ARTHRITIS & RHEUMATISM Vol. 54, No. 8, August 2006, pp 2568–2576 DOI 10.1002/art.22018 © 2006, American College of Rheumatology Mortality in the Catastrophic Antiphospholipid Syndrome Causes of Death and Prognostic Factors in a Series of 250 Patients Silvia Bucciarelli, 1 Gerard Espinosa, 1 Ricard Cervera, 1 Doruk Erkan, 2 Jose ´ A. Go ´mez-Puerta, 1 Manuel Ramos-Casals, 1 Josep Font, 1 and Ronald A. Asherson, 3 for the CAPS Registry Project Group (European Forum on Antiphospholipid Antibodies) Objective. To assess the main causes of death and the prognostic factors that influence mortality in pa- tients with the catastrophic antiphospholipid syndrome (CAPS). Methods. We analyzed the case reports of 250 patients included in the CAPS Registry up to February 2005. To identify prognostic factors for CAPS, we com- pared the main clinical and immunologic features and the types of treatment in the patients who died with those features in the patients who survived. Results. Recovery occurred in 56% of the episodes of CAPS and death occurred in 44%. Cerebral involve- ment, consisting mainly of stroke, cerebral hemorrhage, and encephalopathy, was considered the main cause of death, being present in 27.2% of patients, followed by cardiac involvement (19.8%) and infection (19.8%). The only factor we identified that was prognostic of a higher mortality rate was the presence of systemic lupus ery- thematosus (SLE). A higher recovery rate was associ- ated with combined treatment with anticoagulants (ACs) plus corticosteroids (CS) plus plasma exchange (PE) (77.8%), followed by ACs plus CS plus PE and/or intravenous immunoglobulins (69%). In contrast, con- comitant treatment with cyclophosphamide did not demonstrate additional benefit. Conclusion. Cerebral involvement (mainly con- sisting of stroke), cardiac involvement, and infections were considered the main causes of death in patients with CAPS. The presence of SLE was related to a higher mortality rate. According to the results of the present study, ACs plus CS plus PE should be the first line of therapy in patients with CAPS. The “catastrophic” variant of the antiphospho- lipid syndrome (CAPS) was described by Asherson in 1992 (1) as a condition characterized by multiple vascu- lar occlusive events, usually affecting the small vessels, and presenting over a short period of time, with labora- tory confirmation of the presence of antiphospholipid antibodies. Several large series demonstrating an in- crease in the number of patients with this condition over the last few years have been reported (2,3). Due to the diversity of the clinical and serologic presentations that have been described under the term “catastrophic APS,” an international consensus statement on the classifica- tion of CAPS was developed (4). In 2003, the eponym “Asherson’s syndrome” was proposed for the condition (5). The disorder is characterized by a diffuse throm- botic microvasculopathy with a predilection for the lung, brain, heart, kidney, skin, and gastrointestinal tract. In contrast to classic APS, single venous or arterial occlu- sions of the medium-to-large blood vessels are uncom- mon. However, atypical occlusive events, such as those of the adrenal, pancreatic, splenic, and testicular vessels, characterize CAPS (6). Although patients with CAPS represent 1% of all patients with APS (7), the condition is usually life- 1 Silvia Bucciarelli, MD, Gerard Espinosa, MD, PhD, Ricard Cervera, MD, PhD, FRCP, Jose ´ A. Go ´mez-Puerta, MD, Manuel Ramos-Casals, MD, PhD, Josep Font, MD, PhD: Institut Clı ´nic de Medicina i Dermatologia, Hospital Clı ´nic, Barcelona, Catalonia, Spain; 2 Doruk Erkan, MD: Hospital for Special Surgery, Weill Med- ical College of Cornell University, New York, New York; 3 Ronald A. Asherson, MD: University of the Witwatersrand, Johannesburg, South Africa. Address correspondence and reprint requests to Ricard Cervera, MD, PhD, FRCP, Servei de Malalties Autoimmunes, Hospi- tal Clı ´nic, Villarroel 170, 08036-Barcelona, Catalonia, Spain. E-mail: rcervera@clinic.ub.es. Submitted for publication October 18, 2005; accepted in revised form May 1, 2006. 2568