Легочные васкулиты: диагностика и лечение
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Ключевые слова: легочные васкулиты, эозинофильный гранулематоз с полиангиитом, гранулематоз с полиангиитом, микроскопический полиангиит, синдром Гудпасчера (легочно-почечный), диагностика, лечение, обзор.
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Pulmonary vasculitis is a group of rapidly progressing severe diseases characterized by vascular inflammation, destruction and necrosis of the pulmonary tissue. The pathological process in the lungs varies from diffuse alveolar hemorrhage to inflammation of the parenchyma, pleural effusion, thrombotic and thromboembolic complications. Depending on the size of the affected vessels, vasculites are divided into vasculites of large, medium and small vessels. Most frequently the lung is found in the small vessels vasculitis, including ANCA-associated vasculitis [granulomatosis with polyangiitis (GP), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA)] and the disease is glomerular basement membrane (goodpasture syndrome). Clinical examination of other systems and organs involved in the pathological process, including the skin and kidneys, as well as the detection of autoantibodies can improve approaches to early diagnosis and treatment of vasculitis. Treatment of life-threatening pulmonary bleeding and irreversible damage to organs, especially the kidneys, requires rapid diagnosis of these conditions. Vasculitis is a rare disease with lesions of many organs, and methods of their treatment, including biological, are rapidly developing, which requires the cooperation of doctors of various specialties and specialized centers to achieve better control of the disease.
Keywords: pulmonary vasculitis, eosinophilic granulomatosis with polyangiitis, with polyangiitis granulomatosis, microscopic polyangiitis, goodpasture's syndrome (pulmonary and renal), diagnosis, treatment, review.
2. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65(1): 1-11. https://doi:10.1002/ art.37715
3. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994; 37(2): 187-192.
4. Mandl LA, Solomon DH, Smith EL, Lew RA, Katz JN, Shmerling RH. Using antineutrophil cytoplasmic antibody testing to diagnose vasculitis: can test-ordering guidelines improve diagnostic accuracy? Arch Intern Med. 2002; 162(13): 1509-1514.
5. Frankel SK, Sullivan EJ, Brown KK. Vasculitis: Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa, and Takayasu arteritis. Crit Care Clinics. 2002; 18(4): 855-879.
6. Swerlick RA, Lawley TJ. Cutaneous vasculitis: its relationship to systemic disease. Med Clin North Am. 1989; 73(5): 1221-1235.
7. Guillevin L, Lhote F, Gherardi R. Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: clinical aspects, neurologic manifestations, and treatment. Neurol Clin. 1997; 15(4): 865-886.
8. Davies DJ, Moran JE, Niall JF, Ryan GB. Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? Br Med J (Clin Res Ed). 1982; 285(6342): 606.
9. Choi HK, Liu S, Merkel PA, Colditz GA, Niles JL. Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on myeloperoxidase antibodies.
J Rheumatol. 2001; 28(7): 1584-1590.
10. Savige J, Gillis D, Benson E, Davies D, Esnault V, Falk RJ, Hagen EC, Jayne D, Jennette JC, Paspaliaris B, Pollock W, Pusey C, Savage CO, Silvestrini R, van der Woude F, Wieslander J, Wiik A. International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA). Am J Clin Pathol. 1999; 111(4): 507-513.
11. Finkielman JD, Lee AS, Hummel AM, Viss MA, Jacob GL, Homburger HA, Peikert T, Hoffman GS, Merkel PA, Spiera R, St Clair EW, Davis JC Jr, McCune WJ, Tibbs AK, Ytterberg SR, Stone JH, Specks U; WGET Research Group. ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis. Am J Med. 2007; 120(7): 643. e9-14. https://doi:10.1016/j.amjmed.2006.08.016
12. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, Hauser T, Hellmich B, Jayne D, Kallenberg CG, Merkel PA, Raspe H, Salvarani C, Scott DG, Stegeman C, Watts R, Westman K, Witter J, Yazici H, Luqmani R; European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009; 68(3): 310-317. https://doi:10. 1136/ard.2008.088096
13. Ananthakrishnan L, Sharma N, Kanne JP. Wegener’s granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol. 2009; 192(3): 676-682. https://doi:10.2214/AJR.08.1837
14. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS. Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992; 116(6): 488-498.
15. Hauer HA, Bajema IM, van Houwelingen HC, Ferrario F, Noël LH, Waldherr R, Jayne DR, Rasmussen N, Bruijn JA, Hagen EC; European Vasculitis Study Group (EUVAS). Renal histology in ANCA-associated vasculitis: differences between diagnostic and serologic subgroups. Kidney Int. 2002; 61(1): 80-89. https://doi:10.1046/j.1523-1755.2002. 00089.
16. Anderson G, Coles ET, Crane M, Douglas AC, Gibbs AR, Geddes DM, Peel ET, Wood JB. Wegener's granuloma. A series of 265 British cases seen between 1975 and 1985. A report by a sub-committee of the British Thoracic Society Research Committee. Q J Med. 1992; 83(302): 427-438.
17. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983; 98(1): 76-85.
18. Cordier JF, Valeyre D, Guillevin L, Loire R, Brechot JM. Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest. 1990; 97: 906-912.
19. Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener’s granulomatosis. Am J Surg Pathol. 1991; 15(4): 315-333.
20. Mark EJ, Flieder DB, Matsubara O. Treated Wegener’s granulomatosis: distinctive pathological findings in the lung of 20 patients and what they tell us about the natural history of the disease. Hum Pathol. 1997; 28: 450-458.
21. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008; 26(Suppl 51): S94-104.
22. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine. 1999; 78(1): 26-37.
23. Lanham J, Elkon K, Pusey C, Hughes G. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine. 1984; 63: 65-81.
24. Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, Maurier F, Jouneau S, Bienvenu B, Puéchal X, Aumaître O, Le Guenno G, Le Quellec A, Cevallos R, Fain O, Godeau B, Seror R, Dunogué B, Mahr A, Guilpain P, Cohen P, Aouba A, Mouthon L, Guillevin L; French Vasculitis Study Group. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term follow up of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013; 65(1): 270-281. https://doi:10.1002/art.37721
25. Katzenstein AL. Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review. Am J Clin Pathol. 2000; 114(5): 767-772. https://doi:10.1309/F3FW-J8EB-X913-G1RJ
26. Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003; 115(4): 284-290.
27. Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P, Amouroux J, Casassus P, Jarrousse B. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999; 42(3): 421-430. https://doi:10.1002/1529-0131(199904)42:3<421::AID-ANR5>3.0.CO;2-6
28. Lhote F, Cohen P, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis and Churg-Strauss syndrome. Lupus. 1998; 7(4): 238-258. https://doi:10.1191/096120398678920055
29. Frankel SK, Cosgrove GP, Brown KK. Small vessel vasculitis of the lung. Chron Respir Dis. 2005; 2(2): 75-84. https://doi:10.1191/ 1479972305cd070rs
30. Langford CA, Talar-Williams C, Sneller MC. Use of methotrexate and glucocorticoids in the treatment of Wegener’s granulomatosis. Long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum. 2000; 43(8): 1836-1840. https://doi:10.1002/1529-0131 (200008)43:8<1836::AID-ANR20>3.0.CO;2-R
31. Harper L, Morgan MD, Walsh M, Hoglund P, Westman K, Flossmann O, Tesar V, Vanhille P, de Groot K, Luqmani R, Flores-Suarez LF, Watts R, Pusey C, Bruchfeld A, Rasmussen N, Blockmans D, Savage CO, Jayne D; EUVAS investigators. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2012; 71(6): 955-960. https://doi:10.1136/annrheumdis-2011-200477
32. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Turkiewicz A, Tchao NK, Webber L, Ding L, Sejismundo LP, Mieras K, Weitzenkamp D, Ikle D, Seyfert-Margolis V, Mueller M, Brunetta P, Allen NB, Fervenza FC, Geetha D, Keogh KA, Kissin EY, Monach PA, Peikert T, Stegeman C, Ytterberg SR, Specks U; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010; 363(3): 221-232. https://doi:10.1056/NEJMoa0909905
33. Schmitt WH, Hagen EC, Neumann I, Nowack R, Flores-Suárez LF, van der Woude FJ; European Vasculitis Study Group. Treatment of refractory Wegeners granulomatosis with antithymocyte globulin (ATG): an open study in 15 patients. Kidney Int. 2004; 65(4): 1440-1448. https://doi:10.1111/j.1523-1755.2004.00534.x
34. Haubitz M, de Groot K. Tolerance of mycophenolate mofetil in end-stage renal disease patients with ANCA-associated vasculitis. Clin Nephrol. 2002; 57(6): 421-424.
35. Smith RM, Jones RB, Guerry MJ, Laurino S, Catapano F, Chaudhry A, Smith KG, Jayne DR. Rituximab for remission maintenance in relapsing antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2012; 64(11): 3760-3769. https://doi:10.1002/art.34583
36. Cohen Tervaert JW, Stegeman CA, Kallenberg CG. Novel therapies for anti-neutrophil cytoplasmic antibody-associated vasculitis. Curr Opin Nephrol Hypertens. 2001; 10(2): 211-217.
37. Langford CA, Sneller MC. Biologic therapies in the vasculitides. Curr Opin Rheumatol. 2003; 15: 3-10.
38. Faurschou M, Westman K, Rasmussen N, de Groot K, Flossmann O, Höglund P, Jayne DR; European Vasculitis Study Group. Brief report: long-term outcome of a randomized clinical trial comparing methotrexate to cyclophosphamide for remission induction in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2012; 64(10): 3472-3477. https://doi:10.1002/art. 34547
39. Seo P, Specks U, Keogh KA. Efficacy of rituximab in limited Wegener’s granulomatosis with refractory granulomatous manifestations.
J Rheumatol. 2008; 35(10): 2017-2023.
40. Guillevin L, Lhote F, Cohen P, Jarrousse B, Lortholary O, Généreau T, Léon A, Bussel A. Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients. Arthritis Rheum. 1995; 38(11): 1638-1645.
41. Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis. 2003; 42(6): 1149-1153.
42. Henke D, Falk RJ, Gabriel DA. Successful treatment of diffuse alveolar hemorrhage with activated factor VII. Ann Intern Med. 2004; 140(6): 493-494.
43. Ahmed SH, Aziz T, Cochran J, Highland K. Use of extracorporeal membrane oxygenation in a patient with diffuse alveolar hemorrhage. Chest. 2004; 126(1): 305-309. https://doi:10.1378/chest.126.1.305
44. Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix JP, Kyndt X, Lifermann F, Papo T, Lambert M, Le Noach J, Khellaf M, Merrien D, Puéchal X, Vinzio S, Cohen P, Mouthon L, Cordier JF, Guillevin L; French Vasculitis Study Group. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008; 359(26): 2790-2803. https://doi:10.1056/NEJMoa0802311
45. Reinhold-Keller E, Fink CO, Herlyn K, Gross WL, De Groot K. High rate of renal relapse in 71 patients with Wegener’s granulomatosis under maintenance remission with low-dose methotrexate. Arthritis Rheum. 2002; 47(3): 326-332. https://doi:10.1002/art.10459
46. Metzler C, Fink C, Lamprecht P, Gross W, Reinhold-Keller E. Maintenance of remission with leflunomide in Wegener’s granulomatosis. Rheumatology (Oxford). 2004; 43(3): 315-320. https://doi.org/ 10.1093/rheumatology/keh009
47. Molloy ES, Langford CA. Does the dose of methotrexate influence the rate of relapse in patients with Wegener’s granulomatosis? Nat Clin Pract Rheumatol. 2008; 4(1): 12-13. https://doi:10.1038/ncprheum0675
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1. Flossmann O, Berden A, de Groot K, Hagen C, Harper L, Heijl C, Höglund P, Jayne D, Luqmani R, Mahr A, Mukhtyar C, Pusey C, Rasmussen N, Stegeman C, Walsh M, Westman K. European Vasculitis Study Group. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011; 70(3): 488-494. https://doi:10.1136/ ard.2010.137778
2. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65(1): 1-11. https://doi:10.1002/ art.37715
3. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994; 37(2): 187-192.
4. Mandl LA, Solomon DH, Smith EL, Lew RA, Katz JN, Shmerling RH. Using antineutrophil cytoplasmic antibody testing to diagnose vasculitis: can test-ordering guidelines improve diagnostic accuracy? Arch Intern Med. 2002; 162(13): 1509-1514.
5. Frankel SK, Sullivan EJ, Brown KK. Vasculitis: Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa, and Takayasu arteritis. Crit Care Clinics. 2002; 18(4): 855-879.
6. Swerlick RA, Lawley TJ. Cutaneous vasculitis: its relationship to systemic disease. Med Clin North Am. 1989; 73(5): 1221-1235.
7. Guillevin L, Lhote F, Gherardi R. Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: clinical aspects, neurologic manifestations, and treatment. Neurol Clin. 1997; 15(4): 865-886.
8. Davies DJ, Moran JE, Niall JF, Ryan GB. Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? Br Med J (Clin Res Ed). 1982; 285(6342): 606.
9. Choi HK, Liu S, Merkel PA, Colditz GA, Niles JL. Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on myeloperoxidase antibodies.
J Rheumatol. 2001; 28(7): 1584-1590.
10. Savige J, Gillis D, Benson E, Davies D, Esnault V, Falk RJ, Hagen EC, Jayne D, Jennette JC, Paspaliaris B, Pollock W, Pusey C, Savage CO, Silvestrini R, van der Woude F, Wieslander J, Wiik A. International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA). Am J Clin Pathol. 1999; 111(4): 507-513.
11. Finkielman JD, Lee AS, Hummel AM, Viss MA, Jacob GL, Homburger HA, Peikert T, Hoffman GS, Merkel PA, Spiera R, St Clair EW, Davis JC Jr, McCune WJ, Tibbs AK, Ytterberg SR, Stone JH, Specks U; WGET Research Group. ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis. Am J Med. 2007; 120(7): 643. e9-14. https://doi:10.1016/j.amjmed.2006.08.016
12. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, Hauser T, Hellmich B, Jayne D, Kallenberg CG, Merkel PA, Raspe H, Salvarani C, Scott DG, Stegeman C, Watts R, Westman K, Witter J, Yazici H, Luqmani R; European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009; 68(3): 310-317. https://doi:10. 1136/ard.2008.088096
13. Ananthakrishnan L, Sharma N, Kanne JP. Wegener’s granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol. 2009; 192(3): 676-682. https://doi:10.2214/AJR.08.1837
14. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS. Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992; 116(6): 488-498.
15. Hauer HA, Bajema IM, van Houwelingen HC, Ferrario F, Noël LH, Waldherr R, Jayne DR, Rasmussen N, Bruijn JA, Hagen EC; European Vasculitis Study Group (EUVAS). Renal histology in ANCA-associated vasculitis: differences between diagnostic and serologic subgroups. Kidney Int. 2002; 61(1): 80-89. https://doi:10.1046/j.1523-1755.2002. 00089.
16. Anderson G, Coles ET, Crane M, Douglas AC, Gibbs AR, Geddes DM, Peel ET, Wood JB. Wegener's granuloma. A series of 265 British cases seen between 1975 and 1985. A report by a sub-committee of the British Thoracic Society Research Committee. Q J Med. 1992; 83(302): 427-438.
17. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983; 98(1): 76-85.
18. Cordier JF, Valeyre D, Guillevin L, Loire R, Brechot JM. Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest. 1990; 97: 906-912.
19. Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener’s granulomatosis. Am J Surg Pathol. 1991; 15(4): 315-333.
20. Mark EJ, Flieder DB, Matsubara O. Treated Wegener’s granulomatosis: distinctive pathological findings in the lung of 20 patients and what they tell us about the natural history of the disease. Hum Pathol. 1997; 28: 450-458.
21. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008; 26(Suppl 51): S94-104.
22. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine. 1999; 78(1): 26-37.
23. Lanham J, Elkon K, Pusey C, Hughes G. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine. 1984; 63: 65-81.
24. Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, Maurier F, Jouneau S, Bienvenu B, Puéchal X, Aumaître O, Le Guenno G, Le Quellec A, Cevallos R, Fain O, Godeau B, Seror R, Dunogué B, Mahr A, Guilpain P, Cohen P, Aouba A, Mouthon L, Guillevin L; French Vasculitis Study Group. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term follow up of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013; 65(1): 270-281. https://doi:10.1002/art.37721
25. Katzenstein AL. Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review. Am J Clin Pathol. 2000; 114(5): 767-772. https://doi:10.1309/F3FW-J8EB-X913-G1RJ
26. Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003; 115(4): 284-290.
27. Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P, Amouroux J, Casassus P, Jarrousse B. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999; 42(3): 421-430. https://doi:10.1002/1529-0131(199904)42:3<421::AID-ANR5>3.0.CO;2-6
28. Lhote F, Cohen P, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis and Churg-Strauss syndrome. Lupus. 1998; 7(4): 238-258. https://doi:10.1191/096120398678920055
29. Frankel SK, Cosgrove GP, Brown KK. Small vessel vasculitis of the lung. Chron Respir Dis. 2005; 2(2): 75-84. https://doi:10.1191/ 1479972305cd070rs
30. Langford CA, Talar-Williams C, Sneller MC. Use of methotrexate and glucocorticoids in the treatment of Wegener’s granulomatosis. Long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum. 2000; 43(8): 1836-1840. https://doi:10.1002/1529-0131 (200008)43:8<1836::AID-ANR20>3.0.CO;2-R
31. Harper L, Morgan MD, Walsh M, Hoglund P, Westman K, Flossmann O, Tesar V, Vanhille P, de Groot K, Luqmani R, Flores-Suarez LF, Watts R, Pusey C, Bruchfeld A, Rasmussen N, Blockmans D, Savage CO, Jayne D; EUVAS investigators. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2012; 71(6): 955-960. https://doi:10.1136/annrheumdis-2011-200477
32. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Turkiewicz A, Tchao NK, Webber L, Ding L, Sejismundo LP, Mieras K, Weitzenkamp D, Ikle D, Seyfert-Margolis V, Mueller M, Brunetta P, Allen NB, Fervenza FC, Geetha D, Keogh KA, Kissin EY, Monach PA, Peikert T, Stegeman C, Ytterberg SR, Specks U; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010; 363(3): 221-232. https://doi:10.1056/NEJMoa0909905
33. Schmitt WH, Hagen EC, Neumann I, Nowack R, Flores-Suárez LF, van der Woude FJ; European Vasculitis Study Group. Treatment of refractory Wegeners granulomatosis with antithymocyte globulin (ATG): an open study in 15 patients. Kidney Int. 2004; 65(4): 1440-1448. https://doi:10.1111/j.1523-1755.2004.00534.x
34. Haubitz M, de Groot K. Tolerance of mycophenolate mofetil in end-stage renal disease patients with ANCA-associated vasculitis. Clin Nephrol. 2002; 57(6): 421-424.
35. Smith RM, Jones RB, Guerry MJ, Laurino S, Catapano F, Chaudhry A, Smith KG, Jayne DR. Rituximab for remission maintenance in relapsing antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2012; 64(11): 3760-3769. https://doi:10.1002/art.34583
36. Cohen Tervaert JW, Stegeman CA, Kallenberg CG. Novel therapies for anti-neutrophil cytoplasmic antibody-associated vasculitis. Curr Opin Nephrol Hypertens. 2001; 10(2): 211-217.
37. Langford CA, Sneller MC. Biologic therapies in the vasculitides. Curr Opin Rheumatol. 2003; 15: 3-10.
38. Faurschou M, Westman K, Rasmussen N, de Groot K, Flossmann O, Höglund P, Jayne DR; European Vasculitis Study Group. Brief report: long-term outcome of a randomized clinical trial comparing methotrexate to cyclophosphamide for remission induction in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2012; 64(10): 3472-3477. https://doi:10.1002/art. 34547
39. Seo P, Specks U, Keogh KA. Efficacy of rituximab in limited Wegener’s granulomatosis with refractory granulomatous manifestations.
J Rheumatol. 2008; 35(10): 2017-2023.
40. Guillevin L, Lhote F, Cohen P, Jarrousse B, Lortholary O, Généreau T, Léon A, Bussel A. Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients. Arthritis Rheum. 1995; 38(11): 1638-1645.
41. Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis. 2003; 42(6): 1149-1153.
42. Henke D, Falk RJ, Gabriel DA. Successful treatment of diffuse alveolar hemorrhage with activated factor VII. Ann Intern Med. 2004; 140(6): 493-494.
43. Ahmed SH, Aziz T, Cochran J, Highland K. Use of extracorporeal membrane oxygenation in a patient with diffuse alveolar hemorrhage. Chest. 2004; 126(1): 305-309. https://doi:10.1378/chest.126.1.305
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ФГБОУ ВО «РНИМУ имени Н.И. Пирогова» Минздрава России, Москва, Россия
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E.Kh. ANAEV, I.A. BARANOVA, A.S. BELEVSKY
Pirogov Russian National Research Medical University, Moscow, Russia