Skip to main content
Top
Published in: Current Rheumatology Reports 1/2010

01-02-2010

Update on the Diagnosis, Treatment, and Prognosis of the Catastrophic Antiphospholipid Syndrome

Author: Ricard Cervera

Published in: Current Rheumatology Reports | Issue 1/2010

Login to get access

Abstract

The catastrophic antiphospholipid syndrome (APS) is a potentially life-threatening condition, the diagnosis of which requires a high degree of clinical awareness on the part of attending physicians. Patients with APS present with 1) clinical evidence of multiple organ involvement developed over a very short time; 2) histopathologic evidence of multiple small-vessel occlusions; and 3) laboratory confirmation of the presence of antiphospholipid antibodies, usually in high titer. A combination of anticoagulants, corticosteroids, intravenous immunoglobulins, and plasma exchanges is the basic treatment for all patients with this severe condition. Unfortunately, despite current therapies, the mortality rate is still high (around 30%). However, once patients with catastrophic APS have recovered, they usually follow a stable course with continued anticoagulation and few patients present with a relapse of the catastrophic episode.
Literature
1.
go back to reference Asherson RA: The catastrophic antiphospholipid antibody syndrome. J Rheumatol 1992, 19:508–512.PubMed Asherson RA: The catastrophic antiphospholipid antibody syndrome. J Rheumatol 1992, 19:508–512.PubMed
2.
go back to reference Piette JC, Cervera R, Levy R, et al.: The catastrophic antiphospholipid syndrome—Asherson’s syndrome. Ann Med Intern 2003, 154:95–96. Piette JC, Cervera R, Levy R, et al.: The catastrophic antiphospholipid syndrome—Asherson’s syndrome. Ann Med Intern 2003, 154:95–96.
3.
go back to reference Asherson RA, Cervera R, Piette JC, et al.: Catastrophic antibody syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore) 1998, 77:195–207.CrossRef Asherson RA, Cervera R, Piette JC, et al.: Catastrophic antibody syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore) 1998, 77:195–207.CrossRef
4.
go back to reference Asherson RA, Cervera R, Piette JC, et al.: Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore) 2001, 80:355–376.CrossRef Asherson RA, Cervera R, Piette JC, et al.: Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore) 2001, 80:355–376.CrossRef
5.
go back to reference Bucciarelli S, Espinosa G, Cervera R, et al.: Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum 2006, 54:2568–2576.CrossRefPubMed Bucciarelli S, Espinosa G, Cervera R, et al.: Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum 2006, 54:2568–2576.CrossRefPubMed
6.
go back to reference Cervera R, Piette JC, Font J, et al.: Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002, 46:1019–1027.CrossRefPubMed Cervera R, Piette JC, Font J, et al.: Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002, 46:1019–1027.CrossRefPubMed
7.
go back to reference Asherson RA, Cervera R, de Groot P, et al.: Catastrophic antiphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines. Lupus 2003, 12:530–534.CrossRefPubMed Asherson RA, Cervera R, de Groot P, et al.: Catastrophic antiphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines. Lupus 2003, 12:530–534.CrossRefPubMed
8.
go back to reference Cervera R, Font J, Gómez–Puerta JA, et al.: Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis 2005, 64:1205–1209.CrossRefPubMed Cervera R, Font J, Gómez–Puerta JA, et al.: Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis 2005, 64:1205–1209.CrossRefPubMed
9.
go back to reference •• Cervera R, Bucciarelli S, Plasín MA, et al.: Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009, 32:240–245. This article discusses the largest series (n = 280) of patients with catastrophic APS.CrossRefPubMed •• Cervera R, Bucciarelli S, Plasín MA, et al.: Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009, 32:240–245. This article discusses the largest series (n = 280) of patients with catastrophic APS.CrossRefPubMed
10.
go back to reference Espinosa G, Cervera R, Asherson RA: Catastrophic antiphospholipid syndrome and sepsis: a common link? J Rheumatol 2007, 34:923–926.PubMed Espinosa G, Cervera R, Asherson RA: Catastrophic antiphospholipid syndrome and sepsis: a common link? J Rheumatol 2007, 34:923–926.PubMed
11.
go back to reference Cervera R, Font J, Tincani A, Boffa MC: V Meeting of the European Forum on Antiphospholipid Antibodies. Autoimm Rev 2006, 5:499–507.CrossRef Cervera R, Font J, Tincani A, Boffa MC: V Meeting of the European Forum on Antiphospholipid Antibodies. Autoimm Rev 2006, 5:499–507.CrossRef
12.
go back to reference Asherson RA, Espinosa G, Menahem S, et al.: Relapsing catastrophic antiphospholipid syndrome: report of three cases. Semin Arthritis Rheum 2008, 37:366–372.CrossRefPubMed Asherson RA, Espinosa G, Menahem S, et al.: Relapsing catastrophic antiphospholipid syndrome: report of three cases. Semin Arthritis Rheum 2008, 37:366–372.CrossRefPubMed
13.
go back to reference Gómez–Puerta JA, Cervera R, Espinosa G, et al.: Catastrophic antiphospholipid syndrome during pregnancy and puerperium: maternal and fetal characteristics of 15 cases. Ann Rheum Dis 2007, 66:740–746.CrossRefPubMed Gómez–Puerta JA, Cervera R, Espinosa G, et al.: Catastrophic antiphospholipid syndrome during pregnancy and puerperium: maternal and fetal characteristics of 15 cases. Ann Rheum Dis 2007, 66:740–746.CrossRefPubMed
14.
go back to reference Miesbach W, Asherson RA, Cervera R, et al.: The role of malignancies in patients with catastrophic antiphospholipid (Asherson’s) syndrome. Clin Rheumatol 2007, 26:2109–2114.CrossRefPubMed Miesbach W, Asherson RA, Cervera R, et al.: The role of malignancies in patients with catastrophic antiphospholipid (Asherson’s) syndrome. Clin Rheumatol 2007, 26:2109–2114.CrossRefPubMed
15.
go back to reference Cervera R, Espinosa G, Cordero A, et al.: Intestinal involvement secondary to the antiphospholipid syndrome (APS): clinical and immunologic characteristics of 97 patients: comparison of classic and catastrophic APS. Semin Arthritis Rheum 2007, 36:287–296.CrossRefPubMed Cervera R, Espinosa G, Cordero A, et al.: Intestinal involvement secondary to the antiphospholipid syndrome (APS): clinical and immunologic characteristics of 97 patients: comparison of classic and catastrophic APS. Semin Arthritis Rheum 2007, 36:287–296.CrossRefPubMed
16.
go back to reference Belmont HM, Abramson SB, Lie JT: Pathology and pathogenesis of vascular injury in systemic lupus erythematosus. Arthritis Rheum 1996, 39:9–22.CrossRefPubMed Belmont HM, Abramson SB, Lie JT: Pathology and pathogenesis of vascular injury in systemic lupus erythematosus. Arthritis Rheum 1996, 39:9–22.CrossRefPubMed
17.
go back to reference Bucciarelli S, Espinosa G, Asherson RA, et al.: The acute respiratory distress syndrome in catastrophic antiphospholipid syndrome: analysis of a series of 47 patients. Ann Rheum Dis 2006, 65:81–86.CrossRefPubMed Bucciarelli S, Espinosa G, Asherson RA, et al.: The acute respiratory distress syndrome in catastrophic antiphospholipid syndrome: analysis of a series of 47 patients. Ann Rheum Dis 2006, 65:81–86.CrossRefPubMed
18.
go back to reference Asherson RA, Espinosa G, Cervera R, et al.: Disseminated intravascular coagulation in catastrophic antiphospholipid syndrome: clinical and haematological characteristics of 23 patients. Ann Rheum Dis 2005, 64:943–946.CrossRefPubMed Asherson RA, Espinosa G, Cervera R, et al.: Disseminated intravascular coagulation in catastrophic antiphospholipid syndrome: clinical and haematological characteristics of 23 patients. Ann Rheum Dis 2005, 64:943–946.CrossRefPubMed
19.
go back to reference Espinosa G, Bucciarelli S, Cervera R, et al.: Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies. Ann Rheum Dis 2004, 63:730–736.CrossRefPubMed Espinosa G, Bucciarelli S, Cervera R, et al.: Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies. Ann Rheum Dis 2004, 63:730–736.CrossRefPubMed
20.
go back to reference Asherson RA, Cervera R, Font J: Multiorgan thrombotic disorders in systemic lupus erythematosus: a common link? Lupus 1992, 1:199–203.CrossRefPubMed Asherson RA, Cervera R, Font J: Multiorgan thrombotic disorders in systemic lupus erythematosus: a common link? Lupus 1992, 1:199–203.CrossRefPubMed
21.
go back to reference Asherson RA, Pierangeli SS, Cervera R: Is there a microangiopathic antiphospholipid syndrome? Ann Rheum Dis 2007, 66:429–432.CrossRefPubMed Asherson RA, Pierangeli SS, Cervera R: Is there a microangiopathic antiphospholipid syndrome? Ann Rheum Dis 2007, 66:429–432.CrossRefPubMed
22.
go back to reference Asherson RA, Pierangeli SS, Cervera R: Microangiopathic antiphospholipid-associated syndromes revisited: new concepts relating to antiphospholipid antibodies and syndromes. J Rheumatol 2007, 34:1793–1795.PubMed Asherson RA, Pierangeli SS, Cervera R: Microangiopathic antiphospholipid-associated syndromes revisited: new concepts relating to antiphospholipid antibodies and syndromes. J Rheumatol 2007, 34:1793–1795.PubMed
23.
go back to reference Asherson R, Cervera R: Microvascular and microangiopathic antiphospholipid-associated syndromes (MAPS): semantic or antisemantic? Autoimm Rev 2008, 7:164–167.CrossRef Asherson R, Cervera R: Microvascular and microangiopathic antiphospholipid-associated syndromes (MAPS): semantic or antisemantic? Autoimm Rev 2008, 7:164–167.CrossRef
24.
go back to reference Girardi G, Redecha P, Salmon JE: Heparin prevents antiphospholipid antibody-induced fetal loss by inhibiting complement activation. Nat Med 2004, 10:1222–1226.CrossRefPubMed Girardi G, Redecha P, Salmon JE: Heparin prevents antiphospholipid antibody-induced fetal loss by inhibiting complement activation. Nat Med 2004, 10:1222–1226.CrossRefPubMed
25.
go back to reference Christman JW, Lancaster LH, Blackwell TS: Nuclear factor kappa B: a pivotal role in the systemic inflammatory response syndrome and new target for therapy. Intensive Care Med 1998, 24:1131–1138.CrossRefPubMed Christman JW, Lancaster LH, Blackwell TS: Nuclear factor kappa B: a pivotal role in the systemic inflammatory response syndrome and new target for therapy. Intensive Care Med 1998, 24:1131–1138.CrossRefPubMed
26.
go back to reference Uthman I, Shamseddine A, Taher A: The role of therapeutic plasma exchange in catastrophic antiphospholipid syndrome. Transfus Apher Sci 2005, 33:11–17.CrossRefPubMed Uthman I, Shamseddine A, Taher A: The role of therapeutic plasma exchange in catastrophic antiphospholipid syndrome. Transfus Apher Sci 2005, 33:11–17.CrossRefPubMed
27.
go back to reference Orbach H, Katz U, Sherer Y, Shoenfeld Y: Intravenous immunoglobulin: adverse effects and safe administration. Clin Rev Allergy Immunol 2005, 29:173–184.CrossRefPubMed Orbach H, Katz U, Sherer Y, Shoenfeld Y: Intravenous immunoglobulin: adverse effects and safe administration. Clin Rev Allergy Immunol 2005, 29:173–184.CrossRefPubMed
28.
go back to reference Bayraktar UD, Erkan D, Bucciarelli S, et al.: The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. J Rheumatol 2007, 34:346–352.PubMed Bayraktar UD, Erkan D, Bucciarelli S, et al.: The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. J Rheumatol 2007, 34:346–352.PubMed
29.
go back to reference Tenedious R, Erkan D, Lockshin MD: Rituximab in the primary antiphospholipid syndrome [abstract]. Arthritis Rheum 2005, 52:4078. Tenedious R, Erkan D, Lockshin MD: Rituximab in the primary antiphospholipid syndrome [abstract]. Arthritis Rheum 2005, 52:4078.
30.
go back to reference Erdozain JG, Ruiz–Irastorza G, Egurbide MV, et al.: Sustained response to rituximab of autoimmune hemolytic anemia associated with antiphospholipid syndrome [abstract]. Haematologica 2004, 89:ECR34.PubMed Erdozain JG, Ruiz–Irastorza G, Egurbide MV, et al.: Sustained response to rituximab of autoimmune hemolytic anemia associated with antiphospholipid syndrome [abstract]. Haematologica 2004, 89:ECR34.PubMed
31.
go back to reference Rubenstein E, Arkfeld DG, Metyas S, et al.: Rituximab treatment for resistant antiphospholipid syndrome. J Rheumatol 2006, 33:355–357.PubMed Rubenstein E, Arkfeld DG, Metyas S, et al.: Rituximab treatment for resistant antiphospholipid syndrome. J Rheumatol 2006, 33:355–357.PubMed
32.
go back to reference Dosekun AK, Pollak VE, Glas–Greenwalt P, et al.: Ancrod in systemic lupus erythematosus with thrombosis: clinical and fibrinolysis effects. Arch Intern Med 1984, 144:37–42.CrossRefPubMed Dosekun AK, Pollak VE, Glas–Greenwalt P, et al.: Ancrod in systemic lupus erythematosus with thrombosis: clinical and fibrinolysis effects. Arch Intern Med 1984, 144:37–42.CrossRefPubMed
33.
go back to reference Yang LH, Hoppensteadt DA, Iqbal O, Fareed J: Defibrotide mediated inhibition of serotonin, endothelin-I, thromboxane, and serum induced contraction of canine femoral and pulmonary arterial rings. Thromb Res 1996, 84:167–177.CrossRefPubMed Yang LH, Hoppensteadt DA, Iqbal O, Fareed J: Defibrotide mediated inhibition of serotonin, endothelin-I, thromboxane, and serum induced contraction of canine femoral and pulmonary arterial rings. Thromb Res 1996, 84:167–177.CrossRefPubMed
34.
go back to reference Vero S, Asherson RA, Erkan D: Critical care review: catastrophic antiphospholipid syndrome. J Intensive Care Med 2006, 21:144–159.CrossRef Vero S, Asherson RA, Erkan D: Critical care review: catastrophic antiphospholipid syndrome. J Intensive Care Med 2006, 21:144–159.CrossRef
35.
go back to reference Bucciarelli S, Erkan D, Espinosa G, Cervera R: Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse. Clinic Rev Allerg Immunol 2009, 38:80–84.CrossRef Bucciarelli S, Erkan D, Espinosa G, Cervera R: Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse. Clinic Rev Allerg Immunol 2009, 38:80–84.CrossRef
36.
go back to reference Erkan D, Asherson RA, Espinosa G, et al.: The long-term outcome of catastrophic antiphospholipid syndrome survivors. Ann Rheum Dis 2003, 62:530–533.CrossRefPubMed Erkan D, Asherson RA, Espinosa G, et al.: The long-term outcome of catastrophic antiphospholipid syndrome survivors. Ann Rheum Dis 2003, 62:530–533.CrossRefPubMed
37.
go back to reference Bucciarelli S, Espinosa G, Bové A, et al.: Relapsing catastrophic antiphospholipid syndrome [abstract]. Ann Rheum Dis 2007, 66:304. Bucciarelli S, Espinosa G, Bové A, et al.: Relapsing catastrophic antiphospholipid syndrome [abstract]. Ann Rheum Dis 2007, 66:304.
Metadata
Title
Update on the Diagnosis, Treatment, and Prognosis of the Catastrophic Antiphospholipid Syndrome
Author
Ricard Cervera
Publication date
01-02-2010
Publisher
Current Science Inc.
Published in
Current Rheumatology Reports / Issue 1/2010
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-009-0073-6

Other articles of this Issue 1/2010

Current Rheumatology Reports 1/2010 Go to the issue