What we know and what we don't know about catastrophic antiphospholipid syndrome.

Catastrophic antiphospholipid syndrome (CAPS) is a severe condition with high mortality. Since its description in 1992, an important effort has been made to improve and disseminate knowledge on CAPS. Most of our current knowledge comes from the studies performed using the CAPS Registry, a database created in 2000 to gather as many cases as possible in order to better define this disease. It has demonstrated that this condition has multiple faces and is often triggered by a precipitating factor that leads to a thrombotic microangiopathy and cytokine storm involving almost any organ of the body. Analysis of the CAPS Registry has also shown that patients receiving anticoagulation, glucocorticoids and plasma exchange and/or IVIG have a better prognosis. However, there are still many unresolved questions. In this review we summarize what is known and what is still a matter of research in this condition.

Rodriguez-Pintó I ，Espinosa G ，Cervera R 《-》

Catastrophic antiphospholipid syndrome: Lessons from the "CAPS Registry".

Rodriguez-Pintó I ，Espinosa G ，Cervera R 《-》

Rituximab use in the catastrophic antiphospholipid syndrome: descriptive analysis of the CAPS registry patients receiving rituximab.

The catastrophic variant of the antiphospholipid syndrome (APS) is characterized by thrombosis in multiple organs developing over a short period of time. First-line treatment for the catastrophic APS is the combination of anticoagulation plus corticosteroids plus plasma exchange and/or intravenous immunoglobulin. Despite this regimen, the mortality remains high and new treatment options are needed. By a systematic review of the Catastrophic APS Registry (CAPS Registry), we identified 20 patients treated with rituximab. The purpose of this study is to describe the clinical manifestations, laboratory features, and outcomes of rituximab-treated CAPS patients. In addition, the rationale for using rituximab in catastrophic APS is discussed.

Berman H ，Rodríguez-Pintó I ，Cervera R ，Morel N ，Costedoat-Chalumeau N ，Erkan D ，Shoenfeld Y ，Espinosa G ，Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group (European Forum on Antiphospholipid Antibodies) ... -  《-》

Catastrophic antiphospholipid syndrome: The current management approach.

The current recommendation for catastrophic antiphospholipid syndrome (CAPS) management is the standard triple therapy with anticoagulation (AC), glucocorticoids (GCs), plasma exchange (PE), and/or intravenous immunoglobulins (IVIGs). Of note, only AC has a significant effect on the prognosis of these patients. However, from the experimental or basic point of view, there is only indirect evidence to advocate the use of these immunomodulatory therapies (GC, PE, and IVIG) in CAPS. Recently, there have been reports of severe or refractory CAPS patients treated with the monoclonal antibodies rituximab and eculizumab. The first blocks CD20, a surface protein expressed on the cytoplasmic membrane of B cells, and decreases the generation of pathogenic autoantibodies such as antiphospholipid (aPL) antibodies. The second binds with high affinity to C5 complement protein, inhibiting its cleavage and thus preventing the generation of C5b-C9 complex.

Rodriguez-Pintó I ，Espinosa G ，Cervera R 《-》

Therapeutic and prognostic considerations in catastrophic antiphospholipid syndrome.

Erkan D 《AUTOIMMUNITY REVIEWS》