Skip to main content
Erschienen in: Zeitschrift für Rheumatologie 1/2009

01.02.2009 | CME Weiterbildung • Zertifizierte Fortbildung

Wegener-Granulomatose und mikroskopische Polyangiitis

verfasst von: PD Dr. K. de Groot, E. Reinhold-Keller

Erschienen in: Zeitschrift für Rheumatologie | Ausgabe 1/2009

Einloggen, um Zugang zu erhalten

Zusammenfassung

Die Wegener-Granulomatose (WG) und mikroskopische Polyangiitis (MPA) sind primäre systemische Kleingefäßvaskulitiden, die mit dem Nachweis eines C-ANCA/PR3-ANCA bei der WG und P-ANCA/MPO-ANCA bei der MPA assoziiert sind. Klinisch führend sind Beteiligungen des oberen (nur bei WG) und unteren Respirationstraktes und der Nieren. Die Diagnostik zur Festlegung von Krankheitsausdehnung und -aktivität ist interdisziplinär. Die Therapie erfolgt stadien- und aktivitätsadaptiert. Eine Remissionsinduktion bei der „Early-systemic-WG/MPA“ kann mit Methotrexat (MTX) erfolgen. Bei schwer generalisierten Verläufen ist Cyclophosphamid (CYC) unverzichtbar, bei rapid progressiver Glomerulonephritis (RPGN) mit zusätzlicher Plasmapheresebehandlung. Nach etwa 3- bis 6-monatiger CYC-Therapie wird auf eine remissionserhaltende Therapie mit Azathioprin umgestellt. Alternativen sind Leflunomid, Mycophenolat oder MTX (Kreatinin <150 µmol/l). Alter ≥50 Jahre, Lungenbeteiligung oder Nierenfunktionseinschränkung bei Diagnose sind mit einer erhöhten Mortalität assoziiert. Die Rezidivrate liegt um 50% in 5 Jahren, bei der WG höher als bei der MPA.
Literatur
1.
Zurück zum Zitat Wegener F (1936) Über generalisierte septische Gefäßerkrankung. Verh Dtsch Ges Path 29:202–210 Wegener F (1936) Über generalisierte septische Gefäßerkrankung. Verh Dtsch Ges Path 29:202–210
2.
Zurück zum Zitat Wohlwill F (1923) Über die nur mikroskopisch erkennbare Form der Periarteriitis nodosa. Virchows Archiv für Pathologische Anatomie und Physiologie und für Klinische Medizin 246:377–411CrossRef Wohlwill F (1923) Über die nur mikroskopisch erkennbare Form der Periarteriitis nodosa. Virchows Archiv für Pathologische Anatomie und Physiologie und für Klinische Medizin 246:377–411CrossRef
3.
Zurück zum Zitat Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides: The proposal of an International Consensus Conference. Arthritis Rheum 37:187–192PubMedCrossRef Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides: The proposal of an International Consensus Conference. Arthritis Rheum 37:187–192PubMedCrossRef
4.
Zurück zum Zitat Leavitt RY, Fauci AS, Bloch DA et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101–1107PubMed Leavitt RY, Fauci AS, Bloch DA et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101–1107PubMed
5.
Zurück zum Zitat Herlyn K, Hellmich B, Gross WL, Reinhold-Keller E (2008) Stable incidence of systemic vasculitides in Schleswig-Holstein, Germany. Dtsch Arztebl Int 105:355–361PubMed Herlyn K, Hellmich B, Gross WL, Reinhold-Keller E (2008) Stable incidence of systemic vasculitides in Schleswig-Holstein, Germany. Dtsch Arztebl Int 105:355–361PubMed
6.
Zurück zum Zitat Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R, Gross WL (2005) Stable incidence of primary systemic vasculitides over five years: results from the German vasculitis register. Arthritis Rheum 53:93–99PubMedCrossRef Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R, Gross WL (2005) Stable incidence of primary systemic vasculitides over five years: results from the German vasculitis register. Arthritis Rheum 53:93–99PubMedCrossRef
7.
Zurück zum Zitat Mohammad AJ, Jacobsson LT, Mahr AD et al (2007) Prevalence of Wegener’s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford) 46:1329–1337 Mohammad AJ, Jacobsson LT, Mahr AD et al (2007) Prevalence of Wegener’s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford) 46:1329–1337
8.
Zurück zum Zitat Watts RA, Scott DG, Jayne DR et al (2008) Renal vasculitis in Japan and the UK–are there differences in epidemiology and clinical phenotype? Nephrol Dial Transplant 23:3928–3931PubMedCrossRef Watts RA, Scott DG, Jayne DR et al (2008) Renal vasculitis in Japan and the UK–are there differences in epidemiology and clinical phenotype? Nephrol Dial Transplant 23:3928–3931PubMedCrossRef
9.
Zurück zum Zitat Ormerod A, Cook M (2008) Epidemiology of primary systemic vasculitis in the australian capital territory and south-eastern New South Wales. Intern Med J [Epub ahead of print Sep 2] Ormerod A, Cook M (2008) Epidemiology of primary systemic vasculitis in the australian capital territory and south-eastern New South Wales. Intern Med J [Epub ahead of print Sep 2]
10.
Zurück zum Zitat Chen M, Yu F, Zhang Y, Zhao MH (2008) Antineutrophil cytoplasmic autoantibody-associated vasculitis in older patients. Medicine (Baltimore) 87:203–209 Chen M, Yu F, Zhang Y, Zhao MH (2008) Antineutrophil cytoplasmic autoantibody-associated vasculitis in older patients. Medicine (Baltimore) 87:203–209
11.
Zurück zum Zitat van der Woude FJ, Rasmussen N, Lobatto S et al (1985) Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet 1:425–429 van der Woude FJ, Rasmussen N, Lobatto S et al (1985) Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet 1:425–429
12.
Zurück zum Zitat Gross WL, Lüdemann G, Kiefer G, Lehmann H (1986) Anticytoplasmic antibodies in Wegener’s granulomatosis. Lancet 328:806CrossRef Gross WL, Lüdemann G, Kiefer G, Lehmann H (1986) Anticytoplasmic antibodies in Wegener’s granulomatosis. Lancet 328:806CrossRef
13.
Zurück zum Zitat Nölle B, Specks U, Lüdemann J et al (1989) Anticytoplasmic autoantibodies: Their immunodiagnostic value in wegener’s granulomatosis. Ann Intern Med 111:28–40PubMed Nölle B, Specks U, Lüdemann J et al (1989) Anticytoplasmic autoantibodies: Their immunodiagnostic value in wegener’s granulomatosis. Ann Intern Med 111:28–40PubMed
14.
Zurück zum Zitat Hagen EC, Daha MR, Hermans J et al (1998) Diagnostic value of standardized assays for antineutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. Kidney Int 53:743–753PubMedCrossRef Hagen EC, Daha MR, Hermans J et al (1998) Diagnostic value of standardized assays for antineutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. Kidney Int 53:743–753PubMedCrossRef
15.
Zurück zum Zitat Schonermarck U, Lamprecht P, Csernok E, Gross WL (2001) Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Rheumatology (Oxford) 40:178–184 Schonermarck U, Lamprecht P, Csernok E, Gross WL (2001) Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Rheumatology (Oxford) 40:178–184
16.
Zurück zum Zitat Gross WL, Trabandt A, Csernok E (1998) Pathogenesis of Wegener’s granulomatosis. Ann Med Int 149:280–286 Gross WL, Trabandt A, Csernok E (1998) Pathogenesis of Wegener’s granulomatosis. Ann Med Int 149:280–286
17.
Zurück zum Zitat Csernok E, Lüdemann J, Gross WL, Bainton DF (1990) Ultrastructural localization of proteinase 3, the target antigen of anti-cytoplasmic antibodies circulating in Wegener’s granulomatosis. Am J Pathol 137:1113–1120PubMed Csernok E, Lüdemann J, Gross WL, Bainton DF (1990) Ultrastructural localization of proteinase 3, the target antigen of anti-cytoplasmic antibodies circulating in Wegener’s granulomatosis. Am J Pathol 137:1113–1120PubMed
18.
Zurück zum Zitat Falk RJ, Terrel RS, Charles LA, Jenette JC (1990) Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci U S A 87:4115–4119PubMedCrossRef Falk RJ, Terrel RS, Charles LA, Jenette JC (1990) Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci U S A 87:4115–4119PubMedCrossRef
19.
Zurück zum Zitat Schreiber A, Luft FC, Kettritz R (2004) Membrane proteinase 3 expression and ANCA-induced neutrophil activation. Kidney Int 65:2172–2183PubMedCrossRef Schreiber A, Luft FC, Kettritz R (2004) Membrane proteinase 3 expression and ANCA-induced neutrophil activation. Kidney Int 65:2172–2183PubMedCrossRef
20.
Zurück zum Zitat Schreiber A, Busjahn A, Luft FC, Kettritz R (2003) Membrane expression of proteinase 3 is genetically determined. J Am Soc Nephrol 14:68–75PubMedCrossRef Schreiber A, Busjahn A, Luft FC, Kettritz R (2003) Membrane expression of proteinase 3 is genetically determined. J Am Soc Nephrol 14:68–75PubMedCrossRef
21.
Zurück zum Zitat Witko-Sarsat V, Lesavre P, Lopez S et al (1999) A large subset of neutrophils expressing membrane proteinase 3 is a risk factor for vasculitis and rheumatoid arthritis. J Am Soc Nephrol 10:1224–1233PubMed Witko-Sarsat V, Lesavre P, Lopez S et al (1999) A large subset of neutrophils expressing membrane proteinase 3 is a risk factor for vasculitis and rheumatoid arthritis. J Am Soc Nephrol 10:1224–1233PubMed
22.
Zurück zum Zitat Kettritz R, Choi M, Butt W et al (2002) Phosphatidylinositol 3-kinase controls antineutrophil cytoplasmic antibodies-induced respiratory burst in human neutrophils. J Am Soc Nephrol 13:1740–1749PubMedCrossRef Kettritz R, Choi M, Butt W et al (2002) Phosphatidylinositol 3-kinase controls antineutrophil cytoplasmic antibodies-induced respiratory burst in human neutrophils. J Am Soc Nephrol 13:1740–1749PubMedCrossRef
23.
Zurück zum Zitat Kettritz R, Schreiber A, Luft FC, Haller H (2001) Role of mitogen-activated protein kinases in activation of human neutrophils by antineutrophil cytoplasmic antibodies. J Am Soc Nephrol 12:37–46PubMed Kettritz R, Schreiber A, Luft FC, Haller H (2001) Role of mitogen-activated protein kinases in activation of human neutrophils by antineutrophil cytoplasmic antibodies. J Am Soc Nephrol 12:37–46PubMed
24.
Zurück zum Zitat Morgan MD, Harper L, Williams J, Savage C (2006) Anti-neutrophil cytoplasm-associated glomerulonephritis. J Am Soc Nephrol 17:1224–1234PubMedCrossRef Morgan MD, Harper L, Williams J, Savage C (2006) Anti-neutrophil cytoplasm-associated glomerulonephritis. J Am Soc Nephrol 17:1224–1234PubMedCrossRef
25.
Zurück zum Zitat Pendergraft WF 3rd, Preston GA, Shah RR et al (2004) Autoimmunity is triggered by cPR-3(105–201), a protein complementary to human autoantigen proteinase-3. Nat Med 10:72–79PubMedCrossRef Pendergraft WF 3rd, Preston GA, Shah RR et al (2004) Autoimmunity is triggered by cPR-3(105–201), a protein complementary to human autoantigen proteinase-3. Nat Med 10:72–79PubMedCrossRef
26.
Zurück zum Zitat Kain R, Exner M, Brandes R et al (2008) Molecular mimicry in pauci-immune focal necrotizing glomerulonephritis. Nat Med 14:1088–1096PubMedCrossRef Kain R, Exner M, Brandes R et al (2008) Molecular mimicry in pauci-immune focal necrotizing glomerulonephritis. Nat Med 14:1088–1096PubMedCrossRef
27.
Zurück zum Zitat Xiao H, Heeringa P, Hu P et al (2002) Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest 110:955–963PubMed Xiao H, Heeringa P, Hu P et al (2002) Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest 110:955–963PubMed
28.
Zurück zum Zitat Schlieben DJ, Korbet SM, Kimura RE et al (2005) Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs. Am J Kidney Dis 45:758–761PubMedCrossRef Schlieben DJ, Korbet SM, Kimura RE et al (2005) Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs. Am J Kidney Dis 45:758–761PubMedCrossRef
29.
Zurück zum Zitat Pfister H, Ollert M, Frohlich LF et al (2004) Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo. Blood 104:1411–1418PubMedCrossRef Pfister H, Ollert M, Frohlich LF et al (2004) Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo. Blood 104:1411–1418PubMedCrossRef
30.
Zurück zum Zitat Komocsi A, Lamprecht P, Csernok E et al (2002) Peripheral blood and granuloma CD4(+)CD28(–) T cells are a major source of interferon-gamma and tumor necrosis factor-alpha in Wegener’s granulomatosis. Am J Pathol 160:1717–1724PubMed Komocsi A, Lamprecht P, Csernok E et al (2002) Peripheral blood and granuloma CD4(+)CD28(–) T cells are a major source of interferon-gamma and tumor necrosis factor-alpha in Wegener’s granulomatosis. Am J Pathol 160:1717–1724PubMed
31.
Zurück zum Zitat Lamprecht P (2005) Off balance: T-cells in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Clin Exp Immunol 141:201–210PubMedCrossRef Lamprecht P (2005) Off balance: T-cells in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Clin Exp Immunol 141:201–210PubMedCrossRef
32.
Zurück zum Zitat Voswinkel J, Mueller A, Kraemer JA et al (2006) B lymphocyte maturation in Wegener’s granulomatosis: a comparative analysis of VH genes from endonasal lesions. Ann Rheum Dis 65:859–864PubMedCrossRef Voswinkel J, Mueller A, Kraemer JA et al (2006) B lymphocyte maturation in Wegener’s granulomatosis: a comparative analysis of VH genes from endonasal lesions. Ann Rheum Dis 65:859–864PubMedCrossRef
33.
Zurück zum Zitat Agard C, Mouthon L, Mahr A, Guillevin L (2003) Microscopic polyangiitis and polyarteritis nodosa: how and when do they start? Arthritis Rheum 49:709–715PubMedCrossRef Agard C, Mouthon L, Mahr A, Guillevin L (2003) Microscopic polyangiitis and polyarteritis nodosa: how and when do they start? Arthritis Rheum 49:709–715PubMedCrossRef
34.
Zurück zum Zitat Guillevin L, Cohen P, Mahr A et al (2003) Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients. Arthritis Rheum 49:93–100PubMedCrossRef Guillevin L, Cohen P, Mahr A et al (2003) Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients. Arthritis Rheum 49:93–100PubMedCrossRef
35.
Zurück zum Zitat Watts R, Lane S, Hanslik T et al (2006) Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 65:946–948 Watts R, Lane S, Hanslik T et al (2006) Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 65:946–948
36.
Zurück zum Zitat Savage COS, Winearis CG, Evans DJ et al (1985) Microscopic polyarteriitis: presentation, pathology and prognosis. QJM 56:467–483PubMed Savage COS, Winearis CG, Evans DJ et al (1985) Microscopic polyarteriitis: presentation, pathology and prognosis. QJM 56:467–483PubMed
37.
Zurück zum Zitat Jayne D (2008) Challenges in the management of microscopic polyangiitis: past, present and future. Curr Opin Rheumatol 20:3–9PubMedCrossRef Jayne D (2008) Challenges in the management of microscopic polyangiitis: past, present and future. Curr Opin Rheumatol 20:3–9PubMedCrossRef
38.
Zurück zum Zitat Homma S, Matsushita H, Nakata K (2004) Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Respirology Carlton Vic 9:190–196 Homma S, Matsushita H, Nakata K (2004) Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Respirology Carlton Vic 9:190–196
39.
Zurück zum Zitat Mukhtyar C, Guillevin L, Cid MC et al (2008) EULAR Recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis [Epub April 15] Mukhtyar C, Guillevin L, Cid MC et al (2008) EULAR Recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis [Epub April 15]
40.
Zurück zum Zitat Luqmani RA, Bacon PA, Moots RJ et al (1994) Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM 87:671–678PubMed Luqmani RA, Bacon PA, Moots RJ et al (1994) Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM 87:671–678PubMed
41.
Zurück zum Zitat de Groot K, Gross WL, Herlyn K, Reinhold-Keller E (2001) Development and validation of a disease extent index for Wegener’s granulomatosis. Clin Nephrol 55:31–38 de Groot K, Gross WL, Herlyn K, Reinhold-Keller E (2001) Development and validation of a disease extent index for Wegener’s granulomatosis. Clin Nephrol 55:31–38
42.
Zurück zum Zitat Exley AR, Bacon PA, Luqmani RA et al (1997) Development and initial validation of the Vasvulitis Damage Index (VDI) for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum 40:371–380PubMedCrossRef Exley AR, Bacon PA, Luqmani RA et al (1997) Development and initial validation of the Vasvulitis Damage Index (VDI) for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum 40:371–380PubMedCrossRef
43.
Zurück zum Zitat Tatsis E, Reinhold-Keller E, Steindorf K et al (1999) Wegener’s granulomatosis associated with renal cell carcinoma. Arthritis Rheum 42:751–756PubMedCrossRef Tatsis E, Reinhold-Keller E, Steindorf K et al (1999) Wegener’s granulomatosis associated with renal cell carcinoma. Arthritis Rheum 42:751–756PubMedCrossRef
44.
Zurück zum Zitat Choi HK, Liu S, Merkel PA et al (2001) Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on antimyeloperoxidase antibodies. J Rheumatol 28:1584–1590PubMed Choi HK, Liu S, Merkel PA et al (2001) Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on antimyeloperoxidase antibodies. J Rheumatol 28:1584–1590PubMed
45.
Zurück zum Zitat Csernok E, Holle J, Hellmich B et al (2004) Evaluation of capture ELISA for detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 in Wegener’s granulomatosis: first results from a multicentre study. Rheumatology (Oxford) 43:174–180 Csernok E, Holle J, Hellmich B et al (2004) Evaluation of capture ELISA for detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 in Wegener’s granulomatosis: first results from a multicentre study. Rheumatology (Oxford) 43:174–180
46.
Zurück zum Zitat Hellmich B, Csernok E, Fredenhagen G, Gross WL (2007) A novel high sensitivity ELISA for detection of antineutrophil cytoplasm antibodies against proteinase-3. Clin Exp Rheumatol 25:S1–S5PubMed Hellmich B, Csernok E, Fredenhagen G, Gross WL (2007) A novel high sensitivity ELISA for detection of antineutrophil cytoplasm antibodies against proteinase-3. Clin Exp Rheumatol 25:S1–S5PubMed
47.
Zurück zum Zitat Finkielman JD, Merkel PA, Schroeder D et al (2007) Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Ann Intern Med 147:611–619PubMed Finkielman JD, Merkel PA, Schroeder D et al (2007) Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Ann Intern Med 147:611–619PubMed
48.
Zurück zum Zitat Cordier JF (1996) A lung biopsy is unnecessary in the management of ANCA-positive patients with chest-roentgenographic abnormalities. Sarcoidosis Vasc Diffuse Lung Dis 13:235–237PubMed Cordier JF (1996) A lung biopsy is unnecessary in the management of ANCA-positive patients with chest-roentgenographic abnormalities. Sarcoidosis Vasc Diffuse Lung Dis 13:235–237PubMed
49.
Zurück zum Zitat Komocsi A, Reuter M, Heller M et al (2003) Active disease and residual damage in treated Wegener’s granulomatosis: an observational study using pulmonary high-resolution computed tomography. Eur Radiol 13:36–42PubMed Komocsi A, Reuter M, Heller M et al (2003) Active disease and residual damage in treated Wegener’s granulomatosis: an observational study using pulmonary high-resolution computed tomography. Eur Radiol 13:36–42PubMed
50.
Zurück zum Zitat Murphy JM, Gomez-Anson B, Gillard JH et al (1999) Wegener granulomatosis: MR imaging findings in brain and meninges. Radiology 213:794–799PubMed Murphy JM, Gomez-Anson B, Gillard JH et al (1999) Wegener granulomatosis: MR imaging findings in brain and meninges. Radiology 213:794–799PubMed
51.
Zurück zum Zitat Muhle C, Reinhold-Keller E, Richter C et al (1997) MRI of the nasal cavity, paranasal sinuses and orbit in Wegener’s granulomatosis. Eur Radiol 7:566–570PubMedCrossRef Muhle C, Reinhold-Keller E, Richter C et al (1997) MRI of the nasal cavity, paranasal sinuses and orbit in Wegener’s granulomatosis. Eur Radiol 7:566–570PubMedCrossRef
52.
Zurück zum Zitat Hauer HA, Bajema IM, Van Houwelingen HC et al (2002) Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int 62:1732–1742PubMedCrossRef Hauer HA, Bajema IM, Van Houwelingen HC et al (2002) Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int 62:1732–1742PubMedCrossRef
53.
Zurück zum Zitat de Lind van Wijngaarden RA, Hauer HA, Wolterbeek R et al (2006) Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement. J Am Soc Nephrol 17:2264–2274CrossRef de Lind van Wijngaarden RA, Hauer HA, Wolterbeek R et al (2006) Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement. J Am Soc Nephrol 17:2264–2274CrossRef
54.
Zurück zum Zitat Hellmich B, Flossmann O, Gross WL et al (2007) EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis 66:605–617PubMedCrossRef Hellmich B, Flossmann O, Gross WL et al (2007) EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis 66:605–617PubMedCrossRef
55.
Zurück zum Zitat Rasmussen N, Jayne DRW, Abramowicz D et al (1995) European therapeutic trials in ANCA-associated systemic vasculitis: disease scoring, consensus regimens and proposed clinical trials. Clin Exp Immunol 101:29–34CrossRef Rasmussen N, Jayne DRW, Abramowicz D et al (1995) European therapeutic trials in ANCA-associated systemic vasculitis: disease scoring, consensus regimens and proposed clinical trials. Clin Exp Immunol 101:29–34CrossRef
56.
Zurück zum Zitat Jayne D (2001) Update on the European Vasculitis Study Group Trials. Curr Opin Rheumatol 13:48–55PubMedCrossRef Jayne D (2001) Update on the European Vasculitis Study Group Trials. Curr Opin Rheumatol 13:48–55PubMedCrossRef
57.
Zurück zum Zitat Reinhold-Keller E, Beuge N, Latza U et al (2000) An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 43:1021–1032PubMedCrossRef Reinhold-Keller E, Beuge N, Latza U et al (2000) An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 43:1021–1032PubMedCrossRef
58.
Zurück zum Zitat Reinhold-Keller E, de Groot K, Rudert H et al (1996) Response to trimethroprim-sulfamethoxazole (T/S) in Wegener’s granulomatosis (WG) depends on the phase of disease. Q J Med 89:15–23 Reinhold-Keller E, de Groot K, Rudert H et al (1996) Response to trimethroprim-sulfamethoxazole (T/S) in Wegener’s granulomatosis (WG) depends on the phase of disease. Q J Med 89:15–23
59.
Zurück zum Zitat de Groot K, Rasmussen N, Bacon PA et al (2005) Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 52:2461–2469CrossRef de Groot K, Rasmussen N, Bacon PA et al (2005) Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 52:2461–2469CrossRef
60.
Zurück zum Zitat Fauci AS, Wolff SM (1973) Wegener’s granulomatosis: studies in eighteen patients and a review of the literature. Medicine (Baltimore) 52:535–561 Fauci AS, Wolff SM (1973) Wegener’s granulomatosis: studies in eighteen patients and a review of the literature. Medicine (Baltimore) 52:535–561
61.
Zurück zum Zitat Fauci AS, Haynes BF, Katz P, Wolff SM (1983) Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98:76–85PubMed Fauci AS, Haynes BF, Katz P, Wolff SM (1983) Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98:76–85PubMed
62.
Zurück zum Zitat Hoffman GS, Kerr GS, Leavitt RY et al (1992) Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 116:488–498PubMed Hoffman GS, Kerr GS, Leavitt RY et al (1992) Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 116:488–498PubMed
63.
Zurück zum Zitat de Groot K, Adu D, Savage COS (2001) The value of pulse cyclophosphamide in ANCA-associated vasculitis: meta-analysis and critical review. Nephrol Dial Transplant 16:2018–2027CrossRef de Groot K, Adu D, Savage COS (2001) The value of pulse cyclophosphamide in ANCA-associated vasculitis: meta-analysis and critical review. Nephrol Dial Transplant 16:2018–2027CrossRef
64.
Zurück zum Zitat WGET Research Group (2005) Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J Med 352:351–361CrossRef WGET Research Group (2005) Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J Med 352:351–361CrossRef
65.
Zurück zum Zitat Westman KWA, Bygren PG, Olsson H et al (1998) Relapse rate, renal survival and cancer morbidity in patients with Wegener‘ s granulomatosis or microscopic polyangiitis with renal involvement. JASN 9:842–852PubMed Westman KWA, Bygren PG, Olsson H et al (1998) Relapse rate, renal survival and cancer morbidity in patients with Wegener‘ s granulomatosis or microscopic polyangiitis with renal involvement. JASN 9:842–852PubMed
66.
Zurück zum Zitat Booth AD, Almond MK, Burns A et al (2003) Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis 41:776–784PubMedCrossRef Booth AD, Almond MK, Burns A et al (2003) Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis 41:776–784PubMedCrossRef
67.
Zurück zum Zitat Weidner S, Geuss S, Hafezi-Rachti S et al (2004) ANCA-associated vasculitis with renal involvement: an outcome analysis. Nephrol Dial Transplant 19:1403–1411PubMedCrossRef Weidner S, Geuss S, Hafezi-Rachti S et al (2004) ANCA-associated vasculitis with renal involvement: an outcome analysis. Nephrol Dial Transplant 19:1403–1411PubMedCrossRef
68.
Zurück zum Zitat Pusey CD, Rees AJ, Evans DJ et al (1991) Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies. Kidney Int 40:757–763PubMedCrossRef Pusey CD, Rees AJ, Evans DJ et al (1991) Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies. Kidney Int 40:757–763PubMedCrossRef
69.
Zurück zum Zitat Jayne DR, Gaskin G, Rasmussen N et al (2007) Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 18:2180–2188PubMedCrossRef Jayne DR, Gaskin G, Rasmussen N et al (2007) Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 18:2180–2188PubMedCrossRef
70.
Zurück zum Zitat Flossmann O, de Groot K (2007) When should patients with ANCA-associated vasculitis be switched from induction to maintenance therapy? Nature Clin Prac 3:236–237CrossRef Flossmann O, de Groot K (2007) When should patients with ANCA-associated vasculitis be switched from induction to maintenance therapy? Nature Clin Prac 3:236–237CrossRef
71.
Zurück zum Zitat Jayne D, Rasmussen N, Andrassy K et al (2003) A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 349:36–44PubMedCrossRef Jayne D, Rasmussen N, Andrassy K et al (2003) A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 349:36–44PubMedCrossRef
72.
Zurück zum Zitat Metzler C, Miehle N, Manger K et al (2007) Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener’s granulomatosis. Rheumatology (Oxford) 46:1087–1091 Metzler C, Miehle N, Manger K et al (2007) Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener’s granulomatosis. Rheumatology (Oxford) 46:1087–1091
73.
Zurück zum Zitat Langford CA, Talar-Williams C, Sneller MC (2004) Mycophenolate mofetil for remission maintenance in the treatment of Wegener’s granulomatosis. Arthritis Rheum 51:278–283PubMedCrossRef Langford CA, Talar-Williams C, Sneller MC (2004) Mycophenolate mofetil for remission maintenance in the treatment of Wegener’s granulomatosis. Arthritis Rheum 51:278–283PubMedCrossRef
74.
Zurück zum Zitat Stegeman CA, Cohen Tervaert JW, De Jong PE, Kallenberg CGM (1996) Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. N Engl J Med 335:16–20PubMedCrossRef Stegeman CA, Cohen Tervaert JW, De Jong PE, Kallenberg CGM (1996) Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. N Engl J Med 335:16–20PubMedCrossRef
75.
Zurück zum Zitat Pagnoux C, Hogan SL, Chin H et al (2008) Predictors of treatment resistance and relapse in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis: Comparison of two independent cohorts. Arthritis Rheum 58:2908–2918PubMedCrossRef Pagnoux C, Hogan SL, Chin H et al (2008) Predictors of treatment resistance and relapse in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis: Comparison of two independent cohorts. Arthritis Rheum 58:2908–2918PubMedCrossRef
76.
Zurück zum Zitat Walsh M, Merkel P, Mahr A, Jayne DJ (2007) Systematic review and meta-analysis of relapses in ANCA-associated vasculitis (AAV): What Is the effect of low-dose corticosteroids? J Am Soc Nephrol 18:788ACrossRef Walsh M, Merkel P, Mahr A, Jayne DJ (2007) Systematic review and meta-analysis of relapses in ANCA-associated vasculitis (AAV): What Is the effect of low-dose corticosteroids? J Am Soc Nephrol 18:788ACrossRef
77.
Zurück zum Zitat Lapraik C, Watts R, Bacon P et al (2007) BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology (Oxford) 46:1615–1616 Lapraik C, Watts R, Bacon P et al (2007) BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology (Oxford) 46:1615–1616
78.
Zurück zum Zitat Schmitt WH, Hagen EC, Neumann I et al (2004) Treatment of refractory Wegener’s granulomatosis with antithymocyte globulin (ATG): an open study in 15 patients. Kidney Int 65:1440–1448PubMedCrossRef Schmitt WH, Hagen EC, Neumann I et al (2004) Treatment of refractory Wegener’s granulomatosis with antithymocyte globulin (ATG): an open study in 15 patients. Kidney Int 65:1440–1448PubMedCrossRef
79.
Zurück zum Zitat Lockwood CM, Hale G, Waldman H, Jayne DR (2003) Remission induction in Behcet’s disease following lymphocyte depletion by the anti-CD52 antibody CAMPATH 1-H. Rheumatology (Oxford) 42:1539–1544 Lockwood CM, Hale G, Waldman H, Jayne DR (2003) Remission induction in Behcet’s disease following lymphocyte depletion by the anti-CD52 antibody CAMPATH 1-H. Rheumatology (Oxford) 42:1539–1544
80.
Zurück zum Zitat Birck R, Warnatz K, Lorenz HM et al (2003) 15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis: a six-month open-label trial to evaluate safety and efficacy. J Am Soc Nephrol 14:440–447PubMedCrossRef Birck R, Warnatz K, Lorenz HM et al (2003) 15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis: a six-month open-label trial to evaluate safety and efficacy. J Am Soc Nephrol 14:440–447PubMedCrossRef
81.
Zurück zum Zitat Schmitt WH, Birck R, Heinzel PA et al (2005) Prolonged treatment of refractory Wegener’s granulomatosis with 15-deoxyspergualin: an open study in seven patients. Nephrol Dial Transplant 20:1083–1092PubMedCrossRef Schmitt WH, Birck R, Heinzel PA et al (2005) Prolonged treatment of refractory Wegener’s granulomatosis with 15-deoxyspergualin: an open study in seven patients. Nephrol Dial Transplant 20:1083–1092PubMedCrossRef
82.
Zurück zum Zitat Ferraro A, Day C, Drayson M, Savage C (2005) Effective therapeutic use if rituximab in refractory Wegener’s granulomatosis. Nephrol Dial Transplant 20:622–625PubMedCrossRef Ferraro A, Day C, Drayson M, Savage C (2005) Effective therapeutic use if rituximab in refractory Wegener’s granulomatosis. Nephrol Dial Transplant 20:622–625PubMedCrossRef
83.
Zurück zum Zitat Keogh KA, Ytterberg SR, Fervenza FC et al (2006) Rituximab for refractory Wegener’s granulomatosis: report of a prospective, open-label pilot trial. Am J Respir Crit Care Med 173:180–187PubMedCrossRef Keogh KA, Ytterberg SR, Fervenza FC et al (2006) Rituximab for refractory Wegener’s granulomatosis: report of a prospective, open-label pilot trial. Am J Respir Crit Care Med 173:180–187PubMedCrossRef
84.
Zurück zum Zitat Aries PM, Hellmich B, Voswinkel J et al (2006) Lack of efficacy of rituximab in Wegener’s granulomatosis with refractory granulomatous manifestations. Ann Rheum Dis 65:853–858PubMedCrossRef Aries PM, Hellmich B, Voswinkel J et al (2006) Lack of efficacy of rituximab in Wegener’s granulomatosis with refractory granulomatous manifestations. Ann Rheum Dis 65:853–858PubMedCrossRef
85.
Zurück zum Zitat Booth AD, Jefferson HJ, Ayliffe W et al (2002) Safety and efficacy of TNFalpha blockade in relapsing vasculitis. Ann Rheum Dis 61:559PubMedCrossRef Booth AD, Jefferson HJ, Ayliffe W et al (2002) Safety and efficacy of TNFalpha blockade in relapsing vasculitis. Ann Rheum Dis 61:559PubMedCrossRef
86.
Zurück zum Zitat Lamprecht P (2005) TNF-alpha inhibitors in systemic vasculitides and connective tissue diseases. Autoimmunity reviews 4:28–34PubMedCrossRef Lamprecht P (2005) TNF-alpha inhibitors in systemic vasculitides and connective tissue diseases. Autoimmunity reviews 4:28–34PubMedCrossRef
87.
Zurück zum Zitat Lamprecht P, Voswinkel J, Lilienthal T et al (2002) Effectiveness of TNF-alpha blockade with infliximab in refractory Wegener’s granulomatosis. Rheumatology (Oxford) 41:1303–1307 Lamprecht P, Voswinkel J, Lilienthal T et al (2002) Effectiveness of TNF-alpha blockade with infliximab in refractory Wegener’s granulomatosis. Rheumatology (Oxford) 41:1303–1307
88.
Zurück zum Zitat Flossmann O, Jones RB, Jayne DR, Luqmani RA (2006) Should rituximab be used to treat antineutrophil cytoplasmic antibody associated vasculitis? Ann Rheum Dis 65:841–844PubMedCrossRef Flossmann O, Jones RB, Jayne DR, Luqmani RA (2006) Should rituximab be used to treat antineutrophil cytoplasmic antibody associated vasculitis? Ann Rheum Dis 65:841–844PubMedCrossRef
89.
Zurück zum Zitat Aries PM, Hellmich B, Reinhold-Keller E, Gross WL (2004) High-dose intravenous azathioprine pulse treatment in refractory Wegener’s granulomatosis. Rheumatology (Oxford) 43:1307–1308 Aries PM, Hellmich B, Reinhold-Keller E, Gross WL (2004) High-dose intravenous azathioprine pulse treatment in refractory Wegener’s granulomatosis. Rheumatology (Oxford) 43:1307–1308
90.
Zurück zum Zitat Martinez V, Cohen P, Pagnoux C et al (2008) Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum 58:308–317PubMedCrossRef Martinez V, Cohen P, Pagnoux C et al (2008) Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum 58:308–317PubMedCrossRef
91.
Zurück zum Zitat Reinhold-Keller E, Fink CO, Herlyn K et al (2002) High rate of renal relapse in 71 patients with Wegener’s granulomatosis under maintenance of remission with low-dose methotrexate. Arthritis Rheum 47:326–332PubMedCrossRef Reinhold-Keller E, Fink CO, Herlyn K et al (2002) High rate of renal relapse in 71 patients with Wegener’s granulomatosis under maintenance of remission with low-dose methotrexate. Arthritis Rheum 47:326–332PubMedCrossRef
92.
Zurück zum Zitat Sneller MC, Hoffman GS, Talar-Williams C et al (1995) An analysis of forty-two Wegener’s granulomatosis patients treated with methotrexate and prednisone. Arthritis Rheum 38:608–613PubMedCrossRef Sneller MC, Hoffman GS, Talar-Williams C et al (1995) An analysis of forty-two Wegener’s granulomatosis patients treated with methotrexate and prednisone. Arthritis Rheum 38:608–613PubMedCrossRef
93.
Zurück zum Zitat Villa-Forte A, Clark TM, Gomes M et al (2007) Substitution of methotrexate for cyclophosphamide in Wegener granulomatosis: a 12-year single-practice experience. Medicine (Baltimore) 86:269–277 Villa-Forte A, Clark TM, Gomes M et al (2007) Substitution of methotrexate for cyclophosphamide in Wegener granulomatosis: a 12-year single-practice experience. Medicine (Baltimore) 86:269–277
94.
Zurück zum Zitat Hogan SL, Nachman PH, Wilkman AS et al. (1996) Prognostic markers in patients with anti-neutrophil cytoplasmic autoantibody associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 7:23–32PubMedCrossRef Hogan SL, Nachman PH, Wilkman AS et al. (1996) Prognostic markers in patients with anti-neutrophil cytoplasmic autoantibody associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 7:23–32PubMedCrossRef
Metadaten
Titel
Wegener-Granulomatose und mikroskopische Polyangiitis
verfasst von
PD Dr. K. de Groot
E. Reinhold-Keller
Publikationsdatum
01.02.2009
Verlag
Springer-Verlag
Erschienen in
Zeitschrift für Rheumatologie / Ausgabe 1/2009
Print ISSN: 0340-1855
Elektronische ISSN: 1435-1250
DOI
https://doi.org/10.1007/s00393-008-0425-z

Weitere Artikel der Ausgabe 1/2009

Zeitschrift für Rheumatologie 1/2009 Zur Ausgabe

Einführung zum Thema

Zielkriterien der Behandlung

Mitteilungen der DGRh

Mitteilungen

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Blutdrucksenkung könnte Uterusmyome verhindern

Frauen mit unbehandelter oder neu auftretender Hypertonie haben ein deutlich erhöhtes Risiko für Uterusmyome. Eine Therapie mit Antihypertensiva geht hingegen mit einer verringerten Inzidenz der gutartigen Tumoren einher.

„Jeder Fall von plötzlichem Tod muss obduziert werden!“

17.05.2024 Plötzlicher Herztod Nachrichten

Ein signifikanter Anteil der Fälle von plötzlichem Herztod ist genetisch bedingt. Um ihre Verwandten vor diesem Schicksal zu bewahren, sollten jüngere Personen, die plötzlich unerwartet versterben, ausnahmslos einer Autopsie unterzogen werden.

Hirnblutung unter DOAK und VKA ähnlich bedrohlich

17.05.2024 Direkte orale Antikoagulanzien Nachrichten

Kommt es zu einer nichttraumatischen Hirnblutung, spielt es keine große Rolle, ob die Betroffenen zuvor direkt wirksame orale Antikoagulanzien oder Marcumar bekommen haben: Die Prognose ist ähnlich schlecht.

Schlechtere Vorhofflimmern-Prognose bei kleinem linken Ventrikel

17.05.2024 Vorhofflimmern Nachrichten

Nicht nur ein vergrößerter, sondern auch ein kleiner linker Ventrikel ist bei Vorhofflimmern mit einer erhöhten Komplikationsrate assoziiert. Der Zusammenhang besteht nach Daten aus China unabhängig von anderen Risikofaktoren.

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.