Алгоритм диагностики системных васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами
Алгоритм диагностики системных васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами
Материалы доступны только для специалистов сферы здравоохранения. Авторизуйтесь или зарегистрируйтесь.
Аннотация
Ассоциированные с антинейтрофильными цитоплазматическими антителами системные васкулиты (АНЦА-СВ), включающие гранулематоз с полиангиитом Вегенера (ГПА), микроскопический полиангиит (МПА) и эозинофильный гранулематоз с полиангиитом Черджа–Стросса (ЭГПА), относятся к редким аутоиммунным заболеваниям с тяжелым полиорганным поражением и фатальным прогнозом при отсутствии адекватного лечения. Первоочередное значение для успешной терапии имеет ранняя диагностика, до развития необратимого повреждения органов и систем. Тем не менее, в реальной клинической практике диагностика АНЦА-СВ остается субоптимальной, что дополнительно осложняется отсутствием до настоящего времени классификационных критериев (КК) МПА, ключевым отличием которого является отсутствие гранулематозного воспаления.
На основании анализа 251 больного АНЦА-СВ с доказанной гиперпродукцией АНЦА (средний возраст 43,1 ± 15,9 года, мужчины 40%) и длительностью наблюдения от начала заболевания 3 (0,2–28,5) года, разработан диагностический алгоритм определения нозологической принадлежности АНЦА-СВ, в котором использована последовательная оценка соответствия КК-ЭГПА, альтернативным суррогатным критериям (СК) гранулематозного воспаления и СК некротизирующего васкулита. Диагноз МПА соответствовал общепринятому определению МПА, подтверждался отсутствием КК-ЭГПА, наличием СК васкулита и отсутствием СК гранулематоза. При диагностике ГПА и ЭГПА дополнительно учитывались соответствующие КК.
Применение СК в качестве базового принципа диагностики позволило классифицировать 99% случаев АНЦА-СВ. Наиболее распространенными формами были ГПА (53%) и МПА (37%), значительно реже встречался ЭГПА (9%). МПА характеризовался наиболее агрессивным течением и высокой общей летальностью (18%), при ЭГПА и ГПА показатели выживаемости оказались более благоприятны, общая летальность составила 5–7% (р=0,003). При этом у больных МПА со специфичностью АНЦА к протеиназе-3 выживаемость в первые два года заболевания оказалась ниже, чем в группе МПА с антителами к миелопероксидазе (р=0,04), что в первую очередь обусловлено более высокой частотой легочного кровотечения и быстропрогрессирующего гломерулонефрита. Рецидивы чаще развивались при ЭГПА (80%) и ГПА (64%), чем при МПА (49%). Выявленные различия в группах ГПА, МПА и ЭГПА подтверждают диагностическую ценность алгоритма.
Дальнейшее внедрение предложенного алгоритма в клиническую практику позволит совершенствовать диагностику АНЦА-СВ с уточнением конкретной нозологической формы, во многом определяющей прогноз, и оптимизировать лечение. Решающее значение в диагностике АНЦА-СВ принадлежит целенаправленному и планомерному клиническому обследованию пациентов.
Ключевые слова: антинейтрофильные цитоплазматические антитела, системный васкулит, АНЦА-ассоциированный системный васкулит, гранулематоз с полиангиитом, микроскопический полиангиит, эозинофильный гранулематоз с полиангиитом.
The presented paper deals with the results of the analysis of 251 patients with AAV (mean age 43.1 ± 15.9 years, men 40%, disease duration 3 (0,2-28,5) years, ANCA 100%), based on which the diagnostic algorithm was developed. The algorithm steps include classification criteria of EGPA as well as surrogate markers for granulomatous inflammation (SG) and vasculitis (SV). MPA confirmed by the absence of criteria for EGPA, the presence of SV and the absence of SG.
Due to the algorithm usage, nosological affiliation of AAV was determined in 99% patients. Both GPA and MPA were the most common (53% and 37%), while EGPA was rare (9%). In MPA group the overall mortality was higher (18%) than GPA and EGPA (7-5%), p=0.003. In MPA with anti- proteinase 3 antibody the two-year survival rate was lower than those with anti-myeloperoxidase antibody (p=0.04), mainly because of the high risk for alveolar hemorrhage and rapidly progressive glomerulonephritis. Relapses occurred more frequently in EGPA (80%) and in GPA (64%) and less frequently in MPA (49%). The group differences confirm diagnostic value of the algorithm.
In conclusion, the proposed algorithm will help to improve the diagnosis of AAV. It is important that crucial in the AAV diagnosis belongs focused and systematic clinical examination of patients.
Keywords: antineutrophil cytoplasmic antibody, systemic vasculitis, ANCA-associated systemic vasculitis, granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis.
На основании анализа 251 больного АНЦА-СВ с доказанной гиперпродукцией АНЦА (средний возраст 43,1 ± 15,9 года, мужчины 40%) и длительностью наблюдения от начала заболевания 3 (0,2–28,5) года, разработан диагностический алгоритм определения нозологической принадлежности АНЦА-СВ, в котором использована последовательная оценка соответствия КК-ЭГПА, альтернативным суррогатным критериям (СК) гранулематозного воспаления и СК некротизирующего васкулита. Диагноз МПА соответствовал общепринятому определению МПА, подтверждался отсутствием КК-ЭГПА, наличием СК васкулита и отсутствием СК гранулематоза. При диагностике ГПА и ЭГПА дополнительно учитывались соответствующие КК.
Применение СК в качестве базового принципа диагностики позволило классифицировать 99% случаев АНЦА-СВ. Наиболее распространенными формами были ГПА (53%) и МПА (37%), значительно реже встречался ЭГПА (9%). МПА характеризовался наиболее агрессивным течением и высокой общей летальностью (18%), при ЭГПА и ГПА показатели выживаемости оказались более благоприятны, общая летальность составила 5–7% (р=0,003). При этом у больных МПА со специфичностью АНЦА к протеиназе-3 выживаемость в первые два года заболевания оказалась ниже, чем в группе МПА с антителами к миелопероксидазе (р=0,04), что в первую очередь обусловлено более высокой частотой легочного кровотечения и быстропрогрессирующего гломерулонефрита. Рецидивы чаще развивались при ЭГПА (80%) и ГПА (64%), чем при МПА (49%). Выявленные различия в группах ГПА, МПА и ЭГПА подтверждают диагностическую ценность алгоритма.
Дальнейшее внедрение предложенного алгоритма в клиническую практику позволит совершенствовать диагностику АНЦА-СВ с уточнением конкретной нозологической формы, во многом определяющей прогноз, и оптимизировать лечение. Решающее значение в диагностике АНЦА-СВ принадлежит целенаправленному и планомерному клиническому обследованию пациентов.
Ключевые слова: антинейтрофильные цитоплазматические антитела, системный васкулит, АНЦА-ассоциированный системный васкулит, гранулематоз с полиангиитом, микроскопический полиангиит, эозинофильный гранулематоз с полиангиитом.
________________________________________________
The presented paper deals with the results of the analysis of 251 patients with AAV (mean age 43.1 ± 15.9 years, men 40%, disease duration 3 (0,2-28,5) years, ANCA 100%), based on which the diagnostic algorithm was developed. The algorithm steps include classification criteria of EGPA as well as surrogate markers for granulomatous inflammation (SG) and vasculitis (SV). MPA confirmed by the absence of criteria for EGPA, the presence of SV and the absence of SG.
Due to the algorithm usage, nosological affiliation of AAV was determined in 99% patients. Both GPA and MPA were the most common (53% and 37%), while EGPA was rare (9%). In MPA group the overall mortality was higher (18%) than GPA and EGPA (7-5%), p=0.003. In MPA with anti- proteinase 3 antibody the two-year survival rate was lower than those with anti-myeloperoxidase antibody (p=0.04), mainly because of the high risk for alveolar hemorrhage and rapidly progressive glomerulonephritis. Relapses occurred more frequently in EGPA (80%) and in GPA (64%) and less frequently in MPA (49%). The group differences confirm diagnostic value of the algorithm.
In conclusion, the proposed algorithm will help to improve the diagnosis of AAV. It is important that crucial in the AAV diagnosis belongs focused and systematic clinical examination of patients.
Keywords: antineutrophil cytoplasmic antibody, systemic vasculitis, ANCA-associated systemic vasculitis, granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis.
Список литературы
1. 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.org/ 10.1002/art.37715
2. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr, et al. The American college of rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthr Rheum. 1990;33:1101-7.
3. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094-100.
4. Jayne D. Challenges in the management of microscopic polyangiitis. Current opinion in rheumatology. 2008;20(1):3-9. https://doi.org/ 10.1097/BOR.0b013e3282f370d1
5. Bossuyt X, Cohen Tervaert JW, Arimura Y, Blockmans D, Flores-Suárez LF, Guillevin L, Hellmich B, Jayne D, Jennette JC, Kallenberg CGM, Moiseev S, Novikov P, Radice A, Savige JA, Sinico RA, Specks U, van Paassen P, Zhao MH, Rasmussen N, Damoiseaux J, Csernok E. Position paper: Revised 2017 international consensus ont esting of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol. 2017;13(11):683-692. http://dx.doi.org/ 10.1038/nrrheum.2017.140
6. Devaney KO, Travis WD, Hoffman G, Leavitt R, Lebovics R, Fauci AS. Interpretation of head and neck biopsies in Wegener's granulomatosis. A pathologic study of 126 biopsies in 70 patients. Am J Surg Pathol. 1990;14(6):555-64.
7. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D. Development and validation of a consensus methodology for the classification of the
ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66:222-7.
8. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, Hellmich B, Holle JU, Laudien M, Little MA, Luqmani RA, Mahr A, Merkel PA, Mills J, Mooney J, Segelmark M, Tesar V, Westman K, Vaglio A, Yalçındağ N, Jayne DR, Mukhtyar C. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583-94. https://doi.org/10.1136/annrheumdis-2016-209133
9. Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, Savage C, Adu D. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87(11):671–678.
10. Бекетова Т.В. Асимптомное течение поражения легких при гранулематозе с полиангиитом (Вегенера). Научно-практическая ревматология. 2012;1:102-104. [Beketova TV. Asymptomatic course of lung damage in granulomatosis with polyangiitis (Wegener’s). Rheumatology Science and Practice. 2014;52(1):102-104. (In Russ.)]. http://dx.doi.org/10.14412/1995-4484-2014-102-104
11. Koldingsnes W, Nossent H. Epidemiology of Wegener's granulomatosis in northern Norway. Arthritis and rheumatism. 2000;43(11):2481-2487.
12. Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A, Luberto F, Pipitone N, Salvarani C. Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. Seminars in arthritis and rheumatism. 2014;44(2):202-207. http://dx.doi.org/10.1016/j.semarthrit.2014.05. 005
13. Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis. A ten year study in the United Kingdom. Arthritis and rheumatism. 2000;43(2):414-419.
14. Nesher G, Ben-Chetrit E, Mazal B, Breuer G. The Incidence of Primary Systemic Vasculitis in Jerusalem: A 20-year Hospital-based Retrospective Study. J Rheumatol. 2016;43(6):1072-7. http://dx.doi.org/ 10.3899/jrheum.150557
15. Andrews M, Edmunds M, Campbell A, Walls J, Feehally J. Systemic vasculitis in the 1980s- Is theere are increasing incidence of Wegener's granulomatosis and microscopic polyarteritis? J Royal Coll Phys Lond. 1990; 24:284-8.
16. Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: presentation, pathology and prognosis. QJM. 1985;56(220):467-483.
17. 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.
18. Lane SE, Watts RA, Shepstone L, Scott DG. Primary systemic vasculitis: clinical features and mortality. QJM. 2005;98(2):97-111.
19. Ahn JK, Hwang JW, Lee J, Jeon CH, Cha HS, Koh EM. Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated vasculitides in Korea. Rheumatol Int. 2011;32(10):2979-2986.
20. Huang CK, Fang HC, Chou KJ, Wang JS, Lee PT, Chang TY, Hsu CY, Huang WC, Chung HM, Chen CL. Clinical features of microscopic polyangiitis: a cohort study in a southern Taiwan medical center. Acta Nephrologica. 2012;26(4):198-205. http://dx.doi.org/10.6221/AN. 2011028
21. Захарова Е.В., Яковлев В.Н., Виноградова О.В., Шейх Ж.В., Жидкова Н.В., Алексеев В.Г. ANCA-ассоциированные васкулиты с поражением легких и почек: клинико-морфологическая характеристика, лечение, исходы. Клиническая медицина. 2013;91(7):38-43. [Zakharova EV, Iakovlev VN, Vinogradova OV, Sheikh ZhV, Zhidkova NV, Alekseev VG. ANCA-associated vasculitis affecting lungs and kidneys: clinical and morphological characteristic, treatment, outcomes. Klinicheskaia meditsina. 2013;91(7):38-43 (In Russ.)].
22. Pagnoux C, Carette S, Khalidi NA, Walsh M, Hiemstra TF, Cuthbertson D, Langford C, Hoffman G, Koening CL, Monach PA, Moreland L, Mouthon L, Seo P, Specks U, Ytterberg S, Westman K, Hoglund P, Harper L, Flossman O, Luqmani R, Savage CO, Rasmussen N, de Groot K, Tesar V, Jayne D, Merkel PA, Guillevin L. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts. Clin Exp Rheumatol. 2015;33(2 Suppl 89):77-83.
23. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19-27. http://dx.doi.org/10.1097/MD.0b013e318205a4c6
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. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis rheum. 2013;65(1):270-281. http://dx.doi.org/10.1002/art.37721
25. Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M. Incidence and survival rates in Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford). 2009;48(12):1560-1565. http://dx.doi.org/ 10.1093/rheumatology/kep304
26. Miloslavsky EM, Lu N, Unizony S, Choi HK, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Tchao NK, Fervenza F, Monach PA, Specks U, Stone JH. Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients With Granulomatosis With Polyangiitis (Wegener's): Distinct Patient Subsets. Arthritis Rheumatol. 2016;68(12):2945-2952. http://dx.doi.org/10.1002/art.39812
27. Schönermarck U, Lamprecht P, Csernok E, Gross WL. Prevalence and spectrum of rheumatic diseases associated with proteinase 3 antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Rheumatology (Oxford). 2001;40(2):178-184.
28. Zycinska K, Wardyn KA, Zycinski Z, Zielonka TM. Association between clinical activity and high-resolution tomography findings in pulmonary. Physiol Pharmacol. 2008;59(Suppl. 6):833-838.
29. Pagnoux C, Guillevin L. Churg-Strauss syndrome: evidence for disease subtypes? Curr Opin Rheumatol. 2010;22(1):21-28. http://dx.doi. org/10.1097/BOR.0b013e328333390b
30. Stangou M, Asimaki A, Bamichas G, Christidou F, Zoumbaridis N, Natse T, Galanis N, Christaki P, Patakas D, Sombolos K. Factors influencing patient survival and renal function outcome in pulmonary-renal syndrome associated with ANCA (+) vasculitis: A single-center experience. J Nephrol. 2005;18(1):35-44.
31. Weidner S, Geuss S, Hafezi-Rachti S, Wonka A, Rupprecht HD. ANCA-associated vasculitis with renal involvement: an outcome analysis. Nephrol Dial Transplant. 2004;19(6):1403-1411.
32. Homma S, Matsushita H, Nakata K. Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Respirology. 2004;9(2):190-196.
33. Ando M, Miyazaki E, Ishii T, Mukai Y, Yamasue M, Fujisaki H, Ito T, Nureki S, Kumamoto T. Incidence of myeloperoxidase anti-neutrophil cytoplasmic antibody positivity and microscopic polyangitis in the course of idiopathic pulmonary fibrosis. Respir Med. 2013;107(4):608-615. http://dx.doi.org/10.1016/j.rmed.2013.01.006
34. Foulon G, Delaval P, Valeyre D, Wallaert B, Debray MP, Brauner M, Nicaise P, Cadranel J, Cottin V, Tazi A, Aubier M, Crestani B. ANCA-associated lung fibrosis: Analysis of 17 patients. Respir Med. 2008;102(10):1392-1398. http://dx.doi.org/10.1016/j.rmed.2008.04. 023
35. Puéchal X, Pagnoux C, Perrodeau É, Hamidou M, Boffa JJ, Kyndt X, Lifermann F, Papo T, Merrien D, Smail A, Delaval P, Hanrotel-Saliou C, Imbert B, Khouatra C, Lambert M, Leské C, Ly KH, Pertuiset E, Roblot P, Ruivard M, Subra JF, Viallard JF, Terrier B, Cohen P, Mouthon L, Le Jeunne C, Ravaud P, Guillevin L. Long-term outcomes of the WEGENT trial on remission-maintenance for granulomatosis with polyangiitis or microscopic polyangiitis. Arthritis Rheumatol. 2016;68(3):690-701.
36. Walsh M, Flossmann O, Berden A, Westman K, Höglund P, Stegeman C, Jayne D. Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2012;64(2):542-548. http://dx.doi.org/10.1002/art.33361
37. Sada KE, Yamamura M, Harigai M, Fujii T, Takasaki Y, Amano K, Fujimoto S, Muso E, Murakawa Y, Arimura Y, Makino H. Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study. Arthritis Res Ther. 2015;17(1):305. http://dx.doi.org/10. 1186/s13075-015-0815-y
38. Li ZY, Chang DY, Zhao MH, Chen M. Predictors of treatment resistance and relapse in ANCA-associated vasculitis: a study of 439 cases in a single Chinese center. Arthritis Rheumatol. 2014;66(7):1920-1926. http://dx.doi.org/10.1002/art.38621
39. Pierrot-Deseilligny Despujol C, Pouchot J, Pagnoux C, Coste J, Guillevin L. Predictors at diagnosis of a first Wegener's granulomatosis relapse after obtaining complete remission. Rheumatology(Oxford). 2010;49(11):2181-2190. http://dx.doi.org/10.1093/rheumatology/ keq244
40. Moiseev S, Novikov P, Jayne D, Mukhin N. End-stage renal disease in ANCA-associated vasculitis. Nephrol Dial Transplant. 2017;32(2): 248-253. http://dx.doi.org/10.1093/ndt/gfw046
41. Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, Dalhoff K, Dunogué B, Gross W, Holle J, Humbert M, Jayne D, Jennette JC, Lazor R, Mahr A, Merkel PA, Mouthon L, Sinico RA, Specks U, Vaglio A, Wechsler ME, Cordier JF, Guillevin L. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Int Med. 2015;26(7):545-553. http://dx.doi.org/10.1016/j.ejim.2015.04.022
2. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr, et al. The American college of rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthr Rheum. 1990;33:1101-7.
3. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094-100.
4. Jayne D. Challenges in the management of microscopic polyangiitis. Current opinion in rheumatology. 2008;20(1):3-9. https://doi.org/ 10.1097/BOR.0b013e3282f370d1
5. Bossuyt X, Cohen Tervaert JW, Arimura Y, Blockmans D, Flores-Suárez LF, Guillevin L, Hellmich B, Jayne D, Jennette JC, Kallenberg CGM, Moiseev S, Novikov P, Radice A, Savige JA, Sinico RA, Specks U, van Paassen P, Zhao MH, Rasmussen N, Damoiseaux J, Csernok E. Position paper: Revised 2017 international consensus ont esting of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol. 2017;13(11):683-692. http://dx.doi.org/ 10.1038/nrrheum.2017.140
6. Devaney KO, Travis WD, Hoffman G, Leavitt R, Lebovics R, Fauci AS. Interpretation of head and neck biopsies in Wegener's granulomatosis. A pathologic study of 126 biopsies in 70 patients. Am J Surg Pathol. 1990;14(6):555-64.
7. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D. Development and validation of a consensus methodology for the classification of the
ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66:222-7.
8. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, Hellmich B, Holle JU, Laudien M, Little MA, Luqmani RA, Mahr A, Merkel PA, Mills J, Mooney J, Segelmark M, Tesar V, Westman K, Vaglio A, Yalçındağ N, Jayne DR, Mukhtyar C. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583-94. https://doi.org/10.1136/annrheumdis-2016-209133
9. Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, Savage C, Adu D. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87(11):671–678.
10. [Beketova TV. Asymptomatic course of lung damage in granulomatosis with polyangiitis (Wegener’s). Rheumatology Science and Practice. 2014;52(1):102-104. (In Russ.)]. http://dx.doi.org/10.14412/1995-4484-2014-102-104
11. Koldingsnes W, Nossent H. Epidemiology of Wegener's granulomatosis in northern Norway. Arthritis and rheumatism. 2000;43(11):2481-2487.
12. Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A, Luberto F, Pipitone N, Salvarani C. Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. Seminars in arthritis and rheumatism. 2014;44(2):202-207. http://dx.doi.org/10.1016/j.semarthrit.2014.05. 005
13. Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis. A ten year study in the United Kingdom. Arthritis and rheumatism. 2000;43(2):414-419.
14. Nesher G, Ben-Chetrit E, Mazal B, Breuer G. The Incidence of Primary Systemic Vasculitis in Jerusalem: A 20-year Hospital-based Retrospective Study. J Rheumatol. 2016;43(6):1072-7. http://dx.doi.org/ 10.3899/jrheum.150557
15. Andrews M, Edmunds M, Campbell A, Walls J, Feehally J. Systemic vasculitis in the 1980s- Is theere are increasing incidence of Wegener's granulomatosis and microscopic polyarteritis? J Royal Coll Phys Lond. 1990; 24:284-8.
16. Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: presentation, pathology and prognosis. QJM. 1985;56(220):467-483.
17. 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.
18. Lane SE, Watts RA, Shepstone L, Scott DG. Primary systemic vasculitis: clinical features and mortality. QJM. 2005;98(2):97-111.
19. Ahn JK, Hwang JW, Lee J, Jeon CH, Cha HS, Koh EM. Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated vasculitides in Korea. Rheumatol Int. 2011;32(10):2979-2986.
20. Huang CK, Fang HC, Chou KJ, Wang JS, Lee PT, Chang TY, Hsu CY, Huang WC, Chung HM, Chen CL. Clinical features of microscopic polyangiitis: a cohort study in a southern Taiwan medical center. Acta Nephrologica. 2012;26(4):198-205. http://dx.doi.org/10.6221/AN. 2011028
21. [Zakharova EV, Iakovlev VN, Vinogradova OV, Sheikh ZhV, Zhidkova NV, Alekseev VG. ANCA-associated vasculitis affecting lungs and kidneys: clinical and morphological characteristic, treatment, outcomes. Klinicheskaia meditsina. 2013;91(7):38-43 (In Russ.)].
22. Pagnoux C, Carette S, Khalidi NA, Walsh M, Hiemstra TF, Cuthbertson D, Langford C, Hoffman G, Koening CL, Monach PA, Moreland L, Mouthon L, Seo P, Specks U, Ytterberg S, Westman K, Hoglund P, Harper L, Flossman O, Luqmani R, Savage CO, Rasmussen N, de Groot K, Tesar V, Jayne D, Merkel PA, Guillevin L. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts. Clin Exp Rheumatol. 2015;33(2 Suppl 89):77-83.
23. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19-27. http://dx.doi.org/10.1097/MD.0b013e318205a4c6
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. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis rheum. 2013;65(1):270-281. http://dx.doi.org/10.1002/art.37721
25. Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M. Incidence and survival rates in Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford). 2009;48(12):1560-1565. http://dx.doi.org/ 10.1093/rheumatology/kep304
26. Miloslavsky EM, Lu N, Unizony S, Choi HK, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Tchao NK, Fervenza F, Monach PA, Specks U, Stone JH. Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients With Granulomatosis With Polyangiitis (Wegener's): Distinct Patient Subsets. Arthritis Rheumatol. 2016;68(12):2945-2952. http://dx.doi.org/10.1002/art.39812
27. Schönermarck U, Lamprecht P, Csernok E, Gross WL. Prevalence and spectrum of rheumatic diseases associated with proteinase 3 antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Rheumatology (Oxford). 2001;40(2):178-184.
28. Zycinska K, Wardyn KA, Zycinski Z, Zielonka TM. Association between clinical activity and high-resolution tomography findings in pulmonary. Physiol Pharmacol. 2008;59(Suppl. 6):833-838.
29. Pagnoux C, Guillevin L. Churg-Strauss syndrome: evidence for disease subtypes? Curr Opin Rheumatol. 2010;22(1):21-28. http://dx.doi. org/10.1097/BOR.0b013e328333390b
30. Stangou M, Asimaki A, Bamichas G, Christidou F, Zoumbaridis N, Natse T, Galanis N, Christaki P, Patakas D, Sombolos K. Factors influencing patient survival and renal function outcome in pulmonary-renal syndrome associated with ANCA (+) vasculitis: A single-center experience. J Nephrol. 2005;18(1):35-44.
31. Weidner S, Geuss S, Hafezi-Rachti S, Wonka A, Rupprecht HD. ANCA-associated vasculitis with renal involvement: an outcome analysis. Nephrol Dial Transplant. 2004;19(6):1403-1411.
32. Homma S, Matsushita H, Nakata K. Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Respirology. 2004;9(2):190-196.
33. Ando M, Miyazaki E, Ishii T, Mukai Y, Yamasue M, Fujisaki H, Ito T, Nureki S, Kumamoto T. Incidence of myeloperoxidase anti-neutrophil cytoplasmic antibody positivity and microscopic polyangitis in the course of idiopathic pulmonary fibrosis. Respir Med. 2013;107(4):608-615. http://dx.doi.org/10.1016/j.rmed.2013.01.006
34. Foulon G, Delaval P, Valeyre D, Wallaert B, Debray MP, Brauner M, Nicaise P, Cadranel J, Cottin V, Tazi A, Aubier M, Crestani B. ANCA-associated lung fibrosis: Analysis of 17 patients. Respir Med. 2008;102(10):1392-1398. http://dx.doi.org/10.1016/j.rmed.2008.04. 023
35. Puéchal X, Pagnoux C, Perrodeau É, Hamidou M, Boffa JJ, Kyndt X, Lifermann F, Papo T, Merrien D, Smail A, Delaval P, Hanrotel-Saliou C, Imbert B, Khouatra C, Lambert M, Leské C, Ly KH, Pertuiset E, Roblot P, Ruivard M, Subra JF, Viallard JF, Terrier B, Cohen P, Mouthon L, Le Jeunne C, Ravaud P, Guillevin L. Long-term outcomes of the WEGENT trial on remission-maintenance for granulomatosis with polyangiitis or microscopic polyangiitis. Arthritis Rheumatol. 2016;68(3):690-701.
36. Walsh M, Flossmann O, Berden A, Westman K, Höglund P, Stegeman C, Jayne D. Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2012;64(2):542-548. http://dx.doi.org/10.1002/art.33361
37. Sada KE, Yamamura M, Harigai M, Fujii T, Takasaki Y, Amano K, Fujimoto S, Muso E, Murakawa Y, Arimura Y, Makino H. Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study. Arthritis Res Ther. 2015;17(1):305. http://dx.doi.org/10. 1186/s13075-015-0815-y
38. Li ZY, Chang DY, Zhao MH, Chen M. Predictors of treatment resistance and relapse in ANCA-associated vasculitis: a study of 439 cases in a single Chinese center. Arthritis Rheumatol. 2014;66(7):1920-1926. http://dx.doi.org/10.1002/art.38621
39. Pierrot-Deseilligny Despujol C, Pouchot J, Pagnoux C, Coste J, Guillevin L. Predictors at diagnosis of a first Wegener's granulomatosis relapse after obtaining complete remission. Rheumatology(Oxford). 2010;49(11):2181-2190. http://dx.doi.org/10.1093/rheumatology/ keq244
40. Moiseev S, Novikov P, Jayne D, Mukhin N. End-stage renal disease in ANCA-associated vasculitis. Nephrol Dial Transplant. 2017;32(2): 248-253. http://dx.doi.org/10.1093/ndt/gfw046
41. Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, Dalhoff K, Dunogué B, Gross W, Holle J, Humbert M, Jayne D, Jennette JC, Lazor R, Mahr A, Merkel PA, Mouthon L, Sinico RA, Specks U, Vaglio A, Wechsler ME, Cordier JF, Guillevin L. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Int Med. 2015;26(7):545-553. http://dx.doi.org/10.1016/j.ejim.2015.04.022
2. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr, et al. The American college of rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthr Rheum. 1990;33:1101-7.
3. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094-100.
4. Jayne D. Challenges in the management of microscopic polyangiitis. Current opinion in rheumatology. 2008;20(1):3-9. https://doi.org/ 10.1097/BOR.0b013e3282f370d1
5. Bossuyt X, Cohen Tervaert JW, Arimura Y, Blockmans D, Flores-Suárez LF, Guillevin L, Hellmich B, Jayne D, Jennette JC, Kallenberg CGM, Moiseev S, Novikov P, Radice A, Savige JA, Sinico RA, Specks U, van Paassen P, Zhao MH, Rasmussen N, Damoiseaux J, Csernok E. Position paper: Revised 2017 international consensus ont esting of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol. 2017;13(11):683-692. http://dx.doi.org/ 10.1038/nrrheum.2017.140
6. Devaney KO, Travis WD, Hoffman G, Leavitt R, Lebovics R, Fauci AS. Interpretation of head and neck biopsies in Wegener's granulomatosis. A pathologic study of 126 biopsies in 70 patients. Am J Surg Pathol. 1990;14(6):555-64.
7. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D. Development and validation of a consensus methodology for the classification of the
ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66:222-7.
8. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, Hellmich B, Holle JU, Laudien M, Little MA, Luqmani RA, Mahr A, Merkel PA, Mills J, Mooney J, Segelmark M, Tesar V, Westman K, Vaglio A, Yalçındağ N, Jayne DR, Mukhtyar C. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583-94. https://doi.org/10.1136/annrheumdis-2016-209133
9. Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, Savage C, Adu D. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87(11):671–678.
10. Бекетова Т.В. Асимптомное течение поражения легких при гранулематозе с полиангиитом (Вегенера). Научно-практическая ревматология. 2012;1:102-104. [Beketova TV. Asymptomatic course of lung damage in granulomatosis with polyangiitis (Wegener’s). Rheumatology Science and Practice. 2014;52(1):102-104. (In Russ.)]. http://dx.doi.org/10.14412/1995-4484-2014-102-104
11. Koldingsnes W, Nossent H. Epidemiology of Wegener's granulomatosis in northern Norway. Arthritis and rheumatism. 2000;43(11):2481-2487.
12. Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A, Luberto F, Pipitone N, Salvarani C. Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. Seminars in arthritis and rheumatism. 2014;44(2):202-207. http://dx.doi.org/10.1016/j.semarthrit.2014.05. 005
13. Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis. A ten year study in the United Kingdom. Arthritis and rheumatism. 2000;43(2):414-419.
14. Nesher G, Ben-Chetrit E, Mazal B, Breuer G. The Incidence of Primary Systemic Vasculitis in Jerusalem: A 20-year Hospital-based Retrospective Study. J Rheumatol. 2016;43(6):1072-7. http://dx.doi.org/ 10.3899/jrheum.150557
15. Andrews M, Edmunds M, Campbell A, Walls J, Feehally J. Systemic vasculitis in the 1980s- Is theere are increasing incidence of Wegener's granulomatosis and microscopic polyarteritis? J Royal Coll Phys Lond. 1990; 24:284-8.
16. Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: presentation, pathology and prognosis. QJM. 1985;56(220):467-483.
17. 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.
18. Lane SE, Watts RA, Shepstone L, Scott DG. Primary systemic vasculitis: clinical features and mortality. QJM. 2005;98(2):97-111.
19. Ahn JK, Hwang JW, Lee J, Jeon CH, Cha HS, Koh EM. Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated vasculitides in Korea. Rheumatol Int. 2011;32(10):2979-2986.
20. Huang CK, Fang HC, Chou KJ, Wang JS, Lee PT, Chang TY, Hsu CY, Huang WC, Chung HM, Chen CL. Clinical features of microscopic polyangiitis: a cohort study in a southern Taiwan medical center. Acta Nephrologica. 2012;26(4):198-205. http://dx.doi.org/10.6221/AN. 2011028
21. Захарова Е.В., Яковлев В.Н., Виноградова О.В., Шейх Ж.В., Жидкова Н.В., Алексеев В.Г. ANCA-ассоциированные васкулиты с поражением легких и почек: клинико-морфологическая характеристика, лечение, исходы. Клиническая медицина. 2013;91(7):38-43. [Zakharova EV, Iakovlev VN, Vinogradova OV, Sheikh ZhV, Zhidkova NV, Alekseev VG. ANCA-associated vasculitis affecting lungs and kidneys: clinical and morphological characteristic, treatment, outcomes. Klinicheskaia meditsina. 2013;91(7):38-43 (In Russ.)].
22. Pagnoux C, Carette S, Khalidi NA, Walsh M, Hiemstra TF, Cuthbertson D, Langford C, Hoffman G, Koening CL, Monach PA, Moreland L, Mouthon L, Seo P, Specks U, Ytterberg S, Westman K, Hoglund P, Harper L, Flossman O, Luqmani R, Savage CO, Rasmussen N, de Groot K, Tesar V, Jayne D, Merkel PA, Guillevin L. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts. Clin Exp Rheumatol. 2015;33(2 Suppl 89):77-83.
23. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19-27. http://dx.doi.org/10.1097/MD.0b013e318205a4c6
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. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis rheum. 2013;65(1):270-281. http://dx.doi.org/10.1002/art.37721
25. Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M. Incidence and survival rates in Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford). 2009;48(12):1560-1565. http://dx.doi.org/ 10.1093/rheumatology/kep304
26. Miloslavsky EM, Lu N, Unizony S, Choi HK, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Tchao NK, Fervenza F, Monach PA, Specks U, Stone JH. Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients With Granulomatosis With Polyangiitis (Wegener's): Distinct Patient Subsets. Arthritis Rheumatol. 2016;68(12):2945-2952. http://dx.doi.org/10.1002/art.39812
27. Schönermarck U, Lamprecht P, Csernok E, Gross WL. Prevalence and spectrum of rheumatic diseases associated with proteinase 3 antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Rheumatology (Oxford). 2001;40(2):178-184.
28. Zycinska K, Wardyn KA, Zycinski Z, Zielonka TM. Association between clinical activity and high-resolution tomography findings in pulmonary. Physiol Pharmacol. 2008;59(Suppl. 6):833-838.
29. Pagnoux C, Guillevin L. Churg-Strauss syndrome: evidence for disease subtypes? Curr Opin Rheumatol. 2010;22(1):21-28. http://dx.doi. org/10.1097/BOR.0b013e328333390b
30. Stangou M, Asimaki A, Bamichas G, Christidou F, Zoumbaridis N, Natse T, Galanis N, Christaki P, Patakas D, Sombolos K. Factors influencing patient survival and renal function outcome in pulmonary-renal syndrome associated with ANCA (+) vasculitis: A single-center experience. J Nephrol. 2005;18(1):35-44.
31. Weidner S, Geuss S, Hafezi-Rachti S, Wonka A, Rupprecht HD. ANCA-associated vasculitis with renal involvement: an outcome analysis. Nephrol Dial Transplant. 2004;19(6):1403-1411.
32. Homma S, Matsushita H, Nakata K. Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Respirology. 2004;9(2):190-196.
33. Ando M, Miyazaki E, Ishii T, Mukai Y, Yamasue M, Fujisaki H, Ito T, Nureki S, Kumamoto T. Incidence of myeloperoxidase anti-neutrophil cytoplasmic antibody positivity and microscopic polyangitis in the course of idiopathic pulmonary fibrosis. Respir Med. 2013;107(4):608-615. http://dx.doi.org/10.1016/j.rmed.2013.01.006
34. Foulon G, Delaval P, Valeyre D, Wallaert B, Debray MP, Brauner M, Nicaise P, Cadranel J, Cottin V, Tazi A, Aubier M, Crestani B. ANCA-associated lung fibrosis: Analysis of 17 patients. Respir Med. 2008;102(10):1392-1398. http://dx.doi.org/10.1016/j.rmed.2008.04. 023
35. Puéchal X, Pagnoux C, Perrodeau É, Hamidou M, Boffa JJ, Kyndt X, Lifermann F, Papo T, Merrien D, Smail A, Delaval P, Hanrotel-Saliou C, Imbert B, Khouatra C, Lambert M, Leské C, Ly KH, Pertuiset E, Roblot P, Ruivard M, Subra JF, Viallard JF, Terrier B, Cohen P, Mouthon L, Le Jeunne C, Ravaud P, Guillevin L. Long-term outcomes of the WEGENT trial on remission-maintenance for granulomatosis with polyangiitis or microscopic polyangiitis. Arthritis Rheumatol. 2016;68(3):690-701.
36. Walsh M, Flossmann O, Berden A, Westman K, Höglund P, Stegeman C, Jayne D. Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2012;64(2):542-548. http://dx.doi.org/10.1002/art.33361
37. Sada KE, Yamamura M, Harigai M, Fujii T, Takasaki Y, Amano K, Fujimoto S, Muso E, Murakawa Y, Arimura Y, Makino H. Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study. Arthritis Res Ther. 2015;17(1):305. http://dx.doi.org/10. 1186/s13075-015-0815-y
38. Li ZY, Chang DY, Zhao MH, Chen M. Predictors of treatment resistance and relapse in ANCA-associated vasculitis: a study of 439 cases in a single Chinese center. Arthritis Rheumatol. 2014;66(7):1920-1926. http://dx.doi.org/10.1002/art.38621
39. Pierrot-Deseilligny Despujol C, Pouchot J, Pagnoux C, Coste J, Guillevin L. Predictors at diagnosis of a first Wegener's granulomatosis relapse after obtaining complete remission. Rheumatology(Oxford). 2010;49(11):2181-2190. http://dx.doi.org/10.1093/rheumatology/ keq244
40. Moiseev S, Novikov P, Jayne D, Mukhin N. End-stage renal disease in ANCA-associated vasculitis. Nephrol Dial Transplant. 2017;32(2): 248-253. http://dx.doi.org/10.1093/ndt/gfw046
41. Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, Dalhoff K, Dunogué B, Gross W, Holle J, Humbert M, Jayne D, Jennette JC, Lazor R, Mahr A, Merkel PA, Mouthon L, Sinico RA, Specks U, Vaglio A, Wechsler ME, Cordier JF, Guillevin L. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Int Med. 2015;26(7):545-553. http://dx.doi.org/10.1016/j.ejim.2015.04.022
________________________________________________
2. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr, et al. The American college of rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthr Rheum. 1990;33:1101-7.
3. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094-100.
4. Jayne D. Challenges in the management of microscopic polyangiitis. Current opinion in rheumatology. 2008;20(1):3-9. https://doi.org/ 10.1097/BOR.0b013e3282f370d1
5. Bossuyt X, Cohen Tervaert JW, Arimura Y, Blockmans D, Flores-Suárez LF, Guillevin L, Hellmich B, Jayne D, Jennette JC, Kallenberg CGM, Moiseev S, Novikov P, Radice A, Savige JA, Sinico RA, Specks U, van Paassen P, Zhao MH, Rasmussen N, Damoiseaux J, Csernok E. Position paper: Revised 2017 international consensus ont esting of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol. 2017;13(11):683-692. http://dx.doi.org/ 10.1038/nrrheum.2017.140
6. Devaney KO, Travis WD, Hoffman G, Leavitt R, Lebovics R, Fauci AS. Interpretation of head and neck biopsies in Wegener's granulomatosis. A pathologic study of 126 biopsies in 70 patients. Am J Surg Pathol. 1990;14(6):555-64.
7. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D. Development and validation of a consensus methodology for the classification of the
ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66:222-7.
8. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, Hellmich B, Holle JU, Laudien M, Little MA, Luqmani RA, Mahr A, Merkel PA, Mills J, Mooney J, Segelmark M, Tesar V, Westman K, Vaglio A, Yalçındağ N, Jayne DR, Mukhtyar C. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583-94. https://doi.org/10.1136/annrheumdis-2016-209133
9. Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, Savage C, Adu D. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87(11):671–678.
10. [Beketova TV. Asymptomatic course of lung damage in granulomatosis with polyangiitis (Wegener’s). Rheumatology Science and Practice. 2014;52(1):102-104. (In Russ.)]. http://dx.doi.org/10.14412/1995-4484-2014-102-104
11. Koldingsnes W, Nossent H. Epidemiology of Wegener's granulomatosis in northern Norway. Arthritis and rheumatism. 2000;43(11):2481-2487.
12. Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A, Luberto F, Pipitone N, Salvarani C. Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. Seminars in arthritis and rheumatism. 2014;44(2):202-207. http://dx.doi.org/10.1016/j.semarthrit.2014.05. 005
13. Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis. A ten year study in the United Kingdom. Arthritis and rheumatism. 2000;43(2):414-419.
14. Nesher G, Ben-Chetrit E, Mazal B, Breuer G. The Incidence of Primary Systemic Vasculitis in Jerusalem: A 20-year Hospital-based Retrospective Study. J Rheumatol. 2016;43(6):1072-7. http://dx.doi.org/ 10.3899/jrheum.150557
15. Andrews M, Edmunds M, Campbell A, Walls J, Feehally J. Systemic vasculitis in the 1980s- Is theere are increasing incidence of Wegener's granulomatosis and microscopic polyarteritis? J Royal Coll Phys Lond. 1990; 24:284-8.
16. Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: presentation, pathology and prognosis. QJM. 1985;56(220):467-483.
17. 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.
18. Lane SE, Watts RA, Shepstone L, Scott DG. Primary systemic vasculitis: clinical features and mortality. QJM. 2005;98(2):97-111.
19. Ahn JK, Hwang JW, Lee J, Jeon CH, Cha HS, Koh EM. Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated vasculitides in Korea. Rheumatol Int. 2011;32(10):2979-2986.
20. Huang CK, Fang HC, Chou KJ, Wang JS, Lee PT, Chang TY, Hsu CY, Huang WC, Chung HM, Chen CL. Clinical features of microscopic polyangiitis: a cohort study in a southern Taiwan medical center. Acta Nephrologica. 2012;26(4):198-205. http://dx.doi.org/10.6221/AN. 2011028
21. [Zakharova EV, Iakovlev VN, Vinogradova OV, Sheikh ZhV, Zhidkova NV, Alekseev VG. ANCA-associated vasculitis affecting lungs and kidneys: clinical and morphological characteristic, treatment, outcomes. Klinicheskaia meditsina. 2013;91(7):38-43 (In Russ.)].
22. Pagnoux C, Carette S, Khalidi NA, Walsh M, Hiemstra TF, Cuthbertson D, Langford C, Hoffman G, Koening CL, Monach PA, Moreland L, Mouthon L, Seo P, Specks U, Ytterberg S, Westman K, Hoglund P, Harper L, Flossman O, Luqmani R, Savage CO, Rasmussen N, de Groot K, Tesar V, Jayne D, Merkel PA, Guillevin L. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts. Clin Exp Rheumatol. 2015;33(2 Suppl 89):77-83.
23. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19-27. http://dx.doi.org/10.1097/MD.0b013e318205a4c6
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. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis rheum. 2013;65(1):270-281. http://dx.doi.org/10.1002/art.37721
25. Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M. Incidence and survival rates in Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford). 2009;48(12):1560-1565. http://dx.doi.org/ 10.1093/rheumatology/kep304
26. Miloslavsky EM, Lu N, Unizony S, Choi HK, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Tchao NK, Fervenza F, Monach PA, Specks U, Stone JH. Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients With Granulomatosis With Polyangiitis (Wegener's): Distinct Patient Subsets. Arthritis Rheumatol. 2016;68(12):2945-2952. http://dx.doi.org/10.1002/art.39812
27. Schönermarck U, Lamprecht P, Csernok E, Gross WL. Prevalence and spectrum of rheumatic diseases associated with proteinase 3 antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Rheumatology (Oxford). 2001;40(2):178-184.
28. Zycinska K, Wardyn KA, Zycinski Z, Zielonka TM. Association between clinical activity and high-resolution tomography findings in pulmonary. Physiol Pharmacol. 2008;59(Suppl. 6):833-838.
29. Pagnoux C, Guillevin L. Churg-Strauss syndrome: evidence for disease subtypes? Curr Opin Rheumatol. 2010;22(1):21-28. http://dx.doi. org/10.1097/BOR.0b013e328333390b
30. Stangou M, Asimaki A, Bamichas G, Christidou F, Zoumbaridis N, Natse T, Galanis N, Christaki P, Patakas D, Sombolos K. Factors influencing patient survival and renal function outcome in pulmonary-renal syndrome associated with ANCA (+) vasculitis: A single-center experience. J Nephrol. 2005;18(1):35-44.
31. Weidner S, Geuss S, Hafezi-Rachti S, Wonka A, Rupprecht HD. ANCA-associated vasculitis with renal involvement: an outcome analysis. Nephrol Dial Transplant. 2004;19(6):1403-1411.
32. Homma S, Matsushita H, Nakata K. Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Respirology. 2004;9(2):190-196.
33. Ando M, Miyazaki E, Ishii T, Mukai Y, Yamasue M, Fujisaki H, Ito T, Nureki S, Kumamoto T. Incidence of myeloperoxidase anti-neutrophil cytoplasmic antibody positivity and microscopic polyangitis in the course of idiopathic pulmonary fibrosis. Respir Med. 2013;107(4):608-615. http://dx.doi.org/10.1016/j.rmed.2013.01.006
34. Foulon G, Delaval P, Valeyre D, Wallaert B, Debray MP, Brauner M, Nicaise P, Cadranel J, Cottin V, Tazi A, Aubier M, Crestani B. ANCA-associated lung fibrosis: Analysis of 17 patients. Respir Med. 2008;102(10):1392-1398. http://dx.doi.org/10.1016/j.rmed.2008.04. 023
35. Puéchal X, Pagnoux C, Perrodeau É, Hamidou M, Boffa JJ, Kyndt X, Lifermann F, Papo T, Merrien D, Smail A, Delaval P, Hanrotel-Saliou C, Imbert B, Khouatra C, Lambert M, Leské C, Ly KH, Pertuiset E, Roblot P, Ruivard M, Subra JF, Viallard JF, Terrier B, Cohen P, Mouthon L, Le Jeunne C, Ravaud P, Guillevin L. Long-term outcomes of the WEGENT trial on remission-maintenance for granulomatosis with polyangiitis or microscopic polyangiitis. Arthritis Rheumatol. 2016;68(3):690-701.
36. Walsh M, Flossmann O, Berden A, Westman K, Höglund P, Stegeman C, Jayne D. Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2012;64(2):542-548. http://dx.doi.org/10.1002/art.33361
37. Sada KE, Yamamura M, Harigai M, Fujii T, Takasaki Y, Amano K, Fujimoto S, Muso E, Murakawa Y, Arimura Y, Makino H. Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study. Arthritis Res Ther. 2015;17(1):305. http://dx.doi.org/10. 1186/s13075-015-0815-y
38. Li ZY, Chang DY, Zhao MH, Chen M. Predictors of treatment resistance and relapse in ANCA-associated vasculitis: a study of 439 cases in a single Chinese center. Arthritis Rheumatol. 2014;66(7):1920-1926. http://dx.doi.org/10.1002/art.38621
39. Pierrot-Deseilligny Despujol C, Pouchot J, Pagnoux C, Coste J, Guillevin L. Predictors at diagnosis of a first Wegener's granulomatosis relapse after obtaining complete remission. Rheumatology(Oxford). 2010;49(11):2181-2190. http://dx.doi.org/10.1093/rheumatology/ keq244
40. Moiseev S, Novikov P, Jayne D, Mukhin N. End-stage renal disease in ANCA-associated vasculitis. Nephrol Dial Transplant. 2017;32(2): 248-253. http://dx.doi.org/10.1093/ndt/gfw046
41. Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, Dalhoff K, Dunogué B, Gross W, Holle J, Humbert M, Jayne D, Jennette JC, Lazor R, Mahr A, Merkel PA, Mouthon L, Sinico RA, Specks U, Vaglio A, Wechsler ME, Cordier JF, Guillevin L. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Int Med. 2015;26(7):545-553. http://dx.doi.org/10.1016/j.ejim.2015.04.022
Авторы
Т.В. БЕКЕТОВА
ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой», Москва, Россия
V.A. Nasonova Scientific Research Institute of Rheumatology, Moscow, Russia
ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой», Москва, Россия
________________________________________________
V.A. Nasonova Scientific Research Institute of Rheumatology, Moscow, Russia
Цель портала OmniDoctor – предоставление профессиональной информации врачам, провизорам и фармацевтам.