Systemischer Lupus erythematodes und Antiphospholipid-Syndrom

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.

katastrophales APS - Alveolitis - Myelitis - Purpura - Tamponade

catastrophic APS - alveolitis - myelitis - purpura - tamponade

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