Apheresis in high risk antiphospholipid syndrome pregnancy and autoimmune congenital heart block.

In the first part a prospective cohort study was reported to evaluate the efficacy and safety of a treatment protocol including plasma exchange (PE) or PE plus intravenous immunoglobulins (IVIG) or immunoadsorption (IA) plus IVIG administered in addition to conventional therapy to 22 pregnant women with high-risk APS. The results indicate that PE or IA treatments administered along with IVIG and conventional antithrombotic therapy could be a valuable and safe therapeutic option in pregnant APS women with triple antiphospholipid antibody positivity along with a history of thrombosis and/or one or more severe pregnancy complications. In the second part the efficacy and safety of PE combined with IVIG and steroids were evaluated for the treatment of 10 patients with autoimmune congenital heart block (CHB) by comparing maternal features, pregnancy outcome and side effects with those of 24 CHB patients treated with steroids only. The patients treated with the combined therapy showed a statistically significant regression of 2nd degree blocks, an increase in heart rate at birth and a significantly lower prevalence of pacing in the first year of life. Moreover, no side effects were observed except for a few steroid-related events. If these results are confirmed by large-scale studies, the apheretic procedures could lead to improved outcomes in the treatment of these devastating diseases.

The clinical relevance of the IgM isotype of antiphospholipid antibodies in the vascular antiphospholipid syndrome.

OBJECTIVES: Because it has been suggested that the IgM isotype of antiphospholipid (aPL) antibodies should no longer be included in the laboratory criteria for antiphospholipid syndrome (APS) classification, we assess the clinical relevance of IgM isotype of aPL in a cohort of patients with vascular APS. PATIENTS/METHODS: Mean age, sex, the presence of autoimmune diseases other than systemic lupus erythematosus, risk factors for thrombosis, the type/s and site/s of thromboembolic events, the levels of C3 and C4, and autoantibody profile were evaluated in a large cohort of persistently aPL positive patients fulfilling the Sydney criteria for APS. Patients with isolated IgM isotype were compared for each variable with those with any other aPL antibody combination. RESULTS: One hundred six patients were assessed; of these 55 (51.9%) had venous thromboembolism, 48 (45.3%) arterial thrombosis, and 3 (2.8%) small vessel thrombosis. Positivity to only IgM aPL made possible to classify 13 patients (12.3%) as vascular APS. In all cases the presence of IgM aPL was at medium-high titer, confirmed, and found to be stable in the time. There were four patients with retinal thrombosis (3.8%) and the prevalence of this event was significant in the isolated IgM isotype positive patients (p=0.005). CONCLUSIONS: Data from this investigation give clinical value to the IgM isotype of aPL and suggest to consider aCL and anti-ß2GPI of IgM class as valid laboratory criteria for APS classification, especially when they are associated and stable overtime.

Recovery from catastrophic antiphospholipid syndrome by a plasma exchange procedure: report of four cases and review of the literature.

Catastrophic Antiphospholipid Syndrome (CAPS) is a rare (1%), life-threatening variant of Antiphospholipid Syndrome (APS). It has been found that the recovery rate is best when the treatment protocol includes anticoagulants, steroids and therapeutic plasma exchange (TPE). The treatment of CAPS with TPE is not, however, well defined as procedure modalities have not yet been standardized, and the best replacement fluid for TPE is still a controversial issue. Although the most commonly used one, fresh frozen plasma (FFP), contains natural anticoagulants, it is also made up of clotting factors, complement activation products and cytokines which could worsen CAPS' "thrombotic storm". The successful management of 4 CAPS patients, including TPE sessions initiated in the 1st week from diagnosis and using albumin solution as the replacement fluid, is described here. TPE was performed daily for the first 3 days, then tapered off, and withdrawn on the basis of patient's clinical condition. One of the patients was also treated with anticoagulants, while the others received anticoagulants plus high doses of steroids in addition to TPE. Our results indicate that, when initiated promptly and albumin solution is used as the replacement fluid, TPE can be considered an effective, safe treatment for CAPS.

Case reports of the use of immunoadsorption or plasma exchange in high-risk pregnancies of women with antiphospholipid syndrome.

Conventional treatment of antiphospholipid syndrome (APS) pregnancies with aspirin and/or heparin is sometimes unable to counteract maternal and/or fetal complications. In this article we report the cases of two patients who were unresponsive to conventional treatment for APS during their first pregnancy, and who were treated in the following pregnancy with plasma exchange and immunoadsorption respectively, in addition to conventional therapy. Both patients had a history of thrombotic events, a previous pregnancy loss at the 11th week of gestation and the same antiphospholipid antibody profile (lupus anticoagulant activity and high titers of immunoglobulin G (IgG) anti-beta2 glycoprotein I and IgG anticardiolipin antibodies). Patient 1 was treated from the fourth week of her second pregnancy with weekly plasma exchange. Due to fetal growth restriction and oligohydramnios in the 26th week she delivered, by cesarean section, a healthy female infant weighing 730 g who survived. Patient 2 was treated from the seventh week of her second pregnancy with twice a week protein A immunoadsorption. The pregnancy proceeded normally until the 36th week, when, due to slight intrauterine growth restriction, she delivered a healthy baby girl weighing 2375 g by cesarean section. Anti-beta2 glycoprotein I antibody trends were similar during both types of treatment. On the basis of our findings obtained from only two cases it is impossible to define the best aphaeretic treatment of APS high risk pregnancies. Nevertheless, as a whole these data suggest better disease control using the immunoadsorption technique as compared to plasma exchange, despite their apparently similar anti-beta2 glycoprotein I antibody removal capabilities.