Zusammenfassung
Pulmonale Manifestationen zählen zu den häufigsten Organbeteiligungen der Kleingefäßvaskulitiden, werden aber bei Vaskulitiden großer und mittelgroßer Gefäße nur selten beobachtet. Bis zu 80 % aller Patienten mit einer Granulomatose mit Polyangiitis (GPA) entwickeln im Krankheitsverlauf eine pulmonale Beteiligung, z. B. in Form pulmonaler Granulome, endobronchialer Stenosen oder einer diffusen alveolären Hämorrhagie (DAH). Eine DAH kann auch bei bis zu 30 % aller Patienten mit mikroskopischer Polyangiitis auftreten, bei der granulomatöse Veränderungen per definitionem ausgeschlossen sind. Patienten mit einer eosinophilen Granulomatose mit Polyangiitis (EGPA) erkranken zunächst an einem schweren Asthma bronchiale und entwickeln im weiteren Krankheitsverlauf in bis zu 70 % flüchtige pulmonale Infiltrate als Korrelat einer eosinophilen Alveolitis. Aufgrund des regelhaften Multiorganbefalls erfolgen Diagnose und Therapie in enger interdisziplinärer Abstimmung zwischen Pneumologen und Rheumatologen und anderen Fachärzten. Pulmonale Manifestationen erfordern eine potente immunsuppressive Therapie mit Kortikosteroiden in Kombination mit langwirksamen Immunsuppressiva wie Cyclophosphamid oder Rituximab.
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Literatur
Avdalovic M (2015) Pulmonary vasculature and critical asthma syndromes: a comprehensive review. Clin Rev Allergy Immunol 48:97–103
Berden AE, Ferrario F, Hagen EC et al (2010) Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol 21:1628–1636
Berti A, Cornec D, Casal Moura M et al (2020) Eosinophilic granulomatosis with polyangiitis: clinical predictors of long-term asthma severity. Chest 157(5):1086–1099
Bossuyt X, Cohen Tervaert JW, Arimura Y et al (2017) Position paper: revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol 13:683–692
Bottero P, Bonini M, Vecchio F et al (2007) The common allergens in the Churg-Strauss syndrome. Allergy 62:1288–1294
Castaner E, Alguersuari A, Andreu M et al (2012) Imaging findings in pulmonary vasculitis. Semin Ultrasound CT MR 33:567–579
Churg A, Brallas M, Cronin S et al (1995) Formes frustes of Churg-Strauss Syndrome. Chest 108:320–323
Churg J, Strauss L (1951) Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 27:272–301
Clowse ME, Copland SC, Hsieh TC et al (2011) Ovarian reserve diminished by oral cyclophosphamide therapy for granulomatosis with polyangiitis (Wegener’s). Arthritis Care Res 63:1777–1781
Comarmond C, Pagnoux C, Khellaf M et al (2013) 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 65:270–281
Comarmond C, Crestani B, Tazi A et al (2014) Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature. Medicine (Baltimore) 93:340–349
Cordier JF, Cottin V (2011) Alveolar hemorrhage in vasculitis: primary and secondary. Semin Respir Crit Care Med 32:310–321
Cordier JF, Valeyre D, Guillevin L et al (1990) Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest 97:906–912
Csernok E (2003) Anti-neutrophil cytoplasmic antibodies and pathogenesis of small vessel vasculitides. Autoimmun Rev 2:158–164
Csernok E, Ai M, Gross W et al (2006) Wegener’s autoantigen induces maturation of dendritic cells and licenses them for Th-1 priming via protease activating receptor-2. Blood 107:4440–4448
Damoiseaux J, Csernok E, Rasmussen N et al (2017) Detection of anti-neutrophil cytoplasmic antibodies (ANCA): a multicenter EUVAS (European Vasculitis Study Group) evaluation of the value of indirect immunofluorescence (IIF) versus antigen-specific immuno-assays. Ann Rheum Dis 76:647–653
De Groot K, Adu D, Savage C (2001) The value of pulse cyclophosphamide in ANCA-associated vasculitis: meta-analysis and critical review. Nephrol Dial Transplant 16:2018–2027
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 52:2461–2469
De Groot K, Harper L, Jayne DR et al (2009) Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med 150:670–680
Gayraud M, Guillevin L, Cohen P et al (1997) Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides. Br J Rheumatol 36:1290–1297
Gottschlich S, Ambrosch P, Kramkowski D et al (2006) Head and neck manifestations of Wegener’s granulomatosis. Rhinology 44:227–233
Gouveris H, Karaiskaki N, Koutsimpelas D et al (2013) Treatment for adult idiopathic and Wegener-associated subglottic stenosis. Eur Arch Oto-Rhino-Laryngol 270:989–993
Gross WL, Schnabel A, Trabandt A (2000) New perspectives in pulmonary angiitis. From pulmonary angiitis and granulomatosis to ANCA associated vasculitis. Sarcoidosis Vasc Diffuse Lung Dis 17:33–52
Guillevin L, Lhote F, Gayraud M et al (1996) Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. Medicine 75:17–28
Guillevin L, Durand-Gasselin B, Cevallos R et al (1999a) Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 42:421–430
Guillevin L, Cohen P, Gayraud M et al (1999b) Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 78:26–37
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 cylophospahmide pulses in sixty-five patients. Arthritis Rheum 49:93–100
Guillevin L, Pagnoux C, Karras A et al (2014) Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med 371:1771–1780
Hauser T, Mahr A, Metzler C et al (2008) The leucotriene receptor antagonist montelukast and the risk of Churg-Strauss syndrome: a case-crossover study. Thorax 63:677–682
Hellmich B, Gross WL (2005) Difficult to diagnose manifestations of vasculitis: does an interdisciplinary approach help? Best Pract Res Clin Rheumatol 19:243–261
Hellmich B, Ehlers S, Csernok E et al (2003) Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol 21:S69–S77
Hellmich B, Csernok E, Gross W (2005) Proinflammatory cytokines and autoimmunity in Churg Strauss Syndrome. Ann N Y Acad Sci 1051:121–131
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 66:605–617
Hellmich B, Holl-Ulrich K, Merz H et al (2008) Hypereosinophilic syndrome and Churg-Strauss syndrome: is it clinically relevant to differentiate these syndromes? Internist (Berl) 49:286–296
Herlyn K, Hellmich B, Gross WL et al (2008) Stable incidence of systemic vasculitides in schleswig-holstein, Germany. Dtsch Arztebl Int 105:355–361
Hiemstra TF, Walsh M, Mahr A et al (2010) Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA 304:2381–2388
Hoffman GS, Sechler JM, Gallin JI et al (1991) Bronchoalveolar lavage analysis in Wegener’s granulomatosis. A method to study disease pathogenesis. Am Rev Respir Dis 143:401–407
Hoffman GS, Kerr GS, Leavitt RY et al (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116:488–498
Holle JU, Gross WL, Holl-Ulrich K et al (2010) Prospective long-term follow-up of patients with localised Wegener’s granulomatosis: does it occur as persistent disease stage? Ann Rheum Dis 69:1934–1939
Holle JU, Gross WL, Latza U et al (2011) Improved outcome in 445 patients with Wegener’s granulomatosis in a German vasculitis center over four decades. Arthritis Rheum 63:257–266
Jagadeesh LY, Sangle SR, Verma H et al (2014) Alveolar haemorrhage in eosinophilic granulomatosis and polyangiitis (Churg-Strauss). Clin Rheumatol 33:1177–1179
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 349:36–44
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 18:2180–2188
Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65:1–11
Jones RB, Tervaert JW, Hauser T et al (2010) Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 363:211–220
Karras A, Pagnoux C, Haubitz M et al (2017) Randomised controlled trial of prolonged treatment in the remission phase of ANCA-associated vasculitis. Ann Rheum Dis 76:1662–1668
Katzenstein A, Myers J (1988) Ididopathic pulmonary fibrosis. Clinical relevance of pathologic classification. Am J Respir Crit Care Med 157:199–203
Katzenstein AL (2000) Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review. Am J Clin Pathol 114:767–772
Kim YK, Lee KS, Chung MP et al (2007) Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings. Eur Radiol 17:3157–3165
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 13:36–42
Langford CA, Sneller MC, Hallahan CW et al (1996) Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis. Arthritis Rheum 39:1754–1760
Leavitt R, Fauci A, Bloch D et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101–1107
Lee KS, Kim TS, Fujimoto K et al (2003) Thoracic manifestation of Wegener’s granulomatosis: CT findings in 30 patients. Eur Radiol 13:43–51
Lhote R, Chilles M, Groh M et al (2019) Spectrum and prognosis of ANCA-associated vasculitis-related bronchiectasis: data from 61 patients. J Rheumatol. https://doi.org/10.3899/jrheum.190313. Online ahead of print
Lyons PA, Rayner TF, Trivedi S et al (2012) Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med 367:214–223
Lyons PA, Peters JE, Alberici F et al (2019) Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA atatus. Nat Commun 10(1):5120. https://doi.org/10.1038/s41467-019-12515-9
Mahr A, Moosig F, Neumann T et al (2014) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): evolutions in classification, etiopathogenesis, assessment and management. Curr Opin Rheumatol 26:16–23
Mark E, Matsubara O, Tan-Liu N et al (1988) The pulmonary biopsy in the early diagnosis of Wegener’s granulomatosis. Hum Pathol 19:1065–1071
Martinez F, Chung JH, Digumarthy SR et al (2012) Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation. Radiographics Rev Publ Radiol Soc North Am 32:51–69
Masi AT, Hunder GG, Lie JT et al (1990) The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33:1094–1100
Metzler C, Fink C, Lamprecht P et al (2004) Maintenance of remission with leflunomide in Wegener’s granulomatosis. Rheumatology 63:339–340
Metzler C, Schnabel A, Gross WL et al (2008) A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome. Clin Exp Rheumatol 26:S35–S40
Micheletti RG, Fuxench ZC, Craven A et al (2020) Cutaneous manifestations of anti-neutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. https://doi.org/10.1002/art.41310. Online ahead of print
Mirouse A, Parrot A, Audigier V et al (2020) Severe diffuse alveolar hemorrhage related to autoimmune disease: a multicenter study. Crit Care 24:231. https://doi.org/10.1186/s13054-020-02936-0
Mohammad AJ (2020) An update on the epidemiology of ANCA-associated vasculitis. Rheumatology (Oxford) 59(Suppl 3):iii42–iii50. https://doi.org/10.1093/rheumatology/keaa089
Mohammad AJ, Hot A, Arndt F et al (2014) Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Ann Rheum Dis 2:2014. https://doi.org/10.1136/annrheumdis-2014-206095. (Epub ahead of print)
Monach PA, Arnold LM, Merkel PA (2010) Incidence and prevention of bladder toxicity from cyclophosphamide in the treatment of rheumatic diseases: a data-driven review. Arthritis Rheum 62:9–21
Moosig F, Gross WL, Herrmann K et al (2011) Targeting interleukin-5 in refractory and relapsing Churg-Strauss syndrome. Ann Intern Med 155:341–343
Moosig F, Bremer JP, Hellmich B et al (2013) A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis 72:1011–1017
Mukhtyar C, Flossmann O, B Hellmich B, et al. (2008) 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 67(7):1004–1010
Mukhtyar C, Guillevin L, Cid MC et al (2009) EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 68:310–317
Ntatsaki E, Carruthers D, Chakravarty K et al (2014) BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford) 53:2306–2309
Ognibene FP, Shelhamer JH, Hoffman G et al (1995) Pneumocystis carinii pneumonia: a major complication of immunosuppressive therapy in patients with Wegener’s granulomatosis. Am J Respir Crit Care Med 151:795–799
Pagnoux C, Mahr A, Hamidou MA et al (2008) Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med 359:2790–2803
Quinn KA, Gelbard A, Sibley C et al (2019) Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology (Oxford) 58(12):2203–2211
Rahmattulla C, De Lind Van Wijngaarden RA, Berden AE et al (2015) Renal function and ear, nose, throat involvement in anti-neutrophil cytoplasmic antibody-associated vasculitis: prospective data from the European Vasculitis Society clinical trials. Rheumatology 54(5):899–907
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 43:1021–1032
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 53:93–99
Reuter M, Schnabel A, Wesner F et al (1998) Pulmonary Wegener’s granulomatosis: correlation between high-resolution CT findings and clinical scoring of disease activity. Chest 114:500–506
Reuter M, Biederer J, Both M et al (2003) Radiology of the primary systemic vasculitides. Rofo Fortschr Geb Rontgenstr Neuen Bildgeb Verfahr 175:1184–1192
Ribi C, Cohen P, Pagnoux C et al (2008) Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum 58:586–594
Sable-Fourtassou R, Cohen P, Mahr A et al (2005) Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 143:632–638
Schnabel A, Holl-Ulrich K, Dalhoff K et al (1997) Efficacy of transbronchial biopsy in pulmonary vasculitides. Eur Respir J 10:2738–2743
Schnabel A, Csernok E, Braun J et al (1999) Inflammatory cells and cellular activation in the lower respiratory tract in Churg-Strauss syndrome. Thorax 54:771–778
Schnabel A, Csernok E, Braun J et al (2000) Activation of neutrophils, eosinophils, and lymphocytes in the lower respiratory tract in Wegener’s granulomatosis. Am J Respir Crit Care Med 161:399–405
Sinico RA, Di Toma L, Maggiore U et al (2005) Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 52:2926–2935
Smith R, Jayne R, Merkel P (2019) A randomized, controlled trial of rituximab versus azathioprine after induction of remission with rituximab for patients with ANCA-associated vasculitis and relapsing disease [abstract]. Arthritis Rheum 71(Suppl 10). https://acrabstracts.org/abstract/a-randomized-controlled-trial-of-rituximab-versus-azathioprine-after-induction-of-remission-with-rituximab-for-patients-with-anca-associated-vasculitis-and-relapsing-disease/. Zugegriffen am 22.05.2020
Stegeman CA, Tervaert JW, De Jong PE et al (1996) Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med 335:16–20
Stone JH, Merkel PA, Spiera R et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363:221–232
Tomasson G, Grayson PC, Mahr AD et al (2012) Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis – a meta-analysis. Rheumatology (Oxford) 51:100–109
Travis WD, Colby TV, Lombard C et al (1990) A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation. Am J Surg Pathol 14:1112–1125
Troy LK, Grainge C, Corte TJ et al (2019) Diagnostic accuracy of transbronchial lung cryobiopsy for interstitial lung disease diagnosis (COLDICE): a prospective, comparative study. Lancet Respir Med. https://doi.org/10.1016/s2213-2600(19)30342-x
Unizony S, Villarreal M, Miloslavsky EM et al (2016) Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type. Ann Rheum Dis 75:1166–1169
Vaglio A, Strehl JD, Manger B et al (2012a) IgG4 immune response in Churg-Strauss syndrome. Ann Rheum Dis 71:390–393
Vaglio A, Moosig F, Zwerina J (2012b) Churg-Strauss syndrome: update on pathophysiology and treatment. Curr Opin Rheumatol 24:24–30
Vaglio A, Buzio C, Zwerina J (2013) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art. Allergy 68:261–273
Walsh M, Casian A, Flossmann O et al (2013) Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear. Kidney Int 84:397–402
Walsh M, Merkel PA, Peh CA et al (2020) (2020) Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis. N Engl J Med 382:622–631
Walton E (1958) Giant cell granuloma of the respiratory tract (Wegener’s granulomatosis). Br Med J 2:265–270
Watts RA, Gonzalez-Gay MA, Lane SE et al (2001) Geoepidemiology of systemic vasculitis: comparison of the incidence in two regions of Europe. Ann Rheum Dis 60:170–172
Wechsler ME, Akuthota P, Jayne D et al (2017) Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med 376(20):1921–1932
Wieczorek S, Hellmich B, Gross WL et al (2008) Associations of Churg-Strauss syndrome with the HLA-DRB1 locus, and relationship to the genetics of antineutrophil cytoplasmic antibody-associated vasculitides: comment on the article by Vaglio et al. Arthritis Rheum 58:329–330
Xiao H, Heeringa P, Hu P et al (2002) Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest 110:955–963
Xie G, Roshandel D, Sherva R et al (2013) Association of granulomatosis with polyangiitis (Wegener’s) with HLA-DPB1*04 and SEMA6 A gene variants: evidence from genome-wide analysis. Arthritis Rheum 65:2457–2468
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Hellmich, B., Henes, J., Hetzel, J. (2022). Pulmonale Manifestationen von Vaskulitiden. In: Kreuter, M., Costabel, U., Herth, F.J., Kirsten, D. (eds) Seltene Lungenerkrankungen. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-63651-0_9
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