От пурпуры Шенлейна–Геноха до IgА-васкулита: патогенетические аспекты болезни
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Ключевые слова: системные васкулиты, пурпура Шенлейна–Геноха, геморрагический васкулит, IgA-васкулит, IgA-нефропатия, дефектное гликозилирование IgA, реакция Шварцмана, эндотоксемия.
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Investigation’s history and nomenclature’s evolution of the IgA-vasculitis are presented in the article. Pathogenesis of the renal and skin damages is discussed in details, particularly abnormalities of the IgA-immunity and systemic endotoxemia. Relevant world’s literature is cited.
Keywords: systemic vasculitides, Schönlein–Henoch purpura, hemorrhagic vasculitis, IgA-vasculitis, IgA-nephropathy, defective IgA glycosylation, Shwartzman reaction, endotoxemia.
2. Schönlein JL, in: Allgemeine und specielle Pathologie und Therapie. Vol. 2. 3rd ed. Wurzburg: Herisau; 1837. P. 48.
3. Henoch EH. Uber eine eigenthumbliche form von purpura. Berl Klin Wochnschr. 1874;11:641-643.
4. Henoch EH. Die hamorrhagische diathese-purpura. In: Vorlesungen uber Kinderkrankheiten. Berlin: Hirschwald; 1895. Bd 9. S. 847.
5. Osler W. The Visceral Lesions of Purpura and Allied Conditions. Brit Med J. 1914;1:517.
6. Frank E. Hamorrhagische Diathesen. In: Schittenhelm Handbuch der Krankheiten des Blutes und der blutbildenden Organe. Berlin: Springer; 1925.
7. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb;37(2):187-192.
8. Насонова В.А. Геморрагический васкулит (Болезнь Шенлейна—Геноха). Москва: Медгиз; 1959. 175 с. [Nasonova VA. Gemorragicheskiy vaskulit (Bolezn' Shenleyna—Genokha) [Hemorrhagic vasculitis (Schönlein-Henoch disease)]. Moscow: Medgiz; 1959. 175 p. (In Russ.)].
9. Jennette JC, Falk RJ, Bacon PA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. doi: 10.1002/art.37715
10. Berger J, Hinglais N. Les dépôts intercapillaires d'IgA-IgG. J Urol Nephrol. 1968;74:694-695.
11. Kauffmann RH, Herrmann WA, Meÿer CJ, et al. Circulating IgA-immune complexes in Henoch-Schönlein purpura. A longitudinal study of their relationship to disease activity and vascular deposition of IgA. Am J Med. 1980;69(6):859-866.
12. Pabst O. New concepts in the generation and functions of IgA. Nat Rev Immunol. 2012;12:821-832. doi: 10.1038/nri3322
13. Jacob CM, Pastorino AC, Fahl K, et al. Autoimmunity in IgA deficiency: revisiting the role of IgA as a silent housekeeper. J Clin Immunol. 2008;28 Suppl 1:S56-61. doi: 10.1007/s10875-007-9163-2
14. Novak J, Moldoveanu Z, Renfrow MB. IgA nephropathy and Henoch-Schoenlein purpura nephritis: aberrant glycosylation of IgA1, formation of IgA1-containing immune complexes, and activation of mesangial cells. Contrib Nephrol. 2007;157:134-138. doi: 10.1159/000102455
15. Heineke MH, Ballering AV, Jamin A. New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmun Rev. 2017;16(12):1246-1253. doi: 10.1016/j.autrev.2017.10.009
16. Kokubo T, Hiki Y, Iwase H, Tanaka A, Toma K, Hotta K, Kobayashi Y. Protective role of IgA1 glycans against IgA1self-aggregation and adhesion to extracellular matrix proteins. J Am Soc Nephrol. 1998;9:2048-2054.
17. Novak J, Julian BA, Tomana M, et al. IgA glycosylation and IgA immune complexes in the pathogenesis of IgA nephropathy. Semin Nephrol. 2008;28:78-87. doi: 10.1016/j.semnephrol.2007.10.009
18. Davin JC, Ten Berge IJ, Weening JJ. What is the difference between IgA nephropathy and Henoch-Schonlein purpura nephritis? Kidney Int. 2001;59:823-834. doi: 10.1046/j.1523-1755.2001.059003823.x
19. Waldo FB. Is Henoch-Schönlein purpura the systemic form of IgA nephropathy? Am J Kidney Dis. 1988;12(5):373-377.
20. Smith AC, Molyneux K, Feehally J, Barratt J. O-glycosylation of serum IgA1 antibodies against mucosal and systemic antigens in IgA nephropathy. J Am Soc Nephrol. 2006;17(12):3520-3528. doi: 10.1681/ASN.2006060658
21. Launay P, Grossetete B, Arcos-Fajardo M, et al. Fcalpha receptor (CD89) mediates the development of immunoglobulin A (IgA) nephropathy (Berger's disease). Evidence for pathogenic soluble receptor-IgA complexes in patients and CD89 transgenic mice. J Exp Med. 2000;191:1999-2009.
22. Haddad E, Moura IC, Arcos-Fajardo M, Macher MA, Baudouin V, Alberti C, Loirat C, Monteiro RC, Peuchmaur M. Enhanced expression of the CD71 mesangial IgA1 receptor in Berger disease and Henoch-Schönlein nephritis: association between CD71 expression and IgA deposits. J Am Soc Nephrol. 2003;14:327-337.
23. Wyatt RJ. The complement system in IgA nephropathy and Henoch-Schönlein purpura: functional and genetic aspects. Contrib Nephrology. 1993;104:82-91.
24. Endo M, Ohi H, Ohsawa I, Fujita T, Matsushita M, Fujita T. Glomerular deposition of mannose-binding lectin (MBL) indicates a novel mechanism of complement activation in IgA-nephropathy. Nephrol Dial Transplant. 1998;13:1984-1990.
25. Allen AC, Willis FR, Beattie TJ, Feehally J. Abnormal IgA glycosylation in Schönlein–Henoch purpura restricted to patients with clinical nephritis. Nephrol Dial Transplant. 1998;13:930-934.
26. Vuong MT, Hahn-Zoric M, Lundberg S, et al. Association of soluble CD89 levels with disease progression but not susceptibility in IgA nephropathy. Kidney Int. 2001;78:1281-1287.
27. Van der Helm-van Mil AH, Smith AC, Pouria S, et al. Immunoglobulin A multiple myeloma presenting with Henoch-Schönlein purpura associated with reduced sialylation of IgA1. Br J Haematol. 2003;122(6):915-917.
28. Van Hale HM, Gibson LE, Schroeter AL. Henoch-Schönlein vasculitis: direct immunofluorescence study of uninvolved skin. J Am Acad Dermatol. 1986;15(4 Pt 1):665-670.
29. Faille-Kuyper EH, Kater L, Kuijten RH, et al. Occurrence of vascular IgA deposits in clinically normal skin of patients with renal disease. Kidney Int. 1976;9(5):424-429.
30. Thompson AJ, Chan YL, Woodroffe AJ, et al. Vascular IgA deposits in clinically normal skin of patients with renal disease. Pathology. 1980;12(3):407-413.
31. Hené RJ, Velthuis P, van de Wiel A. et al. The relevance of IgA deposits in vessel walls of clinically normal skin. A prospective study. Arch Intern Med. 1986 Apr;146(4):745-749.
32. Saklayen MG, Schroeter AL, Nafz MA, Jalil K. IgA deposition in the skin of patients with alcoholic liver disease. J Cutan Pathol. 1996;23(1):12-18.
33. Мухин Н.А., Гуляев С.В., Кривошеев О.Г. и др. Клиническое и прогностическое значение поражения желудочно-кишечного тракта при системных сосудистых пурпурах. Терапевтический архив. 2003;75(2):50-54 [Mukhin NA, Gulyaev SV, Krivosheev ОG, et al. Clinical and prognostic implications of gastrointestinal tract lesions in systemic vascular purpuras. Terapevticheskiy Arkhiv = Therapeutic Archive. 2003;75(2):50-54 (In Russ.)].
34. Bode C, Bode JC. Effect of alcohol consumption on the gut. Best Pract Res Clin Gastroenterol. 2003;17(4):575-592.
35. Shwartzman G. A new phenomenon of local skin reactivity to B. Typhosus culture filtrate. Exptl Biol Med. 1928;25(7):560-561.
36. Кривошеев О.Г. Системные сосудистые пурпуры – клинико-этиологические варианты: Дис. канд. мед. наук. Москва; 1999 [Krivosheev OG. Sistemnye sosudistye purpury – kliniko-etiologicheskie varianty [Systemic vascular purpuras: clinical and etiologic types]. Dis. … kand. med. sci. Moscow; 1999 (In Russ.)].
37. Davin JC, Forget P, Mahieu PR. Increased intestinal permeability to (51 Cr) EDTA is correlated with IgA immune complex-plasma levels in children with IgA-associated nephropathies. Acta Paediatr Scand. 1988;77(1):118-124.
38. Davin JC, Mahieu PR. Sequential measurements of intestinal permeability to [51Cr] EDTA in children with Henoch-Schönlein purpura nephritis. Nephron. 1992;60(4):498-499.
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1. Heberden W. Commentaries on history and cure diseases. London; 1802.
2. Schönlein JL, in: Allgemeine und specielle Pathologie und Therapie. Vol. 2. 3rd ed. Wurzburg: Herisau; 1837. P. 48.
3. Henoch EH. Uber eine eigenthumbliche form von purpura. Berl Klin Wochnschr. 1874;11:641-643.
4. Henoch EH. Die hamorrhagische diathese-purpura. In: Vorlesungen uber Kinderkrankheiten. Berlin: Hirschwald; 1895. Bd 9. S. 847.
5. Osler W. The Visceral Lesions of Purpura and Allied Conditions. Brit Med J. 1914;1:517.
6. Frank E. Hamorrhagische Diathesen. In: Schittenhelm Handbuch der Krankheiten des Blutes und der blutbildenden Organe. Berlin: Springer; 1925.
7. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb;37(2):187-192.
8. [Hemorrhagic vasculitis (Schönlein-Henoch disease)]. Moscow: Medgiz; 1959. 175 p. (In Russ.)].
9. Jennette JC, Falk RJ, Bacon PA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. doi: 10.1002/art.37715
10. Berger J, Hinglais N. Les dépôts intercapillaires d'IgA-IgG. J Urol Nephrol. 1968;74:694-695.
11. Kauffmann RH, Herrmann WA, Meÿer CJ, et al. Circulating IgA-immune complexes in Henoch-Schönlein purpura. A longitudinal study of their relationship to disease activity and vascular deposition of IgA. Am J Med. 1980;69(6):859-866.
12. Pabst O. New concepts in the generation and functions of IgA. Nat Rev Immunol. 2012;12:821-832. doi: 10.1038/nri3322
13. Jacob CM, Pastorino AC, Fahl K, et al. Autoimmunity in IgA deficiency: revisiting the role of IgA as a silent housekeeper. J Clin Immunol. 2008;28 Suppl 1:S56-61. doi: 10.1007/s10875-007-9163-2
14. Novak J, Moldoveanu Z, Renfrow MB. IgA nephropathy and Henoch-Schoenlein purpura nephritis: aberrant glycosylation of IgA1, formation of IgA1-containing immune complexes, and activation of mesangial cells. Contrib Nephrol. 2007;157:134-138. doi: 10.1159/000102455
15. Heineke MH, Ballering AV, Jamin A. New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmun Rev. 2017;16(12):1246-1253. doi: 10.1016/j.autrev.2017.10.009
16. Kokubo T, Hiki Y, Iwase H, Tanaka A, Toma K, Hotta K, Kobayashi Y. Protective role of IgA1 glycans against IgA1self-aggregation and adhesion to extracellular matrix proteins. J Am Soc Nephrol. 1998;9:2048-2054.
17. Novak J, Julian BA, Tomana M, et al. IgA glycosylation and IgA immune complexes in the pathogenesis of IgA nephropathy. Semin Nephrol. 2008;28:78-87. doi: 10.1016/j.semnephrol.2007.10.009
18. Davin JC, Ten Berge IJ, Weening JJ. What is the difference between IgA nephropathy and Henoch-Schonlein purpura nephritis? Kidney Int. 2001;59:823-834. doi: 10.1046/j.1523-1755.2001.059003823.x
19. Waldo FB. Is Henoch-Schönlein purpura the systemic form of IgA nephropathy? Am J Kidney Dis. 1988;12(5):373-377.
20. Smith AC, Molyneux K, Feehally J, Barratt J. O-glycosylation of serum IgA1 antibodies against mucosal and systemic antigens in IgA nephropathy. J Am Soc Nephrol. 2006;17(12):3520-3528. doi: 10.1681/ASN.2006060658
21. Launay P, Grossetete B, Arcos-Fajardo M, et al. Fcalpha receptor (CD89) mediates the development of immunoglobulin A (IgA) nephropathy (Berger's disease). Evidence for pathogenic soluble receptor-IgA complexes in patients and CD89 transgenic mice. J Exp Med. 2000;191:1999-2009.
22. Haddad E, Moura IC, Arcos-Fajardo M, Macher MA, Baudouin V, Alberti C, Loirat C, Monteiro RC, Peuchmaur M. Enhanced expression of the CD71 mesangial IgA1 receptor in Berger disease and Henoch-Schönlein nephritis: association between CD71 expression and IgA deposits. J Am Soc Nephrol. 2003;14:327-337.
23. Wyatt RJ. The complement system in IgA nephropathy and Henoch-Schönlein purpura: functional and genetic aspects. Contrib Nephrology. 1993;104:82-91.
24. Endo M, Ohi H, Ohsawa I, Fujita T, Matsushita M, Fujita T. Glomerular deposition of mannose-binding lectin (MBL) indicates a novel mechanism of complement activation in IgA-nephropathy. Nephrol Dial Transplant. 1998;13:1984-1990.
25. Allen AC, Willis FR, Beattie TJ, Feehally J. Abnormal IgA glycosylation in Schönlein–Henoch purpura restricted to patients with clinical nephritis. Nephrol Dial Transplant. 1998;13:930-934.
26. Vuong MT, Hahn-Zoric M, Lundberg S, et al. Association of soluble CD89 levels with disease progression but not susceptibility in IgA nephropathy. Kidney Int. 2001;78:1281-1287.
27. Van der Helm-van Mil AH, Smith AC, Pouria S, et al. Immunoglobulin A multiple myeloma presenting with Henoch-Schönlein purpura associated with reduced sialylation of IgA1. Br J Haematol. 2003;122(6):915-917.
28. Van Hale HM, Gibson LE, Schroeter AL. Henoch-Schönlein vasculitis: direct immunofluorescence study of uninvolved skin. J Am Acad Dermatol. 1986;15(4 Pt 1):665-670.
29. Faille-Kuyper EH, Kater L, Kuijten RH, et al. Occurrence of vascular IgA deposits in clinically normal skin of patients with renal disease. Kidney Int. 1976;9(5):424-429.
30. Thompson AJ, Chan YL, Woodroffe AJ, et al. Vascular IgA deposits in clinically normal skin of patients with renal disease. Pathology. 1980;12(3):407-413.
31. Hené RJ, Velthuis P, van de Wiel A. et al. The relevance of IgA deposits in vessel walls of clinically normal skin. A prospective study. Arch Intern Med. 1986 Apr;146(4):745-749.
32. Saklayen MG, Schroeter AL, Nafz MA, Jalil K. IgA deposition in the skin of patients with alcoholic liver disease. J Cutan Pathol. 1996;23(1):12-18.
33. [Mukhin NA, Gulyaev SV, Krivosheev ОG, et al. Clinical and prognostic implications of gastrointestinal tract lesions in systemic vascular purpuras. Terapevticheskiy Arkhiv = Therapeutic Archive. 2003;75(2):50-54 (In Russ.)].
34. Bode C, Bode JC. Effect of alcohol consumption on the gut. Best Pract Res Clin Gastroenterol. 2003;17(4):575-592.
35. Shwartzman G. A new phenomenon of local skin reactivity to B. Typhosus culture filtrate. Exptl Biol Med. 1928;25(7):560-561.
36. [Krivosheev OG. Sistemnye sosudistye purpury – kliniko-etiologicheskie varianty [Systemic vascular purpuras: clinical and etiologic types]. Dis. … kand. med. sci. Moscow; 1999 (In Russ.)].
37. Davin JC, Forget P, Mahieu PR. Increased intestinal permeability to (51 Cr) EDTA is correlated with IgA immune complex-plasma levels in children with IgA-associated nephropathies. Acta Paediatr Scand. 1988;77(1):118-124.
38. Davin JC, Mahieu PR. Sequential measurements of intestinal permeability to [51Cr] EDTA in children with Henoch-Schönlein purpura nephritis. Nephron. 1992;60(4):498-499.
ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия
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S.V. Guliaev, L.A. Strizhakov, S.V. Moiseev, V.V. Fomin
I.M. Sechenov First Moscow State Medical University Ministry of Health of Russia (Sechenov University), Moscow, Russia