Skip to main content
Top
Published in: Clinical Reviews in Allergy & Immunology 2-3/2009

01-06-2009

Catastrophic Antiphospholipid Syndrome: Treatment, Prognosis, and the Risk of Relapse

Authors: Silvia Bucciarelli, Doruk Erkan, Gerard Espinosa, Ricard Cervera

Published in: Clinical Reviews in Allergy & Immunology | Issue 2-3/2009

Login to get access

Abstract

The “catastrophic” variant of the antiphospholipid syndrome (APS) is characterized by multiple vascular occlusive events, usually affecting small vessels and developing over a short period of time. Although patients with catastrophic APS represent less than 1% of all patients with APS, they are usually in a life-threatening situation with a 50% mortality rate. The purpose of this paper is to review the treatment strategies and prognostic factors in patients with catastrophic APS. A detailed description of the clinical and laboratory features of the syndrome can be found in the other articles of this issue.
Literature
1.
go back to reference Asherson RA (1992) The catastrophic antiphospholipid syndrome. J Rheumatol 19:508–512PubMed Asherson RA (1992) The catastrophic antiphospholipid syndrome. J Rheumatol 19:508–512PubMed
2.
go back to reference Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT et al (2002) Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients. Arthritis Rheum 46:1019–1027PubMedCrossRef Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT et al (2002) Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients. Arthritis Rheum 46:1019–1027PubMedCrossRef
3.
go back to reference Asherson RA, Cervera R, Piette JC, Font J, Lie JT, Burcoglu A et al (1998) Catastrophic antiphospholipid syndrome: clinical and laboratory features of 50 patients. Medicine (Baltimore) 77:195–207CrossRef Asherson RA, Cervera R, Piette JC, Font J, Lie JT, Burcoglu A et al (1998) Catastrophic antiphospholipid syndrome: clinical and laboratory features of 50 patients. Medicine (Baltimore) 77:195–207CrossRef
4.
go back to reference Asherson RA, Cervera R, Piette JC, Shoenfeld Y, Espinosa G, Petri MA et al (2001) Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore) 80:355–376CrossRef Asherson RA, Cervera R, Piette JC, Shoenfeld Y, Espinosa G, Petri MA et al (2001) Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore) 80:355–376CrossRef
5.
go back to reference Asherson RA, Cervera R, de Groot PR, Erkan D, Boffa MC, Piette JC et al (2003) Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 12:530–534PubMedCrossRef Asherson RA, Cervera R, de Groot PR, Erkan D, Boffa MC, Piette JC et al (2003) Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 12:530–534PubMedCrossRef
6.
go back to reference Bucciarelli S, Espinosa G, Cervera R, Erkan D, Gómez-Puerta JA, Ramos-Casals M et al (2006) Mortality in catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum 54:2568–2576PubMedCrossRef Bucciarelli S, Espinosa G, Cervera R, Erkan D, Gómez-Puerta JA, Ramos-Casals M et al (2006) Mortality in catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum 54:2568–2576PubMedCrossRef
7.
go back to reference Girardi G, Redecha P, Salmon JE (2004) Heparin prevents antiphospholipid antibody-induced fetal loss by inhibiting complement activation. Nat Med 10:1222–1226PubMedCrossRef Girardi G, Redecha P, Salmon JE (2004) Heparin prevents antiphospholipid antibody-induced fetal loss by inhibiting complement activation. Nat Med 10:1222–1226PubMedCrossRef
8.
go back to reference Christman JW, Lancaster LH, Blackwell TS (1998) Nuclear factor kappa B: a pivotal role in the systemic inflammatory response syndrome and new target for therapy. Intensive Care Med 24:1131–1138PubMedCrossRef Christman JW, Lancaster LH, Blackwell TS (1998) Nuclear factor kappa B: a pivotal role in the systemic inflammatory response syndrome and new target for therapy. Intensive Care Med 24:1131–1138PubMedCrossRef
9.
go back to reference Uthman I, Shamseddine A, Taher A (2005) The role of therapeutic plasma exchange in catastrophic antiphospholipid syndrome. Transfus Apher Sci 33:11–17PubMedCrossRef Uthman I, Shamseddine A, Taher A (2005) The role of therapeutic plasma exchange in catastrophic antiphospholipid syndrome. Transfus Apher Sci 33:11–17PubMedCrossRef
10.
go back to reference Erkan D, Cervera R, Asherson RA (2003) Catastrophic antiphospholipid syndrome: where do we stand. Arthritis Rheum 48:3320–3327PubMedCrossRef Erkan D, Cervera R, Asherson RA (2003) Catastrophic antiphospholipid syndrome: where do we stand. Arthritis Rheum 48:3320–3327PubMedCrossRef
11.
go back to reference Orbach H, Katz U, Sherer Y, Shoenfeld Y (2005) Intravenous immunoglobulin: adverse effects and safe administration. Clin Rev Allergy Immunol 29:173–184PubMedCrossRef Orbach H, Katz U, Sherer Y, Shoenfeld Y (2005) Intravenous immunoglobulin: adverse effects and safe administration. Clin Rev Allergy Immunol 29:173–184PubMedCrossRef
12.
go back to reference Bayraktar UD, Erkan D, Bucciarelli S, Espinosa G, Asherson RA, Catastrophic Antiphospholipid Syndrome Project Group (2007) The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. J Rheumatol 34:346–352PubMed Bayraktar UD, Erkan D, Bucciarelli S, Espinosa G, Asherson RA, Catastrophic Antiphospholipid Syndrome Project Group (2007) The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. J Rheumatol 34:346–352PubMed
13.
go back to reference Tenedious R, Erkan D, Lockshin MD (2005) Rituximab in the primary antiphospholipid syndrome. Arthritis Rheum 52:4078 (abstract) Tenedious R, Erkan D, Lockshin MD (2005) Rituximab in the primary antiphospholipid syndrome. Arthritis Rheum 52:4078 (abstract)
14.
go back to reference Erdozain JG, Ruiz-Irastorza G, Egurbide MV et al (2004) Sustained response to rituximab of autoimmune hemolytic anemia associated with antiphospholipid syndrome. Haematologica 89:ECR34 (abstract)PubMed Erdozain JG, Ruiz-Irastorza G, Egurbide MV et al (2004) Sustained response to rituximab of autoimmune hemolytic anemia associated with antiphospholipid syndrome. Haematologica 89:ECR34 (abstract)PubMed
15.
go back to reference Rubenstein E, Arkfeld DG, Metyas S, Shinada S, Ehresmann S, Liebman HA (2006) Rituximab treatment for resistant antiphospholipid syndrome. J Rheumatol 33:355–357PubMed Rubenstein E, Arkfeld DG, Metyas S, Shinada S, Ehresmann S, Liebman HA (2006) Rituximab treatment for resistant antiphospholipid syndrome. J Rheumatol 33:355–357PubMed
16.
go back to reference Yamazaki et al (2004) Rituximab decreases antiphospholipid antibody titers and improves hypercoagulable state without recurrent thrombosis: 2-year follow-up. Thromb Res 114:610 (abstract) Yamazaki et al (2004) Rituximab decreases antiphospholipid antibody titers and improves hypercoagulable state without recurrent thrombosis: 2-year follow-up. Thromb Res 114:610 (abstract)
17.
go back to reference Tommasino et al (2004) Short-term lack of efficacy of rituximab in a thrombocytopaenic patient with primary antiphospholipid syndrome. Thromb Res 114:652 (abstract) Tommasino et al (2004) Short-term lack of efficacy of rituximab in a thrombocytopaenic patient with primary antiphospholipid syndrome. Thromb Res 114:652 (abstract)
18.
go back to reference Vero S, Asherson RA, Erkan D (2006) Critical care review: Catastrophic antiphospholipid syndrome. J Intens Care Med 21:144–159CrossRef Vero S, Asherson RA, Erkan D (2006) Critical care review: Catastrophic antiphospholipid syndrome. J Intens Care Med 21:144–159CrossRef
19.
go back to reference Erkan D, Asherson RA, Espinosa G et al (2003) Long term outcome of catastrophic antiphospholipid síndrome survivors. Ann Rheum Dis 62:530–533PubMedCrossRef Erkan D, Asherson RA, Espinosa G et al (2003) Long term outcome of catastrophic antiphospholipid síndrome survivors. Ann Rheum Dis 62:530–533PubMedCrossRef
20.
go back to reference Asherson R, Espinosa G, Menahen S et al (2008) Relapsing catastrophic antiphsopholipid syndrome: Report of three cases. Semin Arthritis Rheum 37:366–372PubMedCrossRef Asherson R, Espinosa G, Menahen S et al (2008) Relapsing catastrophic antiphsopholipid syndrome: Report of three cases. Semin Arthritis Rheum 37:366–372PubMedCrossRef
21.
go back to reference Murphy JJ, Leach IH (1989) Finding at necropsy in the heart of a patient with anticardiolipin syndrome. Br Heart J 62:61–64PubMedCrossRef Murphy JJ, Leach IH (1989) Finding at necropsy in the heart of a patient with anticardiolipin syndrome. Br Heart J 62:61–64PubMedCrossRef
22.
go back to reference Neuwelt CM, Daikh DI, Linfoot JA et al (1997) Catastrophic antiphospholipid syndrome. Responce to repetead plasmapheresis over three years. Arthritis Rheum 40:1534–1539PubMedCrossRef Neuwelt CM, Daikh DI, Linfoot JA et al (1997) Catastrophic antiphospholipid syndrome. Responce to repetead plasmapheresis over three years. Arthritis Rheum 40:1534–1539PubMedCrossRef
23.
go back to reference Undas A, Swadzba J, Undas R, Musial J (1998) Three episodes of acute multiorgan failure in a woman with secondary antiphospholipid syndrome. Pol Arch Med Wewn 100:556–560PubMed Undas A, Swadzba J, Undas R, Musial J (1998) Three episodes of acute multiorgan failure in a woman with secondary antiphospholipid syndrome. Pol Arch Med Wewn 100:556–560PubMed
24.
go back to reference Cerveny K, Sawitzke AD (1999) Relapsing catastrophic antiphsopholipid antibody syndrome: a mimic for thrombotic thrombocytopenic purpura. Lupus 8:477–481PubMedCrossRef Cerveny K, Sawitzke AD (1999) Relapsing catastrophic antiphsopholipid antibody syndrome: a mimic for thrombotic thrombocytopenic purpura. Lupus 8:477–481PubMedCrossRef
25.
go back to reference Gordon A, McLean CA, Ryan P, Roberts SK (2004) Steroids responsive catastrophic antiphospholipid syndrome. J Gastroenterol Hepatol 19:479–480PubMedCrossRef Gordon A, McLean CA, Ryan P, Roberts SK (2004) Steroids responsive catastrophic antiphospholipid syndrome. J Gastroenterol Hepatol 19:479–480PubMedCrossRef
26.
go back to reference Willis MS, Bandarenko N (2005) Relapse of thrombotic thrombocytopenic purpura: is it a continuum of diseases. Semin Throm Haemost 31:700–708CrossRef Willis MS, Bandarenko N (2005) Relapse of thrombotic thrombocytopenic purpura: is it a continuum of diseases. Semin Throm Haemost 31:700–708CrossRef
27.
go back to reference Meroni PL (2008) Pathogenesis of the antiphospholipid syndrome: an additional example of the mosaic of autoimmunity. J Autoimmun 30:99–103PubMedCrossRef Meroni PL (2008) Pathogenesis of the antiphospholipid syndrome: an additional example of the mosaic of autoimmunity. J Autoimmun 30:99–103PubMedCrossRef
28.
go back to reference Andreoli L, Pregnolato F, Burlingame RW, Allegri F, Rizzini S, Fanelli V, Radice A, Corace C, Sinico RA, Meroni PL, Tincani A (2008) Antinucleosome antibodies in primary antiphospholipid syndrome: a hint at systemic autoimmunity. J Autoimmun 30:51–57PubMedCrossRef Andreoli L, Pregnolato F, Burlingame RW, Allegri F, Rizzini S, Fanelli V, Radice A, Corace C, Sinico RA, Meroni PL, Tincani A (2008) Antinucleosome antibodies in primary antiphospholipid syndrome: a hint at systemic autoimmunity. J Autoimmun 30:51–57PubMedCrossRef
29.
go back to reference Shoenfeld Y, Twig G, Katz U, Sherer Y (2008) Autoantibody explosion in antiphospholipid syndrome. J Autoimmun 30:74–83PubMedCrossRef Shoenfeld Y, Twig G, Katz U, Sherer Y (2008) Autoantibody explosion in antiphospholipid syndrome. J Autoimmun 30:74–83PubMedCrossRef
30.
go back to reference Kajiwara T, Yasuda T, Matsuura E (2007) Intracellular trafficking of beta2-glycoprotein I complexes with lipid vesicles in macrophages: implications on the development of antiphospholipid syndrome. J Autoimmun 29:164–173PubMedCrossRef Kajiwara T, Yasuda T, Matsuura E (2007) Intracellular trafficking of beta2-glycoprotein I complexes with lipid vesicles in macrophages: implications on the development of antiphospholipid syndrome. J Autoimmun 29:164–173PubMedCrossRef
31.
go back to reference Cavazzana I, Manuela N, Irene C, Barbara A, Sara S, Orietta BM, Angela T, Francesco T, Luigi MP (2007) Complement activation in anti-phospholipid syndrome: a clue for an inflammatory process. J Autoimmun 28:160–164PubMedCrossRef Cavazzana I, Manuela N, Irene C, Barbara A, Sara S, Orietta BM, Angela T, Francesco T, Luigi MP (2007) Complement activation in anti-phospholipid syndrome: a clue for an inflammatory process. J Autoimmun 28:160–164PubMedCrossRef
32.
go back to reference Koike T, Bohgaki M, Amengual O, Atsumi T (2007) Antiphospholipid antibodies: lessons from the bench. J Autoimmun 28:129–133PubMedCrossRef Koike T, Bohgaki M, Amengual O, Atsumi T (2007) Antiphospholipid antibodies: lessons from the bench. J Autoimmun 28:129–133PubMedCrossRef
33.
go back to reference Meroni PL, Shoenfeld Y (2008) Predictive, protective, orphan autoantibodies: the example of anti-phospholipid antibodies. Autoimmun Rev 7:585–587PubMedCrossRef Meroni PL, Shoenfeld Y (2008) Predictive, protective, orphan autoantibodies: the example of anti-phospholipid antibodies. Autoimmun Rev 7:585–587PubMedCrossRef
34.
go back to reference Cheng HM, Chamley L (2008) Cryptic natural autoantibodies and co-potentiators. Autoimmun Rev 7:431–434PubMedCrossRef Cheng HM, Chamley L (2008) Cryptic natural autoantibodies and co-potentiators. Autoimmun Rev 7:431–434PubMedCrossRef
35.
go back to reference Frank M, Mancek-Keber M, Krzan M, Sodin-Semrl S, Jerala R, Iglic A, Rozman B, Kralj-Iglic V (2008) Prevention of microvesiculation by adhesion of buds to the mother cell membrane—a possible anticoagulant effect of healthy donor plasmma. Autoimmun Rev 7:240–245PubMedCrossRef Frank M, Mancek-Keber M, Krzan M, Sodin-Semrl S, Jerala R, Iglic A, Rozman B, Kralj-Iglic V (2008) Prevention of microvesiculation by adhesion of buds to the mother cell membrane—a possible anticoagulant effect of healthy donor plasmma. Autoimmun Rev 7:240–245PubMedCrossRef
36.
37.
go back to reference Sodin-Semrl S, Rozman B (2007) Beta2-glcyoprotein I and its clinical significance: from gene sequence to protein levels. Autoimmun Rev 6:547–552PubMedCrossRef Sodin-Semrl S, Rozman B (2007) Beta2-glcyoprotein I and its clinical significance: from gene sequence to protein levels. Autoimmun Rev 6:547–552PubMedCrossRef
38.
go back to reference Bagnasco M, Grassia L, Pesce G (2007) The management of the patient with unexpected autoantibody positivity. Autoimmun Rev 6:347–353PubMedCrossRef Bagnasco M, Grassia L, Pesce G (2007) The management of the patient with unexpected autoantibody positivity. Autoimmun Rev 6:347–353PubMedCrossRef
39.
go back to reference Muscal E, Myones BL (2007) The role of autoantibodies in pediatric neuropsychiatric systemic lupus erythematosus. Autoimmun Rev 6:215–217PubMedCrossRef Muscal E, Myones BL (2007) The role of autoantibodies in pediatric neuropsychiatric systemic lupus erythematosus. Autoimmun Rev 6:215–217PubMedCrossRef
Metadata
Title
Catastrophic Antiphospholipid Syndrome: Treatment, Prognosis, and the Risk of Relapse
Authors
Silvia Bucciarelli
Doruk Erkan
Gerard Espinosa
Ricard Cervera
Publication date
01-06-2009
Publisher
Humana Press Inc
Published in
Clinical Reviews in Allergy & Immunology / Issue 2-3/2009
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI
https://doi.org/10.1007/s12016-008-8107-9

Other articles of this Issue 2-3/2009

Clinical Reviews in Allergy & Immunology 2-3/2009 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.