ANCA-assoziierte Vaskulitiden: Diagnose und Therapie von Notfallsituationen

Zusammenfassung

Notfallsituation entstehen bei Patienten mit einer mit anti-neutrophilen zytoplasmatischen Antikörpern assoziierten Vaskulitis (AAV) einerseits durch lebens- oder organbedrohende Manifestationen der Grunderkrankung und andererseits durch Komplikationen der immunsuppressiven Therapie, wie z. B. schwere Infektionen. Von den sehr schweren Manifestationen der AAV sind insbesondere die diffuse alveoläre Hämorrhagie sowie die schwere gastrointestinale Manifestation unmittelbar lebensbedrohend und erfordern daher eine Diagnose und Therapie ohne zeitlichen Verzug. Patienten mit diesen Manifestationen sollten auf einer Intensivstation interdisziplinär überwacht und behandelt werden. Weitere Manifestationen einer AAV, die aufgrund einer Bedrohung der Organfunktion als Notfallsituation gesehen werden können, sind die rapid-progressive Glomerulonephritis, die Beteiligung des zentralen Nervensystems, die subglottische Stenose sowie kardiale Manifestationen und thrombembolische Komplikationen. Die vorliegende Übersicht gibt einen Überblick über die Diagnose, Therapie und Prognose dieser schweren Manifestationen einer AAV.

Abstract

In patients with anti-neutrophil cytoplasm antibody-associated vasculitides, emergency situations can arise from either life- or organ-threatening manifestations of the underlying disease or from complications associated with immunosuppressive treatment, such as severe infections. Among the most severe manifestations of AAV, diffuse alveolar haemorrhage and severe gastrointestinal manifestations are immediately life-threatening and require immediate diagnosis and treatment. Patients with these manifestations should be monitored and treated in an intensive care unit. Further manifestations of AAV are rapid progressive glomerulonephritis, involvement of the central nervous system, subglottic stenosis, cardiac manifestations and thrombembolic events. This review is aimed to focus on the diagnosis, treatment and prognosis of these severe manifestations of the AAVs.

Schlüsselwörter

ANCA-assoziierte Vaskulitis - diffuse alveoläre Hämorrhagie - rapidprogressive Glomerulonephritis - ZNS-Manifestation

Key words

ANCA-associated vasculitis - diffuse alveolar haemorrhage - rapidprogressive glomerulonephritis - CNS involvement

Literatur

• 1 Allenbach Y, Seror R, Pagnoux C. et al . High frequency of thrombembolic events in Churg-Strauss Syndrome, Wegener's granulomatosis and microscopic polyangiitis but not polyarteriitis nodosa: a systemaitc retrospective study of 1 130 patients.  Ann Rheum Dis. 2009;  68 564-567
• 2 Alrawi A, Trobe J, Blaivas M. et al . Brain Biopsy in primary angiitis of the central nervous system.  Neurology. 1999;  53 858-860
• 3 Asmus R, Koltze H, Muhle C. et al . MRI of the head in Wegener's granulomatosis.  Adv Exp Med Biol. 1993;  336 319-321
• 4 Burkhardt O, Köhnlein T, Wrenger E. et al . Predicting outcome and survival in patients with Wegener's granulomatosis treated on the intesive care unit.  Scand J Rheumatol. 2007;  36 119-124
• 5 Calabrese LH, Duna G, Lie JT. Vasculitis in the central nervous system.  Arthritis Rheum. 1997;  40 1189-1201
• 6 Cannady SB, Batra PS, Koening C. et al . Sinonasal Wegener granulomatosis: a single-institution experience with 120 cases.  Laryngoscope. 2009;  119 ((4)) 757-761
• 7 Chu C, Gray L, Goldstein L. et al . Diagnosis of intracranial vasculitis: a mulitdisciplinary approach.  J Neuropathol Exp Neurol. 1998;  57 30-38
• 8 Cruz BA, Ramnoelina J, Mahr A. et al . Prognosis and outcome of 26 patients with systemic necrotizing vasculitis admitted to the intensive care unit.  Rheumatology. 2003;  42 1183-1188
• 9 de Groot K, Harper L, Jayne DR. et al . Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial.  Ann Intern Med.. 2009;  150 ((10)) 670-680
• 10 Frankel S, Schwarz MI. Cardiopulmonary manifestations of vasculitis. In: Ball G, Bridges S eds. Vasculitis, 2 ed Oxford: Oxford University Press; 2008
• 11 Guillevin L, Durand-Gasselin B, Cevallos R. et al . Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients.  Arthritis Rheum.. 1999;  42 ((3)) 421-430
• 12 Guillevin L, Lhote F, Gayraud M. et al . Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome.  Medicine. 1996;  75 17-28
• 13 Ha HK, Lee SH, Rha SE. et al . Radiologic features of vasculitis involving the gastrointestinal tract.  Radiographics.. 2000;  20 ((3)) 779-794
• 14 Haworth S, Savage C, Carr D. et al . Pulmonary haemorrhage complicating Wegeners's granulomatosis and microscopic polyangiitis.  Br Med J. 1985;  290 1775-1778
• 15 Hellmich B, Csernok E, Fredenhagen G. et al . A novel high-sensitivity ELISA for detection of antineutrophil cytoplasm antibodes against proteinase-3.  (submitted) 2006; 
• 16 Hellmich B, Gross W. Difficult to diagnose manifestations of vasculitis: does an interdisciplinary approach help?.  Bailleres Best Pract Res Clin Rheumatol. 2005;  19 ((2)) 243-261
• 17 Hellmich B, Lamprecht P, Aries P. et al . Frühdiagnose von Vaskulitiden.  Z Rheumatol. 2005;  64 538-546
• 18 Hellmich B, Lamprecht P, Gross WL. Advances in the therapy of Wegener's granulomatosis.  Curr Opin Rheumatol.. 2006;  18 ((1)) 25-32
• 19 Hellmich B, Metzler C, Gross W. Churg-Strauss Syndrom: aktueller Stand der Diagnostik und Therapie.  Dtsch Med Wochenschr. 2006;  131 2270-2274
• 20 Hermon MM, Golej J, Emminger W. et al . Acute hemorrhagic respiratory failure caused by Wegener's granulomatosis successfully treated by bronchoalveolar lavage with diluted surfactant.  Wien Klin Wochenschr. 2003;  115 ((21–22)) 793-796
• 21 Hoffman GS, Kerr GS, Leavitt RY. et al . Wegener granulomatosis: an analysis of 158 patients.  Ann Intern Med. 1992;  116 ((6)) 488-498
• 22 Holguin F, Ramadan B, Gal AA. et al . Prognostic factors for hospital mortality and ICU admission in patients with ANCA-related pulmonary vasculitis.  Am J Med Sci.. 2008;  336 ((4)) 321-326
• 23 Jayne D. Challenges in the management of microscopic polyangiitis: past, present and future.  Curr.Opin.Rheumatol. 2008;  20 ((1)) 3-9
• 24 Jayne DR, Gaskin G, Rasmussen N. et al . Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis.  J Am Soc Nephrol.. 2007;  18 ((7)) 2180-2188 Epub 2007 Jun 20.
• 25 Keogh KA, Specks U. Churg-Strauss syndrome clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists.  Am J Med. 2003;  115 ((4)) 284-290
• 26 Khan S, Subla M, Behl D. et al . Outcome of patients with small-vessel vasculitis admitted to a medical ICU.  Chest. 2007;  131 972-976
• 27 Klemmer PJ, Chalermskulrat W, Reif MS. et al . Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis.  Am J Kidney Dis. 2003;  42 ((6)) 1149-1153
• 28 Lauque D, Cadranel J, Lazor R. et al . Microscopic polyangiitis with alveolar hemorrhage A study of 29 cases and review of the literature Groupe d'Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM“O”P).  Medicine (Baltimore). 2000;  79 ((4)) 222-233
• 29 Little MA, Nightingale P, Verburgh CA. et al . Early mortality in systemic vasculitis: relative contribution of adverse events and active vasculitis.  Ann Rheum Dis. 2009;  1 1
• 30 Merkel PA, Lo GH, Holbrook JT. et al . Brief communication: high incidence of venous thrombotic events among patients with Wegener granulomatosis: the Wegener's Clinical Occurrence of Thrombosis (WeCLOT) Study.  Ann Intern Med. 2005;  142 ((8)) 620-626
• 31 Meyer M, Schnabel A, Schatz H. et al . Lack of association between antiphospholipid antibodies and thrombocytopenia in patients with Wegener's granulomatosis.  Semin Arthritis Rheum. 2001;  31 1-3
• 32 Mukhtyar C, Flossmann O, Hellmich B. et al . Outcomes from studies of Antineutrophil Cytoplasm Antibody Associated Vasculitis: a systematic review by the EULAR Systemic Vasculitis Task Force.  Ann Rheum Dis. 2008;  67 1004-1010
• 33 Mukhtyar C, Guillevin L, Cid MC. et al . EULAR recommendations for the management of primary small and medium vessel vasculitis.  Ann Rheum Dis.. 2009;  68 ((3)) 310-317 . Epub 2008 Apr 15.
• 34 Murphy J, Gomez-Anson B, Antoun N. et al . Wegener's granulomatosis: MR imaging findings in brain and meninges.  Radiology. 1999;  213 794-799
• 35 Nagashima T, Maguchi S, Terayama Y. et al . P-ANCA-positive Wegener's granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: case report and review of literature.  Neuropathology. 2000;  20 ((1)) 23-30
• 36 Neumann T, Manger B, Schmid M. et al . Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis.  Medicine (Baltimore). 2009;  88 ((4)) 236-243
• 37 Nishino H, DeReeme R, Rubino F. et al . Wegener's granulomatosis associated with vasculitis of the temporal artery: report of five cases.  Mayo Clin Proc. 1993;  68 115-121
• 38 Nölle B, Specks U, Lüdemann J. et al . Anticytoplasmic autoantibodies: Their immunodiagnostic value in Wegener's granulomatosis.  Ann Intern Med. 1989;  111 28-40
• 39 Pagnoux C, Guilpain P, Guillevin L. Churg-Strauss syndrome.  Curr.Opin.Rheumatol.. 2007;  19 ((1)) 25-32
• 40 Pagnoux C, Mahr A, Cohen P. et al . Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis.  Medicine (Baltimore). 2005;  84 ((2)) 115-128
• 41 Pomper M, Miller T, Stone JH. et al . CNS vasculitis in autoimmune disease: MR imaging findings and correlation with angiography.  Am J Neuroradiol. 1999;  20 75-85
• 42 Rabe C, Appenrodt B, Hoff C. et al . Severe respiratory failure due to diffuse alveolar hemorrhage: Clinical characteristics and outcome of intensive care.  J Crit Care. 2009;  8 8
• 43 Ragab R. Vasculitis manifestations in the gastrointestinal tract.. In: Ball G, Bridges S , eds Vasculitis. Oxford: Oxford University Press; 2008: 1181-1198
• 44 Ravindran R, Watts R. pulmonary haemorrhage in ANCA-associated vasculitis: a 7-year single-center experience.  Ann Rheum Dis. 2009;  68 ((Suppl 3)) 82
• 45 Reinhold-Keller E, Beuge N, Latza U. et al . An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients.  Arthritis Rheum. 2000;  43 ((5)) 1021-1032
• 46 Reinhold-Keller E, de Groot K, Fink C. et al . High rate of renal relapse under maintenance of remission with low dose methotrexate in Wegener's granulomatose.  Arthritis Rheum. 2001; 
• 47 Reinhold-Keller E, de Groot K, Holl-Ulrich K. et al . Severe CNS manifestations as the clinical hallmark in generalized Wegener's granulomatosis consistently negative for antineutrophil cytoplasmic antibodies (ANCA). A report of 3 cases and a review of the literature.  Clin Exp Rheumatol. 2001;  19 ((5)) 541-549
• 48 Reuter M, Biederer J, Both M. et al . Radiology of the primary systemic vasculitides.  Rofo Fortschr Geb Rontgenstr Neuen Bildgeb Verfahr. 2003;  175 ((9)) 1184-1192
• 49 Sanchez-Masiques J, Ettensohn DB. Alveolar hemorrhage in Wegener's granulomatosis.  Am J Med Sci. 1989;  297 ((6)) 390-393
• 50 Schnabel A, Reuter M, Csernok E. et al . Subclinical alveolar bleeding in pulmonary vasculitides: correlation with indices of disease activity.  Eur Resp J. 1999;  14 118-127
• 51 Semple D, Keogh J, Forni L. et al . Vasculitis on the intensive care unit – part 2: treatment and prognosis.  Critical Care. 2004;  9 193-197
• 52 Slobodin G, Hussein A, Rozenbaum M. et al . The emergency room in systemic rheumatic diseases.  2006;  23 ((9)) 667-671
• 53 Soding P, Lockwood CM, Park G. The intesive care of patients with fulminant vasculitis.  Anesthesia Intensive Care. 1994;  22 81-89
• 54 Solans-Laque R, Bosch-Gil J, Canela M. et al . Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis.  Lupus. 2008;  17 ((9)) 832-836
• 55 Specks U, Moder KG, McDonald TJ. Meningeal involvement in Wegener granulomatosis.  Mayo Clin Proc. 2000;  75 ((8)) 856-859
• 56 Stassen PM, Derks RPH, Kallenberg CGM. et al . Venous thromboembolism in ANCA-associated vasculitis--incidence and risk factors.  Rheumatology. 2008;  47 ((4)) 530-534
• 57 Tervaert JW. Vasculitis and the intensive care.  Acta Clin Belg Suppl. 2007;  ((2)) 417-421
• 58 Walton E. Giant cell granuloma of the respiratory tract (Wegener's granulomatosis).  Br Med J. 1958;  2 265-270
• 59 West S. Central nervous system vasculitis.  Current Rheumatology Reports. 2003;  5 116-127
• 60 Wiesner W, Khurana B, Roos P. CT of acute bowel ishemia.  Radiology. 2003;  226 635-650

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