array:22 [ "pii" => "S0025775324001581" "issn" => "00257753" "doi" => "10.1016/j.medcli.2024.02.011" "estado" => "S300" "fechaPublicacion" => "2024-08-01" "aid" => "6597" "copyright" => "Elsevier España, S.L.U.. All rights reserved" "copyrightAnyo" => "2024" "documento" => "article" "crossmark" => 1 "subdocumento" => "rev" "cita" => "Med Clin. 2024;163 Supl 1:S31-S35" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "itemAnterior" => array:18 [ "pii" => "S002577532400157X" "issn" => "00257753" "doi" => "10.1016/j.medcli.2024.02.010" "estado" => "S300" "fechaPublicacion" => "2024-08-01" "aid" => "6596" "copyright" => "Elsevier España, S.L.U." "documento" => "article" "crossmark" => 1 "subdocumento" => "rev" "cita" => "Med Clin. 2024;163 Supl 1:S22-S30" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Review</span>" "titulo" => "Thrombotic antiphospholipid syndrome: From guidelines to clinical management" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "S22" "paginaFinal" => "S30" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome antifosfolípido trombótico: de las guías al manejo clínico" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1597 "Ancho" => 3000 "Tamanyo" => 217592 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Algorithm for the therapy of first and recurrent thrombotic events. * First assure adherence and in-range INR levels before the recurrent event. APS: antiphospholipid syndrome; VKA: vitamin K antagonist; INR: international normalized ratio; LDA: low-dose aspirin; HCQ: hydroxychloroquine; LMWH: low-molecular-weight heparin; DOACs: direct oral anticoagulant drugs.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Diana Paredes-Ruiz, Daniel Martin-Iglesias, Guillermo Ruiz-Irastorza" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Diana" "apellidos" => "Paredes-Ruiz" ] 1 => array:2 [ "nombre" => "Daniel" "apellidos" => "Martin-Iglesias" ] 2 => array:2 [ "nombre" => "Guillermo" "apellidos" => "Ruiz-Irastorza" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S002577532400157X?idApp=UINPBA00004N" "url" => "/00257753/00000163000000S1/v2_202409180553/S002577532400157X/v2_202409180553/en/main.assets" ] "en" => array:19 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Review</span>" "titulo" => "Catastrophic antiphospholipid syndrome: Lessons from the “CAPS Registry”" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "S31" "paginaFinal" => "S35" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Ignasi Rodriguez-Pintó, Gerard Espinosa, Ricard Cervera" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Ignasi" "apellidos" => "Rodriguez-Pintó" ] 1 => array:2 [ "nombre" => "Gerard" "apellidos" => "Espinosa" ] 2 => array:4 [ "nombre" => "Ricard" "apellidos" => "Cervera" "email" => array:1 [ 0 => "rcervera@clinic.cat" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Department of Autoimmune Diseases, Reference Centre for Systemic Autoimmune Diseases (UEC, CSUR) of the Catalan and Spanish Health Systems/Member of ERN-ReCONNET, Hospital Clínic, Barcelona, Catalonia, Spain" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome antifosfolipídico catastrófico: lecciones del <span class="elsevierStyleItalic">Catastrophic antiphospholipid syndrome</span> <span class="elsevierStyleItalic">registry</span>" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1982 "Ancho" => 3341 "Tamanyo" => 399138 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Treatment algorithm for CAPS.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Antiphospholipid syndrome (APS) is a multisystem autoimmune disease characterized by vascular thrombosis and/or pregnancy loss associated with the finding of persistent circulating antiphospholipid antibodies (aPL).<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">1</span></a> Its more severe form – although affecting a minority of patients – is known as catastrophic APS (CAPS). CAPS stands for a life-threatening clinical condition where multiple organ involvement takes place time simultaneously or over a short period of time by microvascular but also by large-vessel thrombosis.<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">2</span></a> The most classical features are a rapid evolution to multiple organ dysfunction, the association to microangiopathic features, the evidence of systemic inflammation, and a high mortality rate despite optimal therapy. Most of our knowledge has been provided by the study of the cases included in the “CAPS Registry”. This is a database created in 2000 under the auspices of the European Forum on Antiphospholipid Antibodies that includes nowadays more than 1000 cases published in the medical literature or reported to the coordinators (the authors of this review article) during the last three decades.<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">3</span></a> This article aims to review what we have learned about CAPS based on the periodical analysis of this registry.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Epidemiology</span><p id="par0010" class="elsevierStylePara elsevierViewall">Classically, CAPS has been considered as an ultrarare disease. However, the epidemiological studies on CAPS are very scarce. In the “Europhospholipid Project”, the largest epidemiological project in APS published to date, CAPS accounted for around 1% of APS cases.<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">4</span></a> After 10 years follow-up of 1000 patients with APS included in the “Europhospholipid Project”, only 9 cases developed CAPS.<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">5</span></a> This would indicate an incidence of 90 cases per 100,000 patients with APS per year.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Pathophysiology</span><p id="par0015" class="elsevierStylePara elsevierViewall">The pathogenesis of CAPS is poorly understood. Given the low incidence and the high mortality rate of CAPS, few studies have had the opportunity to collect adequate samples to drive mechanistic studies.</p><p id="par0020" class="elsevierStylePara elsevierViewall">β2-Glycoptrotein (β2GPI) is thought to be the main autoantigen in APS and probably in CAPS.<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">6</span></a> However, the presence of antibodies alone does not cause thromboembolic manifestations in aPL carriers. Up to date, it is still unclear why some patients develop large-vessels thrombosis confined to a single site (classic APS), while others develop multiple clots affecting mainly small vessels simultaneously or over a short period of time (CAPS). However, as most cases of CAPS report a precipitating factor, it has been postulated that several contributing factors need to converge in a patient to result in this clinical picture.<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">7</span></a> Over the last decades, a combination of genetic, environmental, and inflammatory factors have been recognized as necessary contributors to develop CAPS.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">8</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The presence of circulating aPL is recognized as a thrombophilic state. The molecular mimicry hypothesis postulates that these antibodies are developed after the contact of immune defenses with some microorganisms or peptides that share some amino acid sequences with β2GPI.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">9</span></a> In this way, several skin, lung, and urinary infections as well as some viruses have been associated to the appearance of aPL. However, anti-β2GPI antibodies do not bind to unstimulated endothelium <span class="elsevierStyleItalic">in vivo</span>.<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">10</span></a><span class="elsevierStyleItalic">In vitro</span> studies have shown that these antibodies are able to upregulate tissue factor expression in monocytes and endothelial cells.<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">11</span></a> More recently, it has been disclosed that APS patients have also higher plasma levels of soluble P-selectin, CD40 ligand, vascular cell adhesion molecule 1, and monocyte chemoattractant protein 1.<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">12</span></a> However, the thrombotic storm present in CAPS seems to require other triggers to take place. Interestingly, CAPS follows some prothrombotic situation in 65% of cases.<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a> Most are infections but also surgical procedures or are associated with concomitant malignant disease. Not surprisingly, CAPS patients have been shown to have higher levels of soluble P-selectin and von Willebrand factor during the event than during their quiescent stage of disease and it is well-known that these molecules are released when the immune system is activated.<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">12</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The role of complement has also been increasingly recognized in APS over the last decades. Further, Chaturvedi et al. were able to find complement regulatory gene variants in 60% of patients with CAPS, a proportion very similar to that observed in patients with atypical hemolytic uremic syndrome, and a higher frequency compared to patients with thrombotic APS, SLE or healthy controls.<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">14</span></a> Thus, a pathogenic role of these complement regulatory variants on the development of CAPS has also been proposed.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Clinical features</span><p id="par0035" class="elsevierStylePara elsevierViewall">One of the most characteristic findings in CAPS patients is the frequent association with a triggering factor. They have been identified in more than 60% of patients with CAPS. CAPS follows an infection in half of the cases, but malignant disease and surgeries are other prothrombotic situations also often related to development of CAPS. Less frequent is the association of CAPS with pregnancy or puerperium, drugs or SLE flares.<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Infections are more often reported in pediatric cases while malignancies are more often reported in elderly and males. Although almost any infection has been linked to the development of CAPS, airway infections are the most frequent, followed by urinary tract and skin infections.<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">15</span></a> Malignant disease associated to CAPS tend to be from the hematological spectrum including lymphoma, lymphocytic and myelocytic leukemia. However, there are also cases associated with lung and colon carcinoma.<a class="elsevierStyleCrossRef" href="#bib0245"><span class="elsevierStyleSup">16</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Approximately, two thirds of patients with CAPS are women. Their frequency peaks in their fourth decade of live, although a broad age range is recognized and there are cases reported from the early childhood as well as in the late elderly years. Half of them have never had any symptom of APS but one third fulfills SLE classificatory criteria, especially the younger patients. <a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">CAPS is understood as a thrombotic storm were thrombosis affects any organ system However, some clinical manifestations are thought not to be driven directly by the thrombotic occlusion of the vessels that irrigate the organ but by the cytokine storm that follows the inflammation in several necrotic sites. In this way, clinical manifestations have been traditionally categorized into those attributed to the thrombotic events and those explained by the systemic inflammatory response syndrome although this distinction is often not easy.</p><p id="par0055" class="elsevierStylePara elsevierViewall">The frequency of clinical manifestations in CAPS patients is shown in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>.<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a> According to the “CAPS Registry”, most patients with CAPS have renal involvement with several degrees of renal failure, arterial hypertension, and proteinuria but also some develop renal infarction or venous renal thrombosis. Not surprising, most common pathological finding is acute renal microangiopathy.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0060" class="elsevierStylePara elsevierViewall">Two thirds of patients with CAPS have pulmonary involvement sometimes with diffuse alveolar hemorrhage often linked to laboratory features of thrombotic microangiopathy anemia, hypocomplementemia, and triple aPL positivity. More sporadically some patients develop acute respiratory distress due to the systemic inflammatory response while others present with classical pulmonary emboli. <a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Nervous system involvement is reported in more than half of cases due to stroke and/or encephalopathy.<a class="elsevierStyleCrossRef" href="#bib0250"><span class="elsevierStyleSup">17</span></a> In patients with focal neurological deficits a thrombotic stroke can be often proven, and these cases are usually the ones that report seizures during the event. More difficult is to discern if those patients that present encephalopathy have either microangiopathic thrombosis involving the brain or their encephalopathy is attributable to the cytokine storm.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Likewise, half of cases with CAPS present cardiac manifestations mainly due to heart infarct, angina, valvopathy or Libman Sacks endocarditis. Valves more often affected are those on the left side of the heart (mitral and aortic valves). There are some case reports of intracavitary thrombosis during the CAPS event. <a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Cutaneous involvement is also very frequent in patients with CAPS. They can present with livedo reticularis or racemosa, but some evolve leading to skin necrosis with ulcers and/or digital ischemia.<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a> Splinter hemorrhages have been described in a small percentage of cases.</p><p id="par0080" class="elsevierStylePara elsevierViewall">Less frequent is the involvement of the gastrointestinal tract. Liver is thought to be involved in 40% of cases. However, histological evidence is scarce in the literature and most cases only report liver test elevation that can be induced by a decrease in blood flow because of other reasons than microangiopathic thrombosis or due to drug toxicity during the clinical event. Several reports have shown how CAPS can involve the intestinal or the spleen leading to intestinal infarction or spleen failure. <a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Peripheral vessels thrombosis might be venous or arterial but venous thrombosis is more often reported in the pediatric cases while arterial thrombosis is more often reported in elderly cases.<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">19</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">There are some reports of pancreas, kidneys, testes, and bone marrow infarctions, although the attribution of these clinical manifestations to CAPS is unclear. <a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Differential diagnosis</span><p id="par0095" class="elsevierStylePara elsevierViewall">CAPS diagnostic is often a challenging situation because acute onset of multiple thrombosis and organ dysfunction with thrombocytopenia and microangiopathic hemolytic anemia is often seen in severe ill patients. This clinical picture is more often observed in patients with sepsis with or without disseminated intravascular coagulation (DIC) but also in other rare clinical microangiopathic syndromes. These include thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, heparin induced thrombocytopenia, and HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome among others.<a class="elsevierStyleCrossRefs" href="#bib0265"><span class="elsevierStyleSup">20,21</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">To facilitate this challenging situation, preliminary classification criteria were proposed during the 10th International Congress on aPL in 2002 that were later validated in 2005.<a class="elsevierStyleCrossRefs" href="#bib0275"><span class="elsevierStyleSup">22,23</span></a> A definite CAPS was defined as those cases that have thrombosis in three or more organs over a period of less than a week, biopsy proven microthrombosis in at least one organ, and persistent aPL positivity (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>). The authors of these criteria also proposed which patients could be considered as having probable CAPS. It was so done aiming to keep clinicals on a high alert, due to the rapidly progressive nature of CAPS.</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0105" class="elsevierStylePara elsevierViewall">However, still the differential diagnosis of CAPS is difficult due to the finding of circulating aPL in other clinical conditions and specially in infections and malignant disease.<a class="elsevierStyleCrossRef" href="#bib0285"><span class="elsevierStyleSup">24</span></a> Furthermore, some cases present with clinical features of CAPS together with an infection that led to a DIC, thus making the differential diagnosis between both entities almost impossible. Additionally, patients with thrombotic thrombocytopenia purpura or hemolytic uremic syndrome might develop aPL together with some or all the classical clinical features of renal and neurologic involvement and microangiopathic features (anemia, thrombocytopenia and schistocytes).<a class="elsevierStyleCrossRefs" href="#bib0290"><span class="elsevierStyleSup">25,26</span></a> The presence of antibodies against ADAMTS13 might give the clue to differentiate between them, although it is not always easy.</p><p id="par0110" class="elsevierStylePara elsevierViewall">The search of malignancies is also recommended in CAPS cases. CAPS is associated to malignant diseases which often are hematologic diseases, while solid organ malignancies that have more often been associated to TMA are gastric, breast, prostate, and lung cancer.<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">27</span></a></p><p id="par0115" class="elsevierStylePara elsevierViewall">A recommended set of complementary tests in those patients with suspicion of CAPS and microangiopathic features has been published and can be found in <a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>.</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Treatment</span><p id="par0120" class="elsevierStylePara elsevierViewall">Three therapeutic measures have been proposed as the backbone to manage patients with CAPS. First, the general supportive measures; second, the treatment of any identifiable precipitating factor; and, finally, the so-called specific treatment (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0125" class="elsevierStylePara elsevierViewall">Many patients end up in intensive care units due to multiorgan failure.<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">28</span></a> Thus, appropriate supportive care, according to the organs involved, is mandatory. In this sense, inotropic drugs might be required in cases with unstable hemodynamics, mechanical ventilation might be needed in those with severe respiratory failure and, especially, in those that develop acute respiratory distress syndrome and hemodialysis is needed in cases of severe renal failure.</p><p id="par0130" class="elsevierStylePara elsevierViewall">A precipitating factor is reported in up to 65% of cases with CAPS but they can also favor the evolution of new thrombotic events.<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a> Therefore, early identification and an aggressive treatment of any triggering factor has been recommended trying to decrease the thrombophilic state associated to those situations. Infections are the situations more often reported to precede the catastrophic event, but surgeries are also frequently reported. Thus, antibiotic treatment is mandatory in those cases where an infection can be identified, and special caution is recommended in patients with APS or aPL carriers in perioperative situations.</p><p id="par0135" class="elsevierStylePara elsevierViewall">The specific therapeutic approach to CAPS is based on the pathophysiologic events that are postulated to take place during the catastrophic episode. However, CAPS physiopathology is not yet clear, since only a small number of mechanistic studies have been conducted. Current guidelines were established long time ago, after the analysis of the first 130 cases of the “CAPS Registry” (those CAPS patients included in the registry at that time had been treated according to the preferences of their physicians in charge).<a class="elsevierStyleCrossRef" href="#bib0275"><span class="elsevierStyleSup">22</span></a> The highest survival rate was achieved with the combination of the so-called triple therapy: a combination of anticoagulation, glucocorticoids, and plasma exchange and/or intravenous immunoglobulins.<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">29</span></a> However, Bayraktar et al. showed that, in those patient with CAPS that had also SLE, the addition of cyclophosphamide to the standard treatment achieved a better survival rate.<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">30</span></a></p><p id="par0140" class="elsevierStylePara elsevierViewall">A window of opportunity is opening in many autoimmune diseases with the advent of biologic therapies to the current practice. However, the therapeutic role of biologics in CAPS is still to be defined. Rituximab has been used as adjuvant treatment showing some benefit in several CAPS patients,<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">31</span></a> although the group of patients that have received this treatment is small and the benefit is difficult to clear up.</p><p id="par0145" class="elsevierStylePara elsevierViewall">The increasing evidence of the role of complement in APS has prompted some clinicians to use eculizumab in CAPS patients, mainly in those with microangiopathic features, because this anti-complement biologic therapy is already used in other microangiopathic conditions such as paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome. Although the number of CAPS patients who have received eculizumab is still small, most of them recovered (29 out of 39). However, this finding should be taken with caution since it might be driven by the enthusiasm of those that had good results to publish their cases.<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">32</span></a></p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Prognosis</span><p id="par0150" class="elsevierStylePara elsevierViewall">Long-term prognosis of patients who survive the initial catastrophic event was analyzed in a study of the “CAPS Registry” that demonstrated that 66% of them remained symptom-free with anticoagulation during an average follow-up of 67.2 months.<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">33</span></a> However, 26% of survival patients developed further APS-related thrombosis after the initial CAPS event. Furthermore, 15% of the patients were functionally impaired because of CAPS, including cardiac failure, chronic renal insufficiency, gait abnormalities and visual symptoms. However, prognostic factors that predict long-term outcome of patients who suffer a CAPS event are unknown.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Ethical considerations</span><p id="par0155" class="elsevierStylePara elsevierViewall">Written informed consent was not required.</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Funding</span><p id="par0160" class="elsevierStylePara elsevierViewall">No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article.</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Conflicts of interest</span><p id="par0165" class="elsevierStylePara elsevierViewall">The authors declare no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:15 [ 0 => array:3 [ "identificador" => "xres2245406" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1878067" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres2245407" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1878068" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Epidemiology" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Pathophysiology" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Clinical features" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Differential diagnosis" ] 9 => array:2 [ "identificador" => "sec0030" "titulo" => "Treatment" ] 10 => array:2 [ "identificador" => "sec0035" "titulo" => "Prognosis" ] 11 => array:2 [ "identificador" => "sec0040" "titulo" => "Ethical considerations" ] 12 => array:2 [ "identificador" => "sec0045" "titulo" => "Funding" ] 13 => array:2 [ "identificador" => "sec0050" "titulo" => "Conflicts of interest" ] 14 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2023-10-08" "fechaAceptado" => "2024-02-22" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1878067" "palabras" => array:10 [ 0 => "Catastrophic antiphospholipid syndrome" 1 => "Therapeutics" 2 => "Anticoagulation" 3 => "Glucocorticoids" 4 => "Plasma exchange" 5 => "Intravenous immunoglobulins" 6 => "Rituximab" 7 => "Eculizumab" 8 => "Thrombotic microangiopathy" 9 => "Cytokine storm" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1878068" "palabras" => array:10 [ 0 => "Síndrome antifosfolipídico catastrófico" 1 => "Terapéutica" 2 => "Anticoagulación" 3 => "Glucocorticoides" 4 => "Recambios plasmáticos" 5 => "Inmunoglobulinas intravenosas" 6 => "Rituximab" 7 => "Eculizumab" 8 => "Microangiopatía trombótica" 9 => "Tormenta de citocinas" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening clinical condition that represents the most severe clinical presentation of the antiphospholipid syndrome (APS). It was first described in 1992 in a group of patients that presented with multiorgan involvement and microangiopathic features of APS. Most of the current knowledge of CAPS comes from the analysis of all cases collected at the “CAPS Registry” that was created in 2000 to perform studies on this condition. Most cases are triggered by a prothrombotic situation that leads to a multiorgan thrombosis and a cytokine storm. The analysis of cases included in the “CAPS Registry” has shown that the triple therapy with anticoagulation, glucocorticoids, and plasma exchange and/or intravenous immunoglobulins is associated to a better prognosis of CAPS. The improvement of the knowledge allowed a decrease from the 50% mortality rate reported in the first series to 25–30% in the most recent publications.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El síndrome antifosfolipídico catastrófico (SAFC) es una entidad clínica minoritaria que pone en peligro la vida, y representa la presentación clínica más grave del síndrome antifosfolipídico (SAF). Se describió por primera vez en 1992 en un grupo de pacientes que presentaban afectación multiorgánica y manifestaciones microangiopáticas del SAF. La mayor parte del conocimiento actual sobre el SAFC proviene del análisis de todos los casos recopilados en el <span class="elsevierStyleItalic">Catastrophic antiphospholipid syndrome</span><span class="elsevierStyleItalic">registry</span> («<span class="elsevierStyleItalic">CAPS Registry</span>»), que fue creado en el año 2000 para realizar estudios sobre esta entidad. La mayoría de los casos se desencadenan por una situación protrombótica que conduce a una trombosis multiorgánica y una tormenta de citocinas. El análisis de los casos incluidos en el «<span class="elsevierStyleItalic">CAPS Registry</span>» ha demostrado que la triple terapia con anticoagulación, glucocorticoides y recambios plasmáticos y/o inmunoglobulinas intravenosas se asocia a un mejor pronóstico del SAFC. La mejora del conocimiento ha permitido disminuir la tasa de mortalidad del 50% descrita en la primera serie al 25-30% en las publicaciones más recientes.</p></span>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1982 "Ancho" => 3341 "Tamanyo" => 399138 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Treatment algorithm for CAPS.</p>" ] ] 1 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Organ involved \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">% \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Kidney (N</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">518)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">73 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Renal failure \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">77 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Proteinuria \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">29 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Arterial hypertension \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">24 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Hematuria \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">16 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Lung (N</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">517)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">60 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Acute respiratory distress syndrome \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">36 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Pulmonary embolism \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">26 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Alveolar hemorrhage \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">12 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Pulmonary edema \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Brain (N</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">515)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">56 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Stoke \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">40 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Encephalopathy \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">39 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Seizures \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">15 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Headache \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Heart (N</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">515)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">50 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Heart failure \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">44 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Myocardial infarction \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">30 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Valvulopathy \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">28 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Libman sacks endocarditis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">13 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Skin (N</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">517)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">47 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Livedo reticularis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">43 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Cutaneous necrosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">26 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Cutaneous ulcers \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">24 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Purpura \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">14 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Liver (N</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">515)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">39 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Elevated liver enzymes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">63 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Hepatomegaly \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Liver failure \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">9 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Jaundice \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">7 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Peripheral vessel (N</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">515)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">37 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Peripheral venous thrombosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">69 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Peripheral arterial thrombosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">46 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Gastrointestinal involvement</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">24 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Gastrointestinal bleeding \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">18 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Ileum \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Spleen (N</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">513)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">18 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Adrenal glands (N</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">510)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab3658956.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Clinical manifestations in patients with CAPS (personal data from the “CAPS Registry”).</p>" ] ] 2 => array:8 [ "identificador" => "tbl0010" "etiqueta" => "Table 2" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at2" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1. Evidence of involvement of three or more organs, systems, and/or tissues \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2. Development of manifestations simultaneously or in less than a week \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3. Confirmation by histopathology of small vessel occlusion in at least one organ or tissue \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4. Laboratory confirmation of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibodies, and/or anti-β2 GPI antibodies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Definite CAPS requires all 4 criteria \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Probable CAPS is based on any of the following: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">All four criteria, except for only two organs, systems, and/or sites of tissue involvement \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">All four criteria, except for the laboratory confirmation at least six weeks apart due to the early death of a patient never previously tested for aPL \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Criteria 1, 2, and 4 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Criteria 1, 3, and 4 and the development of a third event in more than a week but less than one month, despite anticoagulation \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab3658957.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Classification criteria for CAPS.</p>" ] ] 3 => array:8 [ "identificador" => "tbl0015" "etiqueta" => "Table 3" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at3" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleItalic">Abbreviations</span>: LA, lupus anticoagulant, aCL, anticardiolipin, aβ2GPI, anti-β2-glycoprotein, HPF4, heparin-platelet factor 4.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Peripheral blood smear \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Chest X-ray \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Funduscopic examination \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• ADAMTS13 activity \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Stool culture \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Anti-nuclear, anti-DNA, anti-Sm, and anti-RNP antibodies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Antiphospholipid antibodies (LA, aCL IgG and IgM, aβ2GPI IgG and IgM) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Bone marrow biopsy \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Positron emission tomography-computed tomography \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Anti-HPF4 antibodies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Biopsy of affected tissues/organs \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">• Complement factor H, factor B, and factor I levels \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab3658958.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Selected tests for patients with microangiopathic features and suspicion of CAPS.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:33 [ 0 => array:3 [ "identificador" => "bib0170" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Diagnosis and management of the antiphospholipid syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "D. 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Journal Information
Review
Catastrophic antiphospholipid syndrome: Lessons from the “CAPS Registry”
Síndrome antifosfolipídico catastrófico: lecciones del Catastrophic antiphospholipid syndrome registry
Ignasi Rodriguez-Pintó, Gerard Espinosa, Ricard Cervera
Corresponding author
Department of Autoimmune Diseases, Reference Centre for Systemic Autoimmune Diseases (UEC, CSUR) of the Catalan and Spanish Health Systems/Member of ERN-ReCONNET, Hospital Clínic, Barcelona, Catalonia, Spain