PP068. Catastrophic antiphospholipid-syndrome (CAPS) - A severe pregnancy complication

Antiphospholipid syndrome (APS), is an autoimmune, hypercoagulable state caused by antibodies against cell-membrane phospholipids provoking arterial and venous thromboses as well as pregnancy-related complications such as miscarriage, stillbirth, preterm delivery, or severe preeclampsia. The syndrome occurs due to the autoimmune production of antibodies against phospholipid (aPL), a cell membrane substance. In particular, the disease is characterised by antibodies against cardiolipin (anti-cardiolipin antibodies) and β2 glycoprotein I. In rare cases, APS can lead to rapid organ failure due to generalised thrombosis. This life-threatening complication is termed "catastrophic antiphospholipid syndrome" (CAPS) and is associated with a high maternal mortality.

To describe the characteristics of patients who developed catastrophic APS triggered during pregnancy and to possibly identify potential risk factors for the development of this complication.

Patients charts of women with autoimmune disorders (such as APS or systemic lupus erythematodes) observed and treated at the University of Graz and The University of Jena between 2007 and 2012 were evaluated.

Four cases of CAPS were identified. In all women CAPS occurred as a severe early onset complication (<34 weeks of gestation) and all women had to be delivered by caesarean section between 27 and 32 weeks. With an "individualized" treatment including plasmapheresis, pregnancy can be prolonged for a short period to at least achieve lung maturation by steroids. Several specific features could be found: HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome-like symptoms, eclampsia-like symptoms (headache, amaurosis), abdominal pain resistent to conventional analgesic therapy, and intrauterine growth restriction. Histologic examination after delivery revealed placental infarctions.

It is important to consider the possibility of the development of catastrophic APS in those patients with signs of HELLP syndrome and multiorgan failure during pregnancy or puerperium, especially in those patients with previous history of abortions and/or thrombosis. In specialised centers prolongation of pregnancy with an individualized treatment including plasmapheresis may be an option.