Systemischer Lupus erythematodes und Antiphospholipid-Syndrom

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.