Атипичное течение болезни Гудпасчера: клиническое наблюдение и обзор литературы

Читать PDF

Аннотация

Болезнь Гудпасчера (син. анти-БМК-болезнь) – редкое аутоиммунное заболевание из группы васкулитов сосудов малого диаметра, характеризующееся наличием антител к базальной мембране клубочка и альвеол (анти-БМК) в циркуляции. Основным проявлением, по традиционным представлениям, является тяжелое сочетанное поражение почек и легких (почечно-легочный синдром).
В статье представлено клиническое наблюдение пациента с атипичным течением заболевания, основным проявлением которого стало фульминантное развитие геморрагического альвеолита. При этом поражение почек протекало с развитием мочевого синдрома и не сопровождалось формированием почечной недостаточности. Лечение плазмаферезом в сочетании с иммуносупрессивной терапией циклофосфамидом и глюкокортикостероидами позволило добиться ремиссии заболевания. В работе представлен обзор современных данных о патогенезе и течении заболевания, которые отражают смену сложившейся парадигмы представлений об анти-БМК-болезни как «заболевании одного удара», неизменно сопровождающемся развитием быстропрогрессирующего гломерулонефрита.

Ключевые слова: болезнь Гудпасчера, анти-БМК-болезнь, антитела к базальной мембране клубочка, геморрагический альвеолит, плазмаферез.

________________________________________________

Goodpasture’s disease (anti-GBM disease) is a rare small vessels vasculitis characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM) and alveolar basement membrane. Common feature of anti-GBM disease is a combination of rapidly progressive glomerulonephritis and alveolar hemorrhage (pulmonary-renal syndrome). We present a case of atypical disease course in a young male patient who developed alveolar hemorrhage without renal failure. The only symptom of renal involvement was isolated hematuria. Plasmapheresis combined with immunosuppression (cyclophosphamide and corticosteroids) was effective. We present a review of state-of-art data on the pathogenesis and disease course of anti-GBM disease.

Keywords: Goodpasture’s disease, anti-GBM disease, anti-glomerular basement membrane antibodies, pulmonary hemorrhage, plasmapheresis.

Список литературы

1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. doi: 10.1002/art.37715
2. Goodpasture E. The significance of certain pulmonary lesions in relation to the etiology of influenza. Am J Med Sci. 1919;(158):863-70.
3. Stanton MC, Tange JD. Goodpasture’s syndrome (pulmonary haemorrhage associated with glomerulonephritis). Austral Ann Med. 1958;7(2):132-44.
4. Wilson CB, Dixon FJ. Anti-glomerular basement membrane antibody-induced glomerulonephritis. Kidney Int. 1973;3(2):74-89.
5. Hellmark T, Segelmark M. Diagnosis and classification of Goodpasture’s disease (anti-GBM). J Autoimmun. 2014;48-49:108-12.
doi: 10.1016/j.jaut.2014.01.024
6. Мухин Н.А. Синдром Гудпасчера: патогенез, диагностика, лечение. Фарматека. 2011;18:8-14 [Mukhin NA. Goodpasture’s syndrome: pathogenesis, diagnosis, treatment. Farmateka = Pharmateka. 2011;18:8-14 (In Russ.)].
7. Canney M, O’Hara PV, McEvoy CM, et al. Spatial and Temporal Clustering of Anti-Glomerular Basement Membrane Disease. Clin J Am Soc Nephrol. 2016;11(8):1392-9. doi: 10.2215/CJN.13591215
8. 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):1162-7. doi: 10.1053/ajkd.2002.33385
9. Pedchenko V, Bondar O, Fogo AB, et al. Molecular Architecture of the Goodpasture Autoantigen in Anti-GBM Nephritis. New Engl J Med. 2010;363(4):343-54. doi: 10.1056/NEJMoa0910500
10. Lerner RA, Glassock RJ, Dixon FJ. The role of anti-glomerular basement membrane antibody in the pathogenesis of human glomerulonephritis. J Exper Med. 1967;126(6):989-1004.
11. Zhao J, Yan Y, Cui Z, Yang R, Zhao M-H. The immunoglobulin G subclass distribution of anti-GBM autoantibodies against rHalpha3(IV)NC1 is associated with disease severity. Hum Immunol. 2009;70(6):425-9. doi: 10.1016/j.humimm.2009.04.004
12. Wilson CB, Smith RC. Goodpasture’s syndrome associated with influenza A2 virus infection. Ann Intern Med. 1972;76(1):91-4.
13. Donaghy M, Rees AJ. Cigarette smoking and lung haemorrhage in glomerulonephritis caused by autoantibodies to glomerular basement membrane. Lancet (London, England). 1983;2(8364):
1390-3.
14. Bombassei GJ, Kaplan AA. The association between hydrocarbon exposure and anti-glomerular basement membrane antibody-mediated disease (Goodpasture’s syndrome). Am J Industr Med. 1992; 21(2):141-53.
15. Clatworthy MR, Wallin EF, Jayne DR. Anti-Glomerular Basement Membrane Disease after Alemtuzumab. New Engl J Med. 2008;359(7):768-9. doi: 10.1056/NEJMc0800484
16. Westman KW, Ericsson UB, Höier-Madsen M, et al. Prevalence of autoantibodies associated with glomerulonephritis, unaffected after extracorporeal shock wave lithotripsy for renal calculi, in a three-year follow-up. Scand J Urol. 1997;31(5):463-7.
17. McAdoo SP, Tanna A, Hrušková 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. doi: 10.1016/j.kint. 2017.03.014
18. Lazor R, Bigay-Gamé L, Cottin V, et al. Alveolar hemorrhage in anti-basement membrane antibody disease: a series of 28 cases. Medicine (Baltimore). 2007;86(3):181-93. doi: 10.1097/md.0b013e318067da56
19. 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):935-9.
20. Berends-De Vries T, Boerma S, Doornabal J, Dikkeschei B, Stegeman C, Veneman T. Goodpasture’s Syndrome with Negative Anti-glomerular Basement Membrane Antibodies. Eur J Case Rep Intern Med. 2017;4(8). doi: 10.12890/2017_000687
21. Hind CR, Bowman C, Winearls CG, Lockwood CM. Recurrence of circulating anti-glomerular basement membrane antibody three years after immunosuppressive treatment and plasma exchange. Clin Nephrol. 1984;21(4):244-6.
22. Liu P, Waheed S, Boujelbane L, Maursetter LJ. Multiple recurrences of anti-glomerular basement membrane disease with variable antibody detection: can the laboratory be trusted? Clin Kidney J. 2016;9(5):657-60. doi: 10.1093/ckj/sfw038
23. Gu B, Magil AB, Barbour SJ. Frequently relapsing anti-glomerular basement membrane antibody disease with changing clinical phenotype and antibody characteristics over time. Clin Kidney J. 2016;9(5):661-4. doi: 10.1093/ckj/sfw048
24. Keller F, Nekarda H. Fatal relapse in Goodpasture’s syndrome 3 years after plasma exchange. Respiration. 1985;48(1):62-6.
25. Segelmark M, Dahlberg P, Wieslander J. Anti-GBM disease with a mild relapsing course and low levels of anti-GBM autoantibodies. Clin Kidney J. 2012;5(6):549-51. doi: 10.1093/ckj/sfs098
26. Glassock RJ. Atypical anti-glomerular basement membrane disease: lessons learned. Clin Kidney J. 2016;9(5):653-6. doi: 10.1093 /ckj/sfw068
27. Troxell ML, Houghton DC. Atypical anti-glomerular basement membrane disease. Clin Kidney J. 2016;9(2):211-21. doi: 10.1093/ ckj/sfv140
28. McAdoo SP, Pusey CD. Clustering of Anti-GBM Disease: Clues to an Environmental Trigger? Clin J Am Soc Nephrol. 2016;11(8):1324-6. doi: 10.2215/CJN.05580516
29. Huart A, Josse A-G, Chauveau D, et al. Outcomes of patients with Goodpasture syndrome: A nationwide cohort-based study from the French Society of Hemapheresis. J Autoimmun. 2016;73:24-9.
doi: 10.1016/j.jaut.2016.05.015
30. De Groot K, Harper L, Jayne DRW, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009;150(10):670-80.
31. Alchi B, Griffiths M, Sivalingam M, Jayne D, Farrington K. Predictors of renal and patient outcomes in anti-GBM disease: clinicopathologic analysis of a two-centre cohort. Nephrol Dialys Transplant. 2015;30(5):814-21. doi: 10.1093/ndt/gfu399
32. Hirayama K, Yamagata K, Kobayashi M, Koyama A. Anti-glomerular basement membrane antibody disease in Japan: part of the nationwide rapidly progressive glomerulonephritis survey in Japan. Clin Exper Nephrol. 2008;12(5):339-47. doi: 10.1007/s10157-008-0051-8
33. McAdoo SP, Pusey CD. Anti-Glomerular Basement Membrane Disease. Clin J Amer Soc Nephrol. 2017;12(7):1162-72.
doi: 10.2215/CJN.01380217
34. Syeda UA, Singer NG, Magrey M. Anti-glomerular basement membrane antibody disease treated with rituximab: A case-based review. Semin Arthritis Rheum. 2013;42(6):567-72. doi: 10.1016/j.semarthrit.2012.10.007
35. Little MA, Pusey CD. Rapidly progressive glomerulonephritis: current and evolving treatment strategies. J Nephrol. 2004;17 Suppl 8:S10-9.

________________________________________________

1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. doi: 10.1002/art.37715
2. Goodpasture E. The significance of certain pulmonary lesions in relation to the etiology of influenza. Am J Med Sci. 1919;(158):863-70.
3. Stanton MC, Tange JD. Goodpasture’s syndrome (pulmonary haemorrhage associated with glomerulonephritis). Austral Ann Med. 1958;7(2):132-44.
4. Wilson CB, Dixon FJ. Anti-glomerular basement membrane antibody-induced glomerulonephritis. Kidney Int. 1973;3(2):74-89.
5. Hellmark T, Segelmark M. Diagnosis and classification of Goodpasture’s disease (anti-GBM). J Autoimmun. 2014;48-49:108-12.
doi: 10.1016/j.jaut.2014.01.024
6. [Mukhin NA. Goodpasture’s syndrome: pathogenesis, diagnosis, treatment. Farmateka = Pharmateka. 2011;18:8-14 (In Russ.)].
7. Canney M, O’Hara PV, McEvoy CM, et al. Spatial and Temporal Clustering of Anti-Glomerular Basement Membrane Disease. Clin J Am Soc Nephrol. 2016;11(8):1392-9. doi: 10.2215/CJN.13591215
8. 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):1162-7. doi: 10.1053/ajkd.2002.33385
9. Pedchenko V, Bondar O, Fogo AB, et al. Molecular Architecture of the Goodpasture Autoantigen in Anti-GBM Nephritis. New Engl J Med. 2010;363(4):343-54. doi: 10.1056/NEJMoa0910500
10. Lerner RA, Glassock RJ, Dixon FJ. The role of anti-glomerular basement membrane antibody in the pathogenesis of human glomerulonephritis. J Exper Med. 1967;126(6):989-1004.
11. Zhao J, Yan Y, Cui Z, Yang R, Zhao M-H. The immunoglobulin G subclass distribution of anti-GBM autoantibodies against rHalpha3(IV)NC1 is associated with disease severity. Hum Immunol. 2009;70(6):425-9. doi: 10.1016/j.humimm.2009.04.004
12. Wilson CB, Smith RC. Goodpasture’s syndrome associated with influenza A2 virus infection. Ann Intern Med. 1972;76(1):91-4.
13. Donaghy M, Rees AJ. Cigarette smoking and lung haemorrhage in glomerulonephritis caused by autoantibodies to glomerular basement membrane. Lancet (London, England). 1983;2(8364):
1390-3.
14. Bombassei GJ, Kaplan AA. The association between hydrocarbon exposure and anti-glomerular basement membrane antibody-mediated disease (Goodpasture’s syndrome). Am J Industr Med. 1992; 21(2):141-53.
15. Clatworthy MR, Wallin EF, Jayne DR. Anti-Glomerular Basement Membrane Disease after Alemtuzumab. New Engl J Med. 2008;359(7):768-9. doi: 10.1056/NEJMc0800484
16. Westman KW, Ericsson UB, Höier-Madsen M, et al. Prevalence of autoantibodies associated with glomerulonephritis, unaffected after extracorporeal shock wave lithotripsy for renal calculi, in a three-year follow-up. Scand J Urol. 1997;31(5):463-7.
17. McAdoo SP, Tanna A, Hrušková 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. doi: 10.1016/j.kint. 2017.03.014
18. Lazor R, Bigay-Gamé L, Cottin V, et al. Alveolar hemorrhage in anti-basement membrane antibody disease: a series of 28 cases. Medicine (Baltimore). 2007;86(3):181-93. doi: 10.1097/md.0b013e318067da56
19. 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):935-9.
20. Berends-De Vries T, Boerma S, Doornabal J, Dikkeschei B, Stegeman C, Veneman T. Goodpasture’s Syndrome with Negative Anti-glomerular Basement Membrane Antibodies. Eur J Case Rep Intern Med. 2017;4(8). doi: 10.12890/2017_000687
21. Hind CR, Bowman C, Winearls CG, Lockwood CM. Recurrence of circulating anti-glomerular basement membrane antibody three years after immunosuppressive treatment and plasma exchange. Clin Nephrol. 1984;21(4):244-6.
22. Liu P, Waheed S, Boujelbane L, Maursetter LJ. Multiple recurrences of anti-glomerular basement membrane disease with variable antibody detection: can the laboratory be trusted? Clin Kidney J. 2016;9(5):657-60. doi: 10.1093/ckj/sfw038
23. Gu B, Magil AB, Barbour SJ. Frequently relapsing anti-glomerular basement membrane antibody disease with changing clinical phenotype and antibody characteristics over time. Clin Kidney J. 2016;9(5):661-4. doi: 10.1093/ckj/sfw048
24. Keller F, Nekarda H. Fatal relapse in Goodpasture’s syndrome 3 years after plasma exchange. Respiration. 1985;48(1):62-6.
25. Segelmark M, Dahlberg P, Wieslander J. Anti-GBM disease with a mild relapsing course and low levels of anti-GBM autoantibodies. Clin Kidney J. 2012;5(6):549-51. doi: 10.1093/ckj/sfs098
26. Glassock RJ. Atypical anti-glomerular basement membrane disease: lessons learned. Clin Kidney J. 2016;9(5):653-6. doi: 10.1093 /ckj/sfw068
27. Troxell ML, Houghton DC. Atypical anti-glomerular basement membrane disease. Clin Kidney J. 2016;9(2):211-21. doi: 10.1093/ ckj/sfv140
28. McAdoo SP, Pusey CD. Clustering of Anti-GBM Disease: Clues to an Environmental Trigger? Clin J Am Soc Nephrol. 2016;11(8):1324-6. doi: 10.2215/CJN.05580516
29. Huart A, Josse A-G, Chauveau D, et al. Outcomes of patients with Goodpasture syndrome: A nationwide cohort-based study from the French Society of Hemapheresis. J Autoimmun. 2016;73:24-9.
doi: 10.1016/j.jaut.2016.05.015
30. De Groot K, Harper L, Jayne DRW, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009;150(10):670-80.
31. Alchi B, Griffiths M, Sivalingam M, Jayne D, Farrington K. Predictors of renal and patient outcomes in anti-GBM disease: clinicopathologic analysis of a two-centre cohort. Nephrol Dialys Transplant. 2015;30(5):814-21. doi: 10.1093/ndt/gfu399
32. Hirayama K, Yamagata K, Kobayashi M, Koyama A. Anti-glomerular basement membrane antibody disease in Japan: part of the nationwide rapidly progressive glomerulonephritis survey in Japan. Clin Exper Nephrol. 2008;12(5):339-47. doi: 10.1007/s10157-008-0051-8
33. McAdoo SP, Pusey CD. Anti-Glomerular Basement Membrane Disease. Clin J Amer Soc Nephrol. 2017;12(7):1162-72.
doi: 10.2215/CJN.01380217
34. Syeda UA, Singer NG, Magrey M. Anti-glomerular basement membrane antibody disease treated with rituximab: A case-based review. Semin Arthritis Rheum. 2013;42(6):567-72. doi: 10.1016/j.semarthrit.2012.10.007
35. Little MA, Pusey CD. Rapidly progressive glomerulonephritis: current and evolving treatment strategies. J Nephrol. 2004;17 Suppl 8:S10-9.

Авторы

М.Л. Буланова 1, Д.В. Потапов 1, Н.М. Буланов 2, Л.В. Лысенко (Козловская) 2

1 ГБУЗ Владимирской области «Областная клиническая больница», Владимир, Россия;
2 ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия

________________________________________________

M.L. Bulanova 1, D.V. Potapov 1, N.M. Bulanov 2, L.V. Lysenko (Kozlovskaya) 2

1 Vladimir Regional Clinical Hospital, Vladimir, Russia;
2 I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia

Назад к списку

Цель портала OmniDoctor – предоставление профессиональной информации врачам, провизорам и фармацевтам.