Современные подходы к диагностике и лечению легочных васкулитов
Современные подходы к диагностике и лечению легочных васкулитов
Анаев Э.Х. Современные подходы к диагностике и лечению легочных васкулитов. Болезни органов дыхания (Прил. к журн. Consilium Medicum). 2018; c. 53–58. DOI: 10.26442/2619-0079.2018.53-58
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Anaev E.Kh. Modern approaches in the diagnosis and management of pulmonary vasculitis. Respiratory Organs Diseases (Suppl. Consilium Medicum). 2018; p. 53–58. DOI: 10.26442/2619-0079.2018.53-58
Современные подходы к диагностике и лечению легочных васкулитов
Анаев Э.Х. Современные подходы к диагностике и лечению легочных васкулитов. Болезни органов дыхания (Прил. к журн. Consilium Medicum). 2018; c. 53–58. DOI: 10.26442/2619-0079.2018.53-58
________________________________________________
Anaev E.Kh. Modern approaches in the diagnosis and management of pulmonary vasculitis. Respiratory Organs Diseases (Suppl. Consilium Medicum). 2018; p. 53–58. DOI: 10.26442/2619-0079.2018.53-58
Легочные васкулиты включают в себя разные самостоятельные заболевания, характеризующиеся воспалением сосудов легких, деструкцией и некрозом легочной ткани. В зависимости от размера пораженных сосудов легочные васкулиты подразделяются на васкулиты крупных, средних и мелких сосудов. Наиболее часто поражение легких встречается при васкулитах мелких сосудов, включая ANCA-ассоциированные васкулиты (гранулематоз с полиангиитом, эозинофильный гранулематоз с полиангиитом и микроскопический полиангиит) и легочно-почечный синдром Гудпасчера. Клиническое обследование других систем и органов, вовлеченных в патологический процесс, в том числе кожи и почек, а также выявление аутоантител, позволяет улучшить подходы к ранней диагностике васкулитов. Лечение жизнеугрожающих легочных кровотечений и необратимого повреждения органов, прежде всего почек, требует быстрой диагностики этих состояний. Методы лечения васкулитов, включая биологические, быстро развиваются. Для достижения лучшего контроля заболевания требуется сотрудничество врачей разных специальностей.
Ключевые слова: легочные васкулиты, гранулематоз с полиангиитом, эозинофильный гранулематоз с полиангиитом, микроскопический полиангиит, антинейтрофильные цитоплазматические антитела, ANCA-васкулиты, диагностика, лечение.
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Pulmonary vasculitis includes a number of distinct disorders that are pathologically characterized by inflammation and destruction of pulmonary vasculature with subsequent lung tissue necrosis. Depending on the size of vessels affected pulmonary vasculitis can be classified into: large-size-vessel, medium-size- vessel and small-size-vessel vasculitis. The lungs are most commonly affected by small vessel vasculitis, including ANCA-associated vasculitis (GPA, EGPA and MPA) and pulmonary-renal Goodpasture syndrome. Clinical examination of other organs and systems involved in a pathological process, including the skin and kidneys, as well as a detection of autoantibodies allow to improve approaches in early diagnosis of vasculitis. Management of life-threatening lung bleeding and irreversible damage of body organs, particularly the kidney, requires an immediate diagnosis of these conditions. Methods of vasculitis management including biological ones develop rapidly. To achieve better disease control cooperation of doctors of different specialties is need.
1. Flossmann O, Berden A, de Groot K et al.; European Vasculitis Study Group. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 2011; 70 (3): 488–94.
2. Yunt ZX, Frankel SK, Brown KK. Diagnosis and management of pulmonary vasculitis. Ther Adv Respir Dis 2012; 6 (6): 375–90.
3. 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.
4. Mandl LA, Solomon DH, Smith EL et al. Using antineutrophil cytoplasmic antibody testing to diagnose vasculitis: can test-ordering guidelines improve diagnostic accuracy? Arch Intern Med 2002; 162 (13): 1509–14.
5. Frankel SK, Sullivan EJ, Brown KK. Vasculitis: Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa, and Takayasu arteritis. Crit Care Clin 2002; 18 (4): 855–79.
6. Swerlick RA, Lawley TJ. Cutaneous vasculitis: its relationship to systemic disease. Med Clin North Am 1989; 73 (5): 1221–35.
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): 865–86.
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 et al. Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on myeloperoxidase antibodies. J Rheumatol 2001; 28 (7): 1584–90.
10. Savige J, Gillis D, Benson E et al. International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA). Am J Clin Pathol 1999; 111 (4): 507–13.
11. Finkielman JD, Lee AS, Hummel AM et al.; 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.
12. Mukhtyar C, Guillevin L, Cid MC et al.; European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009; 68 (3): 310–17.
13. Cordier JF, Valeyre D, Guillevin L et al. Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest 1990; 97: 906–12.
14. Guillevin L, Cohen P, Gayraud M et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine 1999; 78 (1): 26–37.
15. Ananthakrishnan L, Sharma N, Kanne JP. Wegener’s granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol 2009; 192 (3): 676–82.
16. Hoffman GS, Kerr GS, Leavitt RY et al. Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116 (6): 488–98.
17. Anderson G, Coles ET, Crane M et al. 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): 427–38.
18. 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.
19. Travis WD, Hoffman GS, Leavitt RY et al. Surgical pathology of the lung in Wegener’s granulomatosis. Am J Surg Pathol 1991; 15 (4): 315–33.
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: 450–8.
21. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol 2008; 26 (Suppl. 51): S94–104.
22. 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.
23. Katzenstein AL. Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review. Am J Clin Pathol 2000; 114 (5): 767–72.
24. Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 2003; 115 (4): 284–90.
25. Guillevin L, Durand-Gasselin B, Cevallos R et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 1999; 42 (3): 421–30.
26. Lhote F, Cohen P, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis and Churg-Strauss syndrome. Lupus 1998; 7 (4): 238–58.
27. Flossmann O. Risks of treatments and long-term outcomes of systemic ANCA-associated vasculitis. Presse Med 2015; 44 (6 Pt 2): e251–257.
28. Luqmani RA. State of the art in the treatment of systemic vasculitides. Front Immunol 2014; 5: 1–9.
29. 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.
30. Harper L, Morgan MD, Walsh M et al.; 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): 955–60.
31. Stone JH, Merkel PA, Spiera R et al; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363 (3): 221–32.
32. Schmitt WH, Hagen EC, Neumann I et al; European Vasculitis Study Group. Treatment of refractory Wegeners granulomatosis with antithymocyte globulin (ATG): an open study in 15 patients. Kidney Int 2004; 65 (4): 1440–48.
33. Haubitz M, de Groot K. Tolerance of mycophenolate mofetil in end-stage renal disease patients with ANCA-associated vasculitis. Clin Nephrol 2002; 57 (6): 421–4.
34. Smith RM, Jones RB, Guerry MJ et al. Rituximab for remission maintenance in relapsing antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2012; 64 (11): 3760–9.
35. Cohen Tervaert JW, Stegeman CA, Kallenberg CG. Novel therapies for anti-neutrophil cytoplasmic antibody-associated vasculitis. Curr Opin Nephrol Hypertens 2001; 10 (2): 211–7.
36. Langford CA, Sneller MC. Biologic therapies in the vasculitides. Curr Opin Rheumatol 2003; 15: 3–10.
37. Jones RB. Rituximab in the treatment of anti-neutrophil cytoplasm antibody-associated vasculitis. Nephron Clin Pract 2014; 128 (3–4): 243–9.
38. de Groot K, Muhler M, Reinhold-Keller E et al. Induction of remission in Wegener’s granulomatosis with low dose methotrexate. J Rheumatol 1998; 25 (3): 492–5.
39. Jones RB, Tervaert JW, Hauser T et al; European Vasculitis Study Group. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010; 363 (3): 211–20.
40. Gaskin G, Pusey C. Plasmapheresis in antineutrophil cytoplasmic antibody-associated systemic vasculitis. Ther Apher 2001; 5 (3): 176–81.
41. Klemmer PJ, Chalermskulrat W, Reif MS et al. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis 2003; 42 (6): 1149–53.
42. Pagnoux C, Mahr A, Hamidou MA et al; French Vasculitis Study Group. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med 2008; 359 (26): 2790–803.
43. Metzler C, Fink C, Lamprecht P et al. Maintenance of remission with leflunomide in Wegener’s granulomatosis. Rheumatology (Oxford) 2004; 43 (3): 315–20.
44. Hiemstra TF, Walsh M, Mahr A et al; European Vasculitis Study Group (EUVAS). Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA 2010; 304 (21): 2381–8.
45. Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CGM. Trimethoprim-sulfamethoxazole for the prevention of relapses of Wegener’s granulomatosis. N Engl J Med 1996; 335: 16–20.
46. Ohtake T, Kobayashi S, Honjou Y et al. Generalized Wegener’s granulomatosis responding to sulfamethoxazole-trimethoprim monotherapy. Intern Med 2001; 40 (7): 666–70.
________________________________________________
1. Flossmann O, Berden A, de Groot K et al.; European Vasculitis Study Group. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 2011; 70 (3): 488–94.
2. Yunt ZX, Frankel SK, Brown KK. Diagnosis and management of pulmonary vasculitis. Ther Adv Respir Dis 2012; 6 (6): 375–90.
3. 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.
4. Mandl LA, Solomon DH, Smith EL et al. Using antineutrophil cytoplasmic antibody testing to diagnose vasculitis: can test-ordering guidelines improve diagnostic accuracy? Arch Intern Med 2002; 162 (13): 1509–14.
5. Frankel SK, Sullivan EJ, Brown KK. Vasculitis: Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa, and Takayasu arteritis. Crit Care Clin 2002; 18 (4): 855–79.
6. Swerlick RA, Lawley TJ. Cutaneous vasculitis: its relationship to systemic disease. Med Clin North Am 1989; 73 (5): 1221–35.
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): 865–86.
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 et al. Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on myeloperoxidase antibodies. J Rheumatol 2001; 28 (7): 1584–90.
10. Savige J, Gillis D, Benson E et al. International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA). Am J Clin Pathol 1999; 111 (4): 507–13.
11. Finkielman JD, Lee AS, Hummel AM et al.; 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.
12. Mukhtyar C, Guillevin L, Cid MC et al.; European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009; 68 (3): 310–17.
13. Cordier JF, Valeyre D, Guillevin L et al. Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest 1990; 97: 906–12.
14. Guillevin L, Cohen P, Gayraud M et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine 1999; 78 (1): 26–37.
15. Ananthakrishnan L, Sharma N, Kanne JP. Wegener’s granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol 2009; 192 (3): 676–82.
16. Hoffman GS, Kerr GS, Leavitt RY et al. Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116 (6): 488–98.
17. Anderson G, Coles ET, Crane M et al. 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): 427–38.
18. 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.
19. Travis WD, Hoffman GS, Leavitt RY et al. Surgical pathology of the lung in Wegener’s granulomatosis. Am J Surg Pathol 1991; 15 (4): 315–33.
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: 450–8.
21. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol 2008; 26 (Suppl. 51): S94–104.
22. 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.
23. Katzenstein AL. Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review. Am J Clin Pathol 2000; 114 (5): 767–72.
24. Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 2003; 115 (4): 284–90.
25. Guillevin L, Durand-Gasselin B, Cevallos R et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 1999; 42 (3): 421–30.
26. Lhote F, Cohen P, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis and Churg-Strauss syndrome. Lupus 1998; 7 (4): 238–58.
27. Flossmann O. Risks of treatments and long-term outcomes of systemic ANCA-associated vasculitis. Presse Med 2015; 44 (6 Pt 2): e251–257.
28. Luqmani RA. State of the art in the treatment of systemic vasculitides. Front Immunol 2014; 5: 1–9.
29. 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.
30. Harper L, Morgan MD, Walsh M et al.; 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): 955–60.
31. Stone JH, Merkel PA, Spiera R et al; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363 (3): 221–32.
32. Schmitt WH, Hagen EC, Neumann I et al; European Vasculitis Study Group. Treatment of refractory Wegeners granulomatosis with antithymocyte globulin (ATG): an open study in 15 patients. Kidney Int 2004; 65 (4): 1440–48.
33. Haubitz M, de Groot K. Tolerance of mycophenolate mofetil in end-stage renal disease patients with ANCA-associated vasculitis. Clin Nephrol 2002; 57 (6): 421–4.
34. Smith RM, Jones RB, Guerry MJ et al. Rituximab for remission maintenance in relapsing antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2012; 64 (11): 3760–9.
35. Cohen Tervaert JW, Stegeman CA, Kallenberg CG. Novel therapies for anti-neutrophil cytoplasmic antibody-associated vasculitis. Curr Opin Nephrol Hypertens 2001; 10 (2): 211–7.
36. Langford CA, Sneller MC. Biologic therapies in the vasculitides. Curr Opin Rheumatol 2003; 15: 3–10.
37. Jones RB. Rituximab in the treatment of anti-neutrophil cytoplasm antibody-associated vasculitis. Nephron Clin Pract 2014; 128 (3–4): 243–9.
38. de Groot K, Muhler M, Reinhold-Keller E et al. Induction of remission in Wegener’s granulomatosis with low dose methotrexate. J Rheumatol 1998; 25 (3): 492–5.
39. Jones RB, Tervaert JW, Hauser T et al; European Vasculitis Study Group. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010; 363 (3): 211–20.
40. Gaskin G, Pusey C. Plasmapheresis in antineutrophil cytoplasmic antibody-associated systemic vasculitis. Ther Apher 2001; 5 (3): 176–81.
41. Klemmer PJ, Chalermskulrat W, Reif MS et al. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis 2003; 42 (6): 1149–53.
42. Pagnoux C, Mahr A, Hamidou MA et al; French Vasculitis Study Group. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med 2008; 359 (26): 2790–803.
43. Metzler C, Fink C, Lamprecht P et al. Maintenance of remission with leflunomide in Wegener’s granulomatosis. Rheumatology (Oxford) 2004; 43 (3): 315–20.
44. Hiemstra TF, Walsh M, Mahr A et al; European Vasculitis Study Group (EUVAS). Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA 2010; 304 (21): 2381–8.
45. Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CGM. Trimethoprim-sulfamethoxazole for the prevention of relapses of Wegener’s granulomatosis. N Engl J Med 1996; 335: 16–20.
46. Ohtake T, Kobayashi S, Honjou Y et al. Generalized Wegener’s granulomatosis responding to sulfamethoxazole-trimethoprim monotherapy. Intern Med 2001; 40 (7): 666–70.
Авторы
Э.Х.Анаев*
ФГБОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И.Пирогова» Минздрава России. 117997, Россия, Москва, ул. Островитянова, д. 1
N.I.Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation. 117997, Russian Federation, Moscow, ul. Ostrovitianova, d. 1