Subscribe to RSS
DOI: 10.1055/s-0030-1248295
© Georg Thieme Verlag KG Stuttgart · New York
Systemischer Lupus erythematodes und Antiphospholipid-Syndrom
Systemic Lupus Erythematosus and Antiphospholipid SyndromePublication History
Publication Date:
25 February 2010 (online)
![](https://www.thieme-connect.de/media/rheuma/201001/lookinside/thumbnails/10.1055-s-0030-1248295-1.jpg)
Zusammenfassung
Notfälle bei systemischem Lupus erythematodes (SLE) sind selten. So kommen das katastrophale Antiphospholipd-Syndrom (APS), die akute und schwere ZNS-Beteiligung, die hämorrhagische Alveolitis, die thrombotische thrombozytopenische Purpura (TTP), die transverse Myelitis und die Perikardtamponade jeweils nur bei knapp 1% der Patienten mit SLE vor. Die Mortalität dieser Manifestationen aber ist oft erheblich und kann beim katastrophalen APS 40–50%, bei der hämorrhagischen Alveolitis 50–60% und bei der TTP bis zu 90% betragen. Die Erkennung dieser Notfallsituationen erfordert spezielle diagnostische Verfahren wie z. B. ausführliche Gerinnungsanalysen beim APS, die Suche nach Fragmentozyten und die Bestimmung der ADAMTS13-Aktivität bei TTP, die bronchoalveoläre Lavage bei der hämorrhagischen Alveolitis, die Echokardiografie bei der Perikardtampondade oder das MRT bei den neurologischen Manifestationen. Die Therapie verlangt besondere Eingriffe wie die Frischplasmasubstitution oder den großvolumigen Plasmaaustausch bei der TTP, die entlastende Punktion bei der Perikardtamponade und die situationsgerechte Antikoagulation beim katastrophalen APS. Je nach Ausgangslage stellt die Gabe von hochdosierten Kortikosteroiden und von Puls-Cyclophosphamid als alleinige oder zusätzliche Maßnahme den aussichtsreichsten Weg dar, um die oft kritischen Situationen effektiv und innerhalb kurzer Zeit zu beherrschen.
Abstract
Emergencies are rare in systemic lupus erythematosus (SLE). Catastrophic antiphospholipid syndrome (APS), acute and severe CNS involvement, alveolar haemorrhage, thrombotic thrombocytopenic purpura (TTP), transverse myelitis and pericardial tamponade are the main manifestations of SLE-related emergencies, each of them occuring in about 1% of a given lupus population. The mortality of these manifestations, however, is considerable and may amount to 40–50% in APS, 50–60% in alveolar haemorrhage or even 90% in TTP. Timely recognition of these emergencies requires specific evaluations such as extensive clotting tests in APS, the search for schistocytes and analysis of ADAMTS13 activity in TTP, bronchoalveolar lavage in alveolar haemorrhage, echocardiography in pericardial tamponade and magnetic resonance imaging in neurological disease. Likewise, management of these emergencies demands specific therapies such as infusion of fresh frozen plasma or large-volume plasma exchange in TTP, adequate anticoagulation in APS and pericardiocenthesis in pericardial tamponade. High-dose corticosteroids and pulse cyclophosphamide are the most promising measures to intensify the immunosuppressive therapy and to block the SLE-related inflammation within a time-frame appropriate to the given clinical situation.
Schlüsselwörter
katastrophales APS - Alveolitis - Myelitis - Purpura - Tamponade
Key words
catastrophic APS - alveolitis - myelitis - purpura - tamponade
Literatur
- 1 Asherson RA. The catastrophic antiphospholipid antibody syndrome. J Rheumatol. 1992; 19 508-512
- 2 Cervera R, Bucciarelli S, Plasín MA. et al . Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group (European Forum On Antiphospholipid Antibodies). Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun. 2009; 32 240-245
- 3 Cervera R, Piette JC, Font J. et al . Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1 000 patients. Arthritis Rheum. 2002; 46 1019-1027
- 4 Asherson RA, Cervera R, de Groot PG. et al . Catastrophic Antiphospholipid Syndrome Registry Project Group. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus.. 2003; 12 530-534
- 5 Asherson RA, Espinosa G, Cervera R. et al . Disseminated intravascular coagulation in catastrophic antiphospholipid syndrome: clinical and haematological characteristics of 23 patients. Ann Rheum Dis. 2005; 64 943-946
- 6 Bucciarelli S, Espinosa G, Cervera R. et al . European Forum on Antiphospholipid Antibodies. Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum. 2006; 54 2568-2576
- 7 Schur PH, Khoshbin S. Neurologic manifestations of systemic lupus erythematosus. In: UpToDate Waltham, MA, 17.2; retrieved October 11th 2009: http://www.utdol.com/online/content/topic.do?topickey=lupus/10234
- 8 Kleinig TJ, Koszyca B, Blumbergs PC. et al . Fulminant leucocytoclastic brainstem vasculitis in a patient with otherwise indolent systemic lupus erythematosus. Lupus. 2009; 18 486-490
- 9 Rizos T, Siegelin M, Hähnel S. et al . Fulminant onset of cerebral immunocomplex vasculitis as first manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE). Lupus. 2009; 18 361-363
- 10 Karassa FB, Afeltra A, Ambrozic A. et al . Accuracy of anti-ribosomal P protein antibody testing for the diagnosis of neuropsychiatric systemic lupus erythematosus: an international meta-analysis. Arthritis Rheum. 2006; 54 312-324
- 11 Tokunaga M, Saito K, Kawabata D. et al . Efficacy of rituximab (anti-CD20) for refractory systemic lupus erythematosus involving the central nervous system. Ann Rheum Dis. 2007; 66 470-475
- 12 Schwab EP, Schumacher Jr HR, Freundlich B. et al . Pulmonary alveolar hemorrhage in systemic lupus erythematosus. Arthritis and Rheumatism. 1993; 23 8-15
- 13 Quadrelli S, Alvarez C, Arce S. et al . Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies. Lupus. 2009; 18 1053-1060
- 14 Eagen JW, Memoli VA, Roberts JL. et al . Pulmonary hemorrhage in systemic lupus erythematosus. Medicine. 1978; 57 545-560
- 15 Marino CT, Pertschuk LP. Pulmonary hemorrhage in systemic lupus erythematosus. Arch Intern Med. 1981; 141 201-203
- 16 Carette S, Macher AM, Nussbaum A. et al . Severe, acute pulmonary disease with systemic lupus erythematosus: ten years of experience at the National Institutes of Health. Semin Arthritis Rheum. 1984; 14 52-59
- 17 Zamora MR, Warner ML, Tuder R. et al . Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome. Medicine (Baltimore). 1997; 76 192-202
- 18 Specks U. Diffuse alveolar hemorrhage syndromes. Curr Opin Rheumatol. 2001; 13 12-17
- 19 Santos-Ocampo AS, Mandell BF, Fessler BJ. Alveolar hemorrhage in systemic lupus erythematosus. Chest. 2000; 118 1083-1090
- 20 Asherson RA, Cervera R, Piette JC. et al . Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore). 1998; 77 195-207
- 21 Boumpas DT, Austin 3rd HA, Fessler BJ. et al . Systemic lupus erythematosus: emerging concepts. Part 1: Renal, neuropsychiatric, cardiovascular, pulmonary, and hematologic disease. Ann Intern Med. 1995; 122 945-950
- 22 Moake JL. Thrombotic Microangiopathies. N Engl J Med. 2002; 347 589-600
- 23 Tsai HM. The molecular biology of thrombotic microangiopathy. Kidney Int. 2006; 70 16-23
- 24 Furlan M, Robles R, Galbusera M. et al . Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the haemolytic-uremic syndrome. N Engl J Med. 1998; 339 1578-1584
- 25 Rieger M, Mannucci PM, Kremer Hovinga JA. et al . ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood. 2005; 106 1262-1267
- 26 Mannucci PM, Vanoli M, Forza I. et al . Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue disease (systemic lupus erythematosus and systemic sclerosis). Haematologica. 2003; 88 914-918
- 27 Kwok SK, Ju JH, Cho CS. et al . Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study. Lupus. 2009; 18 16-21
- 28 Coppo P, Bengoufa D, Veyradier A. et al . Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement. Medicine (Baltimore). 2004; 83 233-244
- 29 Rock GA, Shumak KH, Buskard NA. et al . Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis study group. N Engl J Med. 1991; 325 393-397
- 30 Ling HT, Field JJ, Blinder MA. Sustained response with rituximab in patients with thrombotic thrombocytopenic purpura: a report of 13 cases and review of the literature. Am J Hematol.. 2009; 84 418-421
- 31 Theodoridou A, Settas L. Demyelination in rheumatic diseases. J Neurol Neurosurg Psychiatry. 206; 77 290-295
- 32 Lukjanowicz M, Brzosko M. Myelitis in the course of systemic lupus erythematosus. Polskie Archiwum medycyny wewnetrznej. 2009; 119 67-72
- 33 Kovacs B, Lafferty TL, Brent LH. et al . Tranverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature. Ann Rheum Dis. 2000; 59 120-124
- 34 Transverse Myelitis Consortium Working Group. . Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002; 59 499-505
- 35 . The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum. 1999; 42 599-608
- 36 Andreanakos AA, Duffy J, Suzuki M. et al . Transverse myelopathy in systemic lupus erythematosus. Report of three cases and review of the literature. Ann Intern Med. 1975; 83 616-624
- 37 Barile-Fabris L, Ariza-Andraca R, Olquín-Ortega L. et al . Controlled clinical trial of IV cyclophosphamide versus IV methyprednisolone in severe neurological manifestations in systemic lupus erythematosus. Ann Rheum Dis. 2005; 64 620-625
- 38 Doherty NE, Siegel RJ. Cardiovascular manifestations of systemic lupus erythematosus. Am Heart J. 1985; 110 1257-1265
- 39 Rosenbaum E, Krebs E, Cohen M. et al . The spectrum of clinical manifestations, outcome and treatment of pericardial tamponade in patients with systemic lupus erythematosus: a retrospective study and literature review. Lupus. 2009; 18 608-612
Korrespondenzadresse
Prof. Dr. J. O. Schröder
Universitätsklinikum
Schleswig-Holstein
Campus Kiel
2. Medizinische Klinik
Sektion Rheumatologie
Schittenhelmstraße 12
24105 Kiel
Phone: +49/0431/597 59 01
Fax: +49/0431/597 56 10
Email: o.schroeder@med2.uni-kiel.de