. et al. Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: a long-term follow-up. Pediatr Allergy Immunol 2001; 12: 166–172.

30. Walport M.J., Davies K.A., Botto M. et al. C3 nephritic factor and SLE: report of four cases and review of the literature. QJM 1994; 87: 609–615.

31. Gewurz A.T., Imherr S.M., Strauss S. et al. C3 nephritic factor and hypocomplementaemia in a clinically healthy individual. Clin Exp Immunol 1983; 54: 253–258.

32. Strobel S., Zimmering M., Papp K. et al. Anti-factor B auto-antibody in dense deposit disease. Mol Immunol 2010; 47: 1476–1483.

33. Chen Q., Müller D., Rudolph B. et al. Combined C3b and factor B autoantibodies and MPGN type II. N Engl J Med 2011; 365: 2340–2342.

34. Meri S., Koistinen V., Miettinen A. et al. Activation of the alternative pathway of complement by monoclonal lambda light chains in membranoproliferative glomerulonephritis. J Exp Med 1992; 175: 939–950.

35. Jokiranta T.S., Solomon A., Pangburn M.K. et al. Nephritogenic lambda light chain dimer: a unique human miniautoantibody against complement factor H. J Immunol 1999; 163: 4590–4596.

36. Sethi S., Sukov W.R., Zhang Y. et al. Dense deposit disease associated with monoclonal gammopathy of undetermined significance. Am J Kidney Dis 2010; 56: 977–982.

37. Sethi S., Fervenza F.C. Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification. Semin Nephrol 2011; 31: 341–348.

38. Dragon-Durey M.A., Frémeaux-Bacchi V., Loirat C. et al. Heterozygous and homozygous factor H deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. J Am Soc Nephrol 2004; 15: 787–795.

39. Martínez-Barricarte R., Heurich M., Valdes-Cañedo F. et al. Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation. J Clin Invest 2010; 120: 3702–3712.

40. Licht C., Heinen S., Józsi M. et al. Deletion of Lys224 in regulatory domain 4 of factor H reveals a novel pathomechanism for dense deposit disease (MPGN II). Kidney Int 2006; 70: 42–50.

41. Habbig S., Mihatsch M.J., Heinen S. et al. C3 deposition glomerulopathy due to a functional factor H defect. Kidney Int 2009; 75: 1230–1234.

42. Wu J. Structure of complement fragment C3b-factor H and implications for host protection by complement regulators. Nat Immunol 2009; 10: 728–733.

43. Schejbel L., Schmidt I.M., Kirchhoff M. et al. Complement factor H deficiency and endocapillary glomerulonephritis due to paternal isodisomy and a novel factor H mutation. Genes Immun 2011; 12: 90–99.

44. Servais A., Frémeaux-Bacchi V., Lequintrec M. et al. Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome. J Med Genet 2007; 44: 193–99.

45. Servais A., Noël L.H., Dragon-Durey M.A. et al. Heterogeneous pattern of renal disease associated with homozygous factor H deficiency. Hum Pathol 2011; 42: 1305–1311.

46. Barbour T.D., Johnson S.A., Cohney S.C. et al. Thrombotic microangiopathy and associated renal disorders. Nephrol Dial Transplant 2012; 27: 2673–2685.

47. Appel G.B., Cook H.T., Hageman G. et al. Membranoproliferative glomerulonephritis type II (dense deposit disease): an update. J Am Soc Nephrol 2005; 16: 1392–1403.

48. Sethi S., Fervenza FC., Zhang Y. et al. Proliferative glomerulo-nephritis secondary to dysfunction of the alternative pathway of complement. Clin J Am Soc Nephrol 2011; 6: 1009–1017.

49. Zhang Y., Meyer N.C., Wang K. et al. Causes of alternative pathway dysregulation in dense deposit disease. Clin J Am Soc Nephrol 2012; 7: 265–274.

50. Nakopoulou L. Membranoproliferative glomerulonephritis. Nephrol Dial Transplant 2001; 16: 71–73.

51. Williams D.G., Scopes J.W., Peters D.K. Hypocomplementaemic membranoproliferative glomerulonephritis and nephrotic syndrome associated with partial lipodystrophy of the face and trunk. Proc R Soc Med 1972; 65: 591.

52. Pickering M.C., D’Agati V.D., Nester C.M. et al. C3 glomerulopathy: consensus report. Kidney Int 2013; 84: 1079–1089.

53. Nasr S.H., Valeri A.M., Appel G.B. et al. Dense deposit disease: clinicopathologic study of 32 pediatric and adult patients. Clin J Am Soc Nephrol 2009; 4: 22–32.

54. Cameron J.S., Vick R.M., Ogg C.S. et al. Plasma C3 and C4 concentrations in management of glomerulonephritis. Br Med J 1973; 3: 668–672.

55. Duvall-Young J., MacDonald M.K., McKechnie N.M. Fundus changes in (type II) mesangiocapillary glomerulonephritis simulating drusen: a histopathological report. Br J Ophthalmol 1989; 73: 297–302.

56. Mullins R.F., Aptsiauri N., Hageman G.S. Structure and composition of drusen associated with glomerulonephritis: implications for the role of complement activation in drusen bio-genesis. Eye 2001; 15: 3: 390–395.

57. Lu D.F., Moon M., Lanning L.D. et al. Clinical features and outcomes of 98 children and adults with dense deposit disease. Pediatr Nephrol 2012; 27: 773–781.

58. Walker P.D., Ferrario F., Joh K. et al. Dense deposit disease is not a membranoproliferative glomerulonephritis. Mod Pathol 2007; 20: 605–616.

59. Reis, E.S., Falcao, D.A., Isaac L. Clinical aspects and molecular basis of primary deficiencies of complement component C3 and its regulatory proteins factor I and factor H. Scand. J Immunol 2006; 63: 155–168.

60. Gale D.P., Goicoechea de Jorge E., Cook H.T. et al. Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis. Lancet 2010; 376: 794–801.

61. Athanasiou Y., Voskarides K., Gale DP. et al. Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees. Clin J Am Soc Nephrol 2011; 6: 1436–1446.

62. Gale D.P., Pickering M.C. Regulating complement in the kidney: insights from CFHR5 nephropathy. Dis Model Mech 2011; 4: 721–726.

63. Vernon K.A., Gale D.P., Goicoechea de Jorge E. et al. Recurrence of complement factor H-related protein 5 nephropathy in a renal transplant. Am J Transplant 2011; 11: 152–155.

64. Nester C.M, Smith R.J. Treatment options for C3 glomerulopathy. Curr Opin Nephrol Hypertens 2013; 22: 231–237.

65. Group KDIGOKGW. KDIGO Clinical Practice Guideline for Glomerulonphritis. Kidney Int (Suppl) 2012; 2: 198–199.

66. Daina E., Noris M., Remuzzi G. Eculizumab in a patient with dense-deposit disease. N Engl J Med 2012; 366: 1161–1163.

67. McCaughan J.A., O’Rourke D.M., Courtney A.E. Recurrent dense deposit disease after renal transplantation: an emerging role for complementary therapies. Am J Transplant 2012; 12: 1046–1051.

68. Banks R.A., May S., Wallington T. Acute renal failure in dense deposit disease: recovery after plasmapheresis. Br Med J (Clin Res Ed) 1982; 284: 1874–1875.

69. Krmar R.T., Holtback U., Linne T. et al. Acute renal failure in dense deposit disease: complete recovery after combination therapy with immunosuppressant and plasma exchange. Clin Nephrol 2011; 75: 1: 4–10.

70. West C.D., Witte D.P., McAdams A.J. Composition of nephritic factor-generated glomerular deposits in membranoproliferative glomerulonephritis type 2. Am J Kidney Dis 2001; 37: 1120–1130.

71. Vivarelli M., Pasini A., Emma F. Eculizumab for the treatment of dense-deposit disease. N Engl J Med 2012; 366: 1163– 1165.

72. Abrera-Abeleda M.A., Nishimura C., Frees K. et al. Allelic variants of complement genes associated with dense deposit disease. J Am Soc Nephrol 2011; 22: 1551–1559.

73. Bomback A.S., Smith R.J., Barile G.R. et al. Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol 2012; 7: 748–756.

74. Marks S.D., Rees L. Spontaneous clinical improvement in dense deposit disease. Pediatr Nephrol 2000; 14: 322–324.

75. Angelo J.R., Bell C.S., Braun M.C. Allograft failure in kidney transplant recipients with membranoproliferative glomerulo-nephritis. Am J Kidney Dis 2011; 57: 291–299.


Дополнительные файлы

Для цитирования: Длин В.В., Игнатова М.С. НЕФРОПАТИИ, СВЯЗАННЫЕ С ПАТОЛОГИЕЙ СИСТЕМЫ КОМПЛЕМЕНТА. Российский вестник перинатологии и педиатрии. 2016;61(6):21-31. https://doi.org/10.21508/1027-4065-2016-61-6-21-31

For citation: Dlin V.V., Ignatova M.S. NEPHROPATHIES ASSOCIATED WITH COMPLEMENT SYSTEM PATHOLOGY. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2016;61(6):21-31. (In Russ.) https://doi.org/10.21508/1027-4065-2016-61-6-21-31

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