116:
inflammatory response syndrome (SIRS). Peripheral thrombosis may be encountered affecting veins and arteries. Intra-abdominal thrombosis may lead to pain. The body contains β2-GPI, a glycoprotein in the blood, which is considered a natural anticoagulant due to its inhibitory effects on certain aspects of platelet synthesis and function. Because the formation of anti-β2-GPI antibodies can occur after exposure to bacteria, the body favors a hypercoagulable state, which has been noted to activate toll-like receptor 4 resulting in what is known as a cytokine storm or a thrombotic storm. A thrombotic storm may also occur due to the following precipitating events: alterations in coagulation and fibrinolysis, which induce high mortality rates, and infections amongst pediatric patients where IgM and IgG anti-β2-GPI antibodies induce an endothelial signal, leading to a procoagulant state. It is also hypothesized that thrombotic storms occur due to prothrombotic genetic risk factors, which trigger a sped-up form of thrombosis after its first occurrence, rather than being caused solely by environmental factors. Cardiovascular, nervous, kidney, and lung system complications are common. More specifically with the heart, Asherson's syndrome can lead to complications such as mitral valve regurgitation (MVR) in which the mitral valve does not shut properly allowing backflow of blood into the heart as well as angina (chest pain) and myocardial infarction (heart attack). Furthermore, complications in the kidneys may occur, including low urine production and high blood pressure, while complications with the lungs can result in rapid breathing (hyperventilation) and low oxygen levels (hypoxemia). The affected individual may exhibit skin
55:
161:. Patients with high aPL (positive antiphospholipid antibodies) must have microthrombosis in multiple organs to definitively be diagnosed with CAPSâa high antilipid antibody count is not sufficient for diagnosis. To be diagnosed with CAPS, a patient must have three or more new organ thromboses developing within less than a week, and a
165:
must be later performed testing for microthrombosis in order to accurately diagnose the patient with 'definite CAPS'. If the patient has two or less new organ thromboses within a week but a biopsy still indicates the presence of microthrombosis, the patient is not considered to have CAPS. Positive
115:
Clinically, the syndrome affects at least three organs and may affect many organ systems. The syndrome usually occurs with small vessel thromboses affecting organ systems such as the gastrointestinal tract and manifestations of the acute respiratory distress syndrome (ARDS), a type of systemic
216:
As a disease associated with high morbidity and mortality, CAPS requires an aggressive multidisciplinary treatment strategy. The following treatments have been used in various combinations: anticoagulation, glucocorticoids, plasma exchange, cyclophosphamide, intravenous immunoglobulins, and
139:
Furthermore, the syndrome has been shown to manifest multi-organ failures and miscarriages in the context of pregnancies. 54% of pregnant mothers with the disease experienced fetal loss, and around 50% of pregnant mothers died from complications associated with the syndrome.
148:
Two specific types of blood tests are used to aid in the diagnosis of
Asherson's syndrome. A coagulation blood test is used to measure and determine the blood's ability to clot and how fast it takes to clot, indicating the presence of lupus anticoagulant in the blood. An
435:
Ortel, Thomas L.; Kitchens, Craig S.; Erkan, Doruk; BrandĂŁo, Leonardo R.; Hahn, Susan; James, Andra H.; Kulkarni, Roshni; Manco-Johnson, Marilyn J.; Pericak-Vance, Margaret; Vance, Jeffery (1 December 2012). "Clinical causes and treatment of the thrombotic storm".
217:
anti-platelet agents. Anticoagulation is given to stop the thrombophilic state, and it promotes the clot breakdown, while steroids downregulate the cytokine storm that is thought to be responsible for systemic inflammatory response syndrome (SIRS).
552:
GĂłmezâPuerta, JosĂŠ A; Cervera, Ricard; Espinosa, Gerard; Asherson, Ronald A; GarcĂaâCarrasco, Mario; da Costa, Izaias P; Andrade, Danieli C O; Borba, Eduardo F; Makatsaria, Alexander; Bucciarelli, Silvia; RamosâCasals, Manuel (June 2007).
231:
Recent investigational treatment therapies include high doses of
Rituxan (Rituximab) and eculizumab, which are both humanized monoclonal antibodies that target B cell malignancies and prevent C5 complement cleavage,
856:
166:
test are often repeated due to the fact that antilipid antibodies can be present in the body for short stints due to infection or drug use. Along with that, individuals may or may not have a history of
91:, platelets, and immune cells, ultimately causing a large inflammatory immune response and widespread clotting. CAPS was first described by Ronald Asherson in 1992. The syndrome exhibits
343:
Garcia-Carrasco, M.; Mendoza-Pinto, C.; Macias-Diaz, S.; Vazquez de Lara, F.; Etchegaray-Morales, I.; Galvez-Romero, J.L.; Mendez-Martinez, S.; Cervera, R. (2015-07-22).
76:
79:
causes multi-organ failure. The syndrome is caused by antiphospholipid antibodies that target a group of proteins in the body that are associated with
686:
Erkan, Doruk; Espinosa, Gerard; Cervera, Ricard (December 2010). "Catastrophic antiphospholipid syndrome: Updated diagnostic algorithms".
720:
174:. Association with another disease such as lupus is called a secondary APS unless it includes the defining criteria for CAPS.
754:
409:
157:
antibodies' presence in the blood. Individuals with CAPS often exhibit a positive test to antilipid antibodies, typically
555:"Catastrophic antiphospholipid syndrome during pregnancy and puerperium: maternal and fetal characteristics of 15 cases"
150:
913:
886:
92:
344:
171:
104:
54:
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may occur due to the arterial clotting involvement. Death may result from multiple organ failure.
305:
40:
908:
611:
479:
129:
225:
8:
679:
639:"Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease"
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44:
918:
814:
781:
765:
734:
663:
638:
587:
554:
461:
420:
389:
281:
256:
342:
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801:
750:
703:
668:
592:
574:
534:
526:
465:
453:
405:
364:
325:
286:
88:
35:
809:
793:
760:
742:
695:
658:
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582:
566:
516:
445:
415:
397:
356:
317:
276:
268:
521:
797:
746:
699:
401:
360:
321:
206:
191:
154:
96:
345:"The role of infectious diseases in the catastrophic antiphospholipid syndrome"
221:
210:
202:
121:
902:
805:
654:
578:
530:
100:
80:
570:
823:
707:
672:
596:
538:
457:
368:
329:
290:
48:
388:
RodrĂguez-PintĂł, Ignasi; Espinosa, Gerard; Cervera, Ricard (2014-11-07).
272:
848:
187:
84:
449:
220:
Additional steps may have to be taken to manage circulatory problems,
881:
551:
780:
Kazzaz, Nayef M.; McCune, W. Joseph; Knight, Jason S. (May 2016).
198:
125:
117:
860:
505:"Kidney disease in primary anti-phospholipid antibody syndrome"
162:
133:
732:
387:
257:"Catastrophic antiphospholipid syndrome: a clinical review"
434:
306:"The catastrophic antiphospholipid (Asherson's) syndrome"
158:
733:
RodrĂguez-PintĂł, I.; Espinosa, G.; Cervera, R. (2014).
782:"Treatment of catastrophic antiphospholipid syndrome"
838:
685:
637:
Aguiar, Cassyanne L.; Erkan, Doruk (December 2013).
502:
75:, is a rare autoimmune disease in which widespread,
503:Gracia-Tello, Borja; Isenberg, David (2017-07-01).
779:
197:Specific therapy includes the use of intravenous
120:and necrosis. Cerebral manifestations may lead to
900:
95:, multiple organ thromboses, and in some cases
741:. Rare Diseases of the Immune System: 249â262.
643:Therapeutic Advances in Musculoskeletal Disease
484:NORD (National Organization for Rare Disorders)
396:. Rare Diseases of the Immune System: 249â262.
250:
248:
246:
735:"The Catastrophic Antiphospholipid Syndrome"
630:
390:"The Catastrophic Antiphospholipid Syndrome"
182:Treatments may involve the following steps:
636:
254:
243:
53:
813:
764:
662:
586:
520:
419:
280:
303:
616:National Organization of Rare Disorders
901:
65:Catastrophic antiphospholipid syndrome
22:Catastrophic antiphospholipid syndrome
304:Asherson, Ronald A. (December 2006).
255:Nayer, Ali; Ortega, Luis M. (2014).
13:
739:Antiphospholipid Antibody Syndrome
394:Antiphospholipid Antibody Syndrome
14:
930:
834:
151:enzyme-linked immunosorbent assay
609:
559:Annals of the Rheumatic Diseases
786:Current Opinion in Rheumatology
773:
726:
714:
186:Prevention includes the use of
153:(ELISA) test is done to detect
110:
103:or catastrophic variant of the
603:
545:
496:
472:
428:
381:
336:
297:
1:
236:
190:for infection and parenteral
798:10.1097/BOR.0000000000000269
747:10.1007/978-3-319-11044-8_20
700:10.1016/j.autrev.2010.08.005
402:10.1007/978-3-319-11044-8_20
361:10.1016/j.autrev.2015.07.009
322:10.1016/j.autrev.2006.06.005
177:
143:
7:
522:10.1093/rheumatology/kew307
438:Expert Review of Hematology
10:
935:
261:Journal of Nephropathology
93:thrombotic microangiopathy
872:
842:
194:for susceptible patients.
172:connective tissue disease
105:antiphospholipid syndrome
34:
26:
21:
655:10.1177/1759720X13502919
914:Intensive care medicine
723:Retrieved on 2009-06-02
571:10.1136/ard.2006.061671
41:Intensive care medicine
130:Myocardial infarctions
77:intravascular clotting
612:"Asherson's Syndrome"
480:"Asherson's Syndrome"
99:and is considered an
688:Autoimmunity Reviews
349:Autoimmunity Reviews
310:Autoimmunity Reviews
273:10.12860/jnp.2014.03
226:respiratory distress
73:Asherson's syndrome
45:otorhinolaryngology
873:External resources
896:
895:
756:978-3-319-11043-1
450:10.1586/ehm.12.56
411:978-3-319-11043-1
355:(11): 1066â1071.
89:endothelial cells
71:), also known as
62:
61:
16:Medical condition
926:
840:
839:
828:
827:
817:
777:
771:
770:
768:
730:
724:
718:
712:
711:
683:
677:
676:
666:
634:
628:
627:
625:
623:
607:
601:
600:
590:
549:
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542:
524:
515:(7): 1069â1080.
500:
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493:
491:
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476:
470:
469:
432:
426:
425:
423:
385:
379:
378:
376:
375:
340:
334:
333:
301:
295:
294:
284:
252:
207:plasma exchanges
58:
57:
30:Catastrophic APS
19:
18:
934:
933:
929:
928:
927:
925:
924:
923:
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851:
837:
832:
831:
778:
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757:
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727:
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715:
684:
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635:
631:
621:
619:
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550:
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429:
412:
386:
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373:
371:
341:
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302:
298:
253:
244:
239:
205:, and possibly
203:corticosteroids
192:anticoagulation
180:
155:anticardiolipin
146:
113:
97:tissue necrosis
52:
17:
12:
11:
5:
932:
922:
921:
916:
911:
894:
893:
890:
889:
877:
876:
874:
870:
869:
866:
865:
852:
847:
846:
844:
843:Classification
836:
835:External links
833:
830:
829:
792:(3): 218â227.
772:
755:
725:
713:
678:
649:(6): 305â314.
629:
602:
565:(6): 740â746.
544:
495:
471:
444:(6): 653â659.
427:
410:
380:
335:
296:
241:
240:
238:
235:
234:
233:
229:
222:kidney failure
218:
214:
211:immunoglobulin
209:, intravenous
195:
179:
176:
145:
142:
122:encephalopathy
112:
109:
60:
59:
38:
32:
31:
28:
24:
23:
15:
9:
6:
4:
3:
2:
931:
920:
917:
915:
912:
910:
907:
906:
904:
888:
884:
883:
879:
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871:
863:
862:
858:
854:
853:
850:
845:
841:
825:
821:
816:
811:
807:
803:
799:
795:
791:
787:
783:
776:
767:
762:
758:
752:
748:
744:
740:
736:
729:
722:
717:
709:
705:
701:
697:
693:
689:
682:
674:
670:
665:
660:
656:
652:
648:
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633:
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584:
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576:
572:
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564:
560:
556:
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523:
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510:
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499:
485:
481:
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251:
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232:respectively.
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81:phospholipids
78:
74:
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909:Rheumatology
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728:
716:
694:(2): 74â79.
691:
687:
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646:
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632:
620:. Retrieved
615:
605:
562:
558:
547:
512:
509:Rheumatology
508:
498:
487:. Retrieved
483:
474:
441:
437:
430:
393:
383:
372:. Retrieved
352:
348:
338:
316:(2): 64â67.
313:
309:
299:
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181:
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111:Presentation
72:
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63:
49:rheumatology
622:29 November
267:(1): 9â17.
188:antibiotics
170:or another
132:may occur.
27:Other names
903:Categories
721:MD Consult
489:2020-12-04
374:2022-11-13
237:References
85:antibodies
919:Syndromes
806:1040-8711
579:0003-4967
531:1462-0324
466:207211666
178:Treatment
144:Diagnosis
87:activate
36:Specialty
882:Orphanet
824:26927441
708:20696282
673:24294304
597:17223653
539:27550302
458:23216595
369:26209907
330:17138244
291:24644537
126:seizures
83:. These
864:: D68.6
815:4958413
766:7153043
664:3836378
588:1954660
421:7153043
282:3956908
199:heparin
134:Strokes
118:purpura
101:extreme
887:464343
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618:. NORD
610:NORD.
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224:, and
163:biopsy
51:
462:S2CID
168:lupus
820:PMID
802:ISSN
751:ISBN
704:PMID
669:PMID
624:2020
593:PMID
575:ISSN
535:PMID
527:ISSN
454:PMID
406:ISBN
365:PMID
326:PMID
287:PMID
201:and
124:and
69:CAPS
857:ICD
810:PMC
794:doi
761:PMC
743:doi
696:doi
659:PMC
651:doi
583:PMC
567:doi
517:doi
446:doi
416:PMC
398:doi
357:doi
318:doi
277:PMC
269:doi
159:IgG
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