Catastrophic antiphospholipid syndrome: Clinical characteristics and outcomes in a non-caucasian population - A...

BackgroundCatastrophic Antiphospholipid Syndrome (CAPS) is a rare but highly severe manifestation of antiphospholipid syndrome (APS), occurring in fewer than 1% of APS patients, but is associated with mortality of around 25-37%, despite treatment. It is characterized by rapid onset of widespread thrombotic events leading to microangiopathy, intravascular thrombosis with multiorgan failure, involving three or more organs within one week. Most published data originate from European or multinational registries like the CAPS Registry. However, data on CAPS in Latin American populations, who may differ in genetic background, socioeconomic status, and healthcare access, remain scarce.MethodsThe objective was to describe the clinical characteristics, treatment strategies, and outcomes of patients diagnosed with CAPS at a tertiary referral center in Mexico City over 5 years. We conducted a retrospective cohort study of patients who fulfilled the CAPS classification criteria between January 2019 and December 2023.ResultsNineteen patients were included (74% female; median age 38.4 years). Six had primary APS, while 11 had concomitant systemic lupus erythematosus (SLE). Based on the 2019 EULAR aPL titers classification, seven patients were classified as low-risk and 12 as high-risk. In 47%, CAPS was the first manifestation of APS. Precipitating factors were identified in 94.7% of cases, most commonly infections and anticoagulant withdrawal. The most frequently affected were the arterial and venous circulation (74% and 68%, respectively), hematologic manifestations (47%), kidneys (42%), lungs (42%), and the heart (32%). Treatment strategies included anticoagulation (84%), corticosteroids (79%), plasma exchange (79%), triple therapy (74%), and, in selected cases, immunosuppressants. Overall mortality was 42%; among survivors, 75% achieved full recovery with no relapses.ConclusionThis study represents one of the largest single-center series of CAPS in a non-Caucasian population. Our findings reveal a predominance of high-risk, secondary APS-mainly SLE-associated-with thrombocytopenia. Nearly half presented with CAPS as the initial APS manifestation. Limited access to full triple therapy significantly impacted mortality, which decreased from 100% with partial therapy to 21% with complete regimens. Early recognition, prompt management, and multidisciplinary care were associated with favourable outcomes, underscoring the need for adaptable therapeutic strategies in resource-limited settings.