ABSTRACT
El síndrome antifosfolípidos catastrófico (SAFC) es una entidad rara. Se han reportado aproximadamente 600 casos en todo el mundo, y se desconoce la prevalencia en México. Objetivo: Conocer la prevalencia estimada de SAFC en México. Material y métodos: Se realizó una búsqueda bibliográfica de casos clínicos aislados o series de casos en los diversos buscadores, utilizando los términos «síndrome antifosfolípidos catastrófico» y «México», en mayo de 2022. Resultados: Encontramos una serie de casos retrospectivos en necropsias que incluyeron 12 casos, dos reportes que incluyeron 2 casos cada uno, y también se encontraron reportes de 11 casos clínicos aislados; estas publicaciones se generaron entre 2003 y 2020. En total, se tienen datos de 27 casos de SAFC, de los cuales 16 corresponden al síndrome antifosfolípidos primario, 10 en asociación con lupus eritematoso sistémico y 1 caso de esclerosis sistémica. La tasa de prevalencia estimada en la población mexicana en 2022 es de 2 casos por cada 10.000.000 de habitantes. La mortalidad estimada fue del 68% en esta serie de casos. Conclusión: Los casos de SAFC en México están subreportados; sin embargo, identificarlos ayudará a mejorar las estrategias diagnósticas y terapéuticas que se utilizan actualmente en el país, incentivando la implementación de la triple terapia y, en casos refractarios, el uso de eculizumab, para reducir la mortalidad actual. (AU)
Catastrophic antiphospholipid syndrome (CAPS) is a rare entity, approximately 600 cases have been reported around the world, and the prevalence in Mexico is unknown. Objective: To determine the estimated prevalence of CAPS in Mexico. Material and methods: A literature search of isolated clinical cases or case series was conducted in diverse search engines, using the terms: «catastrophic antiphospholipid syndrome» and «Mexico» in May 2022. Results: We found a series of retrospective cases in autopsies that included 12 cases, two reports that included 2 cases each, and reports of 11 isolated clinical cases; these publications were generated between 2003 and 2020. In total, we collected data on 27 cases of CAPS, of which 16 correspond to primary antiphospholipid syndrome, 10 are associated with systemic lupus erythematosus, and 1 case corresponds to systemic sclerosis. The estimated prevalence rate in the Mexican population in 2022 is 2 cases per 10,000,000 inhabitants. The estimated mortality was 68% in this case series. Conclusion: Cases of catastrophic antiphospholipid syndrome in Mexico are underreported; identifying them will help improve current diagnostic and therapeutic strategies used in the country, encouraging the implementation of triple therapy and, in refractory cases, the use of eculizumab, to reduce current mortality. (AU)
Subject(s)
Humans , Antiphospholipid Syndrome/epidemiology , Antiphospholipid Syndrome/mortality , Mexico , Prevalence , Antiphospholipid Syndrome/therapyABSTRACT
Catastrophic antiphospholipid syndrome (CAPS) is a rare entity, approximately 600 cases have been reported around the world, and the prevalence in Mexico is unknown. OBJECTIVE: To determine the estimated prevalence of CAPS in Mexico. MATERIAL AND METHODS: A literature search of isolated clinical cases or case series was conducted in diverse search engines, using the terms: "Catastrophic Antiphospholipid Syndrome" and "Mexico" in May 2022. RESULTS: We found a series of retrospective cases in autopsies that included 12 cases, two reports that included 2 cases each, and reports of 11 isolated clinical cases; these publications were generated between 2003 and 2020. In total, we collected data on 27 cases of CAPS, of which 16 correspond to primary antiphospholipid syndrome, 10 are associated with systemic lupus erythematosus, and 1 case corresponds to systemic sclerosis. The estimated prevalence rate in the Mexican population in 2022 is 2 cases per 10,000,000 inhabitants. The estimated mortality was 68% in this case series. CONCLUSION: Cases of catastrophic antiphospholipid syndrome in Mexico are underreported; identifying them will help improve current diagnostic and therapeutic strategies used in the country, encouraging the implementation of triple therapy and, in refractory cases, the use of eculizumab, to reduce current mortality.
ABSTRACT
OBJECTIVES: To describe the real-world experience of eculizumab use in patients with catastrophic antiphospholipid syndrome (CAPS) according to the information provided by the "CAPS Registry". METHODS: We analyzed the demographic, clinical and immunological data from all the patients included in the "CAPS Registry" treated with eculizumab and described the indications for eculizumab administration, dose, outcome, use of prophylactic vaccines and adverse effects. RESULTS: The "CAPS Registry" currently includes 584 patients from whom 39 (6.7%) were treated with eculizumab (it was used as a rescue therapy in 30 cases while in 6 cases it was used as first line therapy). Mean age of eculizumab treated patients was 39 years (SD = 14.6), 72% were female, 77% had a primary APS and 79% had a precipitating factor before the CAPS event. Thrombocytopenia was present in 28 (72%) cases and features of microangiopathic hemolytic anemia were present in 15 (38.5%). Twenty-nine (74.4%) patients recovered from the episode of CAPS (four showed only partial remission). Symptoms worsened in 9 patients, from whom 5 finally died despite the treatment. There was only one relapse after a median follow up of 10.7 months. The most common treatment regimen was 900 mg weekly for four weeks and 1200 mg fortnightly. CONCLUSION: According to the real-world experience provided by the "CAPS Registry", eculizumab can be considered in some patients with CAPS refractory to previous therapies, especially if they present with features of complement-mediated thrombotic microangiopathy.
Subject(s)
Antibodies, Monoclonal, Humanized , Antiphospholipid Syndrome , Adult , Anemia, Hemolytic , Antibodies, Monoclonal, Humanized/therapeutic use , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Antiphospholipid Syndrome/drug therapy , Catastrophic Illness , Female , Humans , Male , Middle Aged , Registries , ThrombocytopeniaABSTRACT
ABSTRACT The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the development of thrombotic events and/or obstetric morbidity in the presence of antiphospholipid antibodies (aPL), such as the lupus anticoagulant (LA), anticardiolipin antibodies (aCL) or anti- β 2-glycoprotein I antibodies (a β2 GPI). In 1992, Ronald A. Asherson described a very aggressive clinical variant of this syndrome characterized by the development of multiple thrombotic manifestations, simultaneously or in a short period of time. The term catastrophic APS was proposed and since then it is known by this name.
RESUMEN El síndrome antifosfolípido (SAF) es una enfermedad sistêmica autoinmune, caracterizada por el desarrollo de eventos trombóticos y/o morbilidad obstétrica en presencia de anticuerpos antifosfolípidos (aPL), tales como el anticoagulante lúpico (AL), los anticuerpos anticardiolipina (aCL) o anticuerpos anti- β2-glicoproteína I (aβ2GPI). En 1992, Ronald A. Asherson describió una variante clínica muy agresiva de este síndrome, caracterizada por el desarrollo de múltiples manifestaciones trombóticas, de manera simultánea o dentro de un corto periodo de tiempo. Se propuso entonces el término SAF catastrófico y desde entonces se le ha conocido por ese nombre.