Myeloperoxidase–Antineutrophil Cytoplasmic Antibody (ANCA)–Positive Granulomatosis With Polyangiitis (Wegener's) Is a Clinically Distinct Subset of ANCA-Associated Vasculitis: A Retrospective Analysis of 315 Patients From a German Vasculitis Referral Center

Arthritis and Rheumatology

Volumn 68, Issue 12, 2016, Pages 2953-2963

  Schirmer, Jan H ^a   Wright, Marvin N ^b   Herrmann, Kristine ^a   Laudien, Martin ^c   Nölle, Bernhard ^c   Reinhold Keller, Eva ^a   Bremer, Jan P ^a   Moosig, Frank ^a   Holle, Julia U ^a  

^a Klinikum Bad Bramstedt   (Germany)

^b UNIVERSITY OF LÜBECK   (Germany)

^c UNIVERSITY OF KIEL   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

CYCLOPHOSPHAMIDE; MYELOBLASTIN; MYELOPEROXIDASE; NEUTROPHIL CYTOPLASMIC ANTIBODY; RITUXIMAB; IMMUNOLOGIC FACTOR; IMMUNOSUPPRESSIVE AGENT; PEROXIDASE;

ADULT; ANCA ASSOCIATED VASCULITIS; ARTICLE; CONTROLLED STUDY; DISEASE COURSE; FEMALE; HUMAN; IMMUNOSUPPRESSIVE TREATMENT; LUNG FIBROSIS; MAJOR CLINICAL STUDY; MALE; MIDDLE AGED; MORTALITY; OUTCOME ASSESSMENT; PHENOTYPE; PRIORITY JOURNAL; RECURRENCE RISK; SUBGLOTTIC STENOSIS; SURVIVAL RATE; WEGENER GRANULOMATOSIS; ADOLESCENT; AGE DISTRIBUTION; AGED; CASE CONTROL STUDY; COMPLICATION; EYE DISEASES; GERMANY; GRANULOMATOSIS WITH POLYANGIITIS; IMMUNOLOGY; KIDNEY DISEASES; LARYNGOSTENOSIS; OTORHINOLARYNGOLOGIC DISEASES; PERIPHERAL NERVOUS SYSTEM DISEASES; PROPORTIONAL HAZARDS MODEL; RECURRENT DISEASE; RETROSPECTIVE STUDY; YOUNG ADULT;

ADOLESCENT; ADULT; AGE DISTRIBUTION; AGED; ANTIBODIES, ANTINEUTROPHIL CYTOPLASMIC; CASE-CONTROL STUDIES; CYCLOPHOSPHAMIDE; EYE DISEASES; FEMALE; GERMANY; GRANULOMATOSIS WITH POLYANGIITIS; HUMANS; IMMUNOLOGIC FACTORS; IMMUNOSUPPRESSIVE AGENTS; KIDNEY DISEASES; LARYNGOSTENOSIS; MALE; MIDDLE AGED; MYELOBLASTIN; OTORHINOLARYNGOLOGIC DISEASES; PERIPHERAL NERVOUS SYSTEM DISEASES; PEROXIDASE; PROPORTIONAL HAZARDS MODELS; RECURRENCE; RETROSPECTIVE STUDIES; RITUXIMAB; SURVIVAL RATE; YOUNG ADULT;

EID: 84997834612     PISSN: 23265191     EISSN: 23265205     Source Type: Journal    
DOI: 10.1002/art.39786     Document Type: Article

References (48)

1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 2013;65:1–11.
2. Pagnoux C, Wolter NE. Vasculitis of the upper airways. Swiss Med Wkly 2012;142:w13541.
3. Alam DS, Seth R, Sindwani R, Woodson EA, Rajasekaran K. Upper airway manifestations of granulomatosis with polyangiitis. Cleve Clin J Med 2012;79 Suppl 3:S16–21.
4. Holle JU, Gross WL, Latza U, Nölle B, Ambrosch P, Heller M, et al. Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades. Arthritis Rheum 2011;63:257–66.
5. Schirmer JH, Wright MN, Vonthein R, Herrmann K, Nolle B, Both M, et al. Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort. Rheumatology (Oxford) 2016;55:71–9.
6. Flossmann O, Berden A, de Groot K, Hagen C, Harper L, Heijl C, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 2011;70:488–94.
7. 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;40:178–84.
8. Lionaki S, Blyth ER, Hogan SL, Hu Y, Senior BA, Jennette CE, et al. Classification of antineutrophil cytoplasmic autoantibody vasculitides: the role of antineutrophil cytoplasmic autoantibody specificity for myeloperoxidase or proteinase 3 in disease recognition and prognosis. Arthritis Rheum 2012;64:3452–62.
9. Robson J, Doll H, Suppiah R, Flossmann O, Harper L, Höglund P, et al. Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials. Ann Rheum Dis 2015;74:177–84.
10. Mukhtyar C, Flossmann O, Hellmich B, Bacon P, Cid M, Cohen-Tervaert JW, et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force. Ann Rheum Dis 2008;67:1004–10.
11. Mohammad AJ, Segelmark M. A population-based study showing better renal prognosis for proteinase 3 antineutrophil cytoplasmic antibody (ANCA)-associated nephritis versus myeloperoxidase ANCA-associated nephritis. J Rheumatol 2014;41:1366–73.
12. Unizony S, Villarreal M, Miloslavsky EM, Lu N, Merkel PA, Spiera R, et al. Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type. Ann Rheum Dis 2016;75:1166–9.
13. Watts RA, Scott DG. ANCA vasculitis: to lump or split? Why we should study MPA and GPA separately. Rheumatology (Oxford) 2012;51:2115–7.
14. Lyons PA, Rayner TF, Trivedi S, Holle JU, Watts RA, Jayne DR, et al. Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med 2012;367:214–23.
15. Ono N, Niiro H, Ueda A, Sawabe T, Nishizaka H, Furugo I, et al. Characteristics of MPO-ANCA-positive granulomatosis with polyangiitis: a retrospective multi-center study in Japan. Rheumatol Int 2015;35:555–9.
16. Ikeda S, Arita M, Misaki K, Kashiwagi Y, Ito Y, Yamada H, et al. Comparative investigation of respiratory tract involvement in granulomatosis with polyangiitis between PR3-ANCA positive and MPO-ANCA positive cases: a retrospective cohort study. BMC Pulm Med 2015;15:78.
17. Tsuchida Y, Shibuya M, Shoda H, Sumitomo S, Kubo K, Setoguchi K, et al. Characteristics of granulomatosis with polyangiitis patients in Japan. Mod Rheumatol 2015;25:219–23.
18. Kim HW, Kim JW, Im CH, Shin KC, Lee EY, Lee EB, et al. The clinicopathologic characteristics of granulomatosis with polyangiitis (Wegener's): a retrospective study of 45 patients in Korea. Mod Rheumatol 2013;23:864–71.
19. Katsuyama T, Sada KE, Makino H. Current concept and epidemiology of systemic vasculitides. Allergol Int 2014;63:505–13.
20. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. 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.
21. Abdulkader R, Lane SE, Scott DG, Watts RA. Classification of vasculitis: EMA classification using CHCC 2012 definitions. Ann Rheum Dis 2013;72:1888.
22. Reinhold-Keller E, Beuge N, Latza U, de Groot K, Rudert H, Nolle B, et al. An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 2000;43:1021–32.
23. Hellmich B, Flossmann O, Gross WL, Bacon P, Cohen-Tervaert JW, Guillevin L, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis 2007;66:605–17.
24. Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM 1994;87:671–8.
25. Exley AR, Bacon PA, Luqmani RA, Kitas GD, Gordon C, Savage CO, et al. Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum 1997;40:371–80.
26. The Wegener's Granulomatosis Etanercept Trial Research Group. Limited versus severe Wegener's granulomatosis: baseline data on patients in the Wegener's Granulomatosis Etanercept Trial. Arthritis Rheum 2003;48:2299–309.
27. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009;68:310–7.
28. Benjamini Y, Hochberg Y. Controlling the false discovery rate: a practical and powerful approach to multiple testing. J R Stat Soc Ser B 1995;57:289–300.
29. Cox DR. Regression models and life-tables. J R Stat Soc Ser B 1972;34:187–220.
30. Corral-Gudino L, Borao-Cengotita-Bengoa M, Del Pino-Montes J, Lerma-Márquez JL. Overall survival, renal survival and relapse in patients with microscopic polyangiitis: a systematic review of current evidence. Rheumatology (Oxford) 2011;50:1414–23.
31. Furuta S, Chaudhry AN, Hamano Y, Fujimoto S, Nagafuchi H, Makino H, et al. Comparison of phenotype and outcome in microscopic polyangiitis between Europe and Japan. J Rheumatol 2014;41:325–33.
32. Weidner S, Geuss S, Hafezi-Rachti S, Wonka A, Rupprecht HD. ANCA-associated vasculitis with renal involvement: an outcome analysis. Nephrol Dial Transplant 2004;19:1403–11.
33. Franssen CF, Gans RO, Arends B, Hageluken C, ter Wee PM, Gerlag PG, et al. Differences between anti-myeloperoxidase- and anti-proteinase 3-associated renal disease. Kidney Int 1995;47:193–9.
34. Franssen C, Gans R, Kallenberg C, Hageluken C, Hoorntje S. Disease spectrum of patients with antineutrophil cytoplasmic autoantibodies of defined specificity: distinct differences between patients with anti-proteinase 3 and anti-myeloperoxidase autoantibodies. J Intern Med 1998;244:209–16.
35. Waxman J, Bose WJ. Laryngeal manifestations of Wegener's granulomatosis: case reports and review of the literature. J Rheumatol 1986;13:408–11.
36. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488–98.
37. Lebovics RS, Hoffman GS, Leavitt RY, Kerr GS, Travis WD, Kammerer W, et al. The management of subglottic stenosis in patients with Wegener's granulomatosis. Laryngoscope 1992;102:1341–5.
38. Hoffman GS, Thomas-Golbanov CK, Chan J, Akst LM, Eliachar I. Treatment of subglottic stenosis, due to Wegener's granulomatosis, with intralesional corticosteroids and dilation. J Rheumatol 2003;30:1017–21.
39. Rottem M, Fauci AS, Hallahan CW, Kerr GS, Lebovics R, Leavitt RY, et al. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr 1993;122:26–31.
40. Terrier B, Dechartres A, Girard C, Jouneau S, Kahn JE, Dhote R, et al. Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis: results from a multicentre experience. Rheumatology (Oxford) 2015;54:1852–7.
41. Comarmond C, Crestani B, Tazi A, Hervier B, Adam-Marchand S, Nunes H, et al. Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature. Medicine (Baltimore) 2014;93:340–9.
42. Ando Y, Okada F, Matsumoto S, Mori H. Thoracic manifestation of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-related disease: CT findings in 51 patients. J Comput Assist Tomogr 2004;28:710–6.
43. Hogan SL, Falk RJ, Chin H, Cai J, Jennette CE, Jennette JC, et al. Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. Ann Intern Med 2005;143:621–31.
44. Puéchal X, Pagnoux C, Perrodeau É, Hamidou M, Boffa JJ, Kyndt X, et al. Long-term outcomes among participants in the WEGENT Trial of remission-maintenance therapy for granulomatosis with polyangiitis (Wegener's) or microscopic polyangiitis. Arthritis Rheumatol 2016;68:690–701.
45. Booth AD, Almond MK, Burns A, Ellis P, Gaskin G, Neild GH, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis 2003;41:776–84.
46. Casian A, Jayne D. Management of alveolar hemorrhage in lung vasculitides. Semin Respir Crit Care Med 2011;32:335–45.
47. Samson M, Puéchal X, Devilliers H, Ribi C, Cohen P, Bienvenu B, et al. Mononeuritis multiplex predicts the need for immunosuppressive or immunomodulatory drugs for EGPA, PAN and MPA patients without poor-prognosis factors. Autoimmun Rev 2014;13:945–53.
48. Pagnoux C, Carette S, Khalidi NA, Walsh M, Hiemstra TF, Cuthbertson D, et al. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts. Clin Exp Rheumatol 2015;33 Suppl 89:S77–83.