Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry

Thrombotic Microangiopathy and the Kidney

“…In CAPS, the international registry (522 episodes) reported the incidence of TMA to be 14%, and overall mortality was 37%, with no subgroup analysis according to treatment. The most frequent treatment regimens used were anticoagulation plus glucocorticoids (19%) or anticoagulation, glucocorticoids and PE with or without intravenous Ig (18%), with only 0.2% receiving eculizumab (112). There is some evidence from mouse models and observational clinical data that complement may be involved in the pathophysiology of CAPS; successful use of eculizumab has been reported in case reports and series, and a prospective trial of preemptive eculizumab to enable renal transplantation is ongoing (Clinicaltrials.gov identifier: NCT01029587) (13).…”

Catastrophic antiphospholipid syndrome: A case with unusual findings revealed in autopsy and a brief literature update

“…It is important to note however that anti-ß2glycoprotein-I was added to the APS laboratory criteria only in 2006 and the testing was not widely available earlier; most of the cases cited above were reported prior to 2006 (9). The kidney is the leading site of thrombosis in CAPS, occurring in 73% of cases; however, unlike the other reports mentioned, there was no renal thrombosis in our patient (4). In addition, it is noteworthy that CAPS developed while our patient was on LDA and with all recorded INRs >3.0, with the exception of one value of 2.4.…”

Síndrome antifosfolípidos catastrófico. Reporte de caso y revisión bibliográfica

“…En la última revisión del International CAPS Registry se comentan 500 casos registrados entre 1992 y 2014, agregándose continuamente nuevos casos al registro, como el presente 3 . Respecto del diagnóstico, se han establecido criterios diagnósticos para el SAF catastrófico definido o confirmado, los cuales son: 1) Afectación de tres o más órganos, sistemas o tejidos.…”

“…El 60% de los casos han correspondido a un SAF primario y un 30% se han asociado a lupus eritematoso sistémico. De los factores predominantes, destacan las infecciones (49%), la cirugía (17%) y la malignidad (16%) 3 .…”

“…Los hallazgos bioquímicos más sobresalientes son la trombocitopenia (67%) y la presencia de anticoagulante lúpico (83%), anticuerpos anticardiolipinas IgG (81%) y anticuerpos anti-B2 glucoproteína IgG (78%). Los anticuerpos antinucleares solo están presente en el 57%, los anti-DNA en el 32% y los anti-ENA en el 10% 3 . En cuanto al tratamiento, se han revisado tratamientos individuales y combinados.…”