Semin Thromb Hemost 2012; 38(04): 333-338
DOI: 10.1055/s-0032-1304718
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Update on the Catastrophic Antiphospholipid Syndrome and the “CAPS Registry”

Ricard Cervera
1   Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain
,
Gerard Espinosa
1   Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain
› Author Affiliations
Further Information

Publication History

Publication Date:
05 March 2012 (online)

Abstract

Although less than 1% of patients with the antiphospholipid syndrome (APS) develop the catastrophic variant known as catastrophic antiphospholipid syndrome (CAPS), its potentially lethal outcome emphasizes its importance in clinical medicine today. However, the rarity of this variant makes it extraordinarily difficult to study in any systematic way. To collate all the published case reports as well as the newly diagnosed cases from all over the world, an international registry of patients with CAPS (“CAPS Registry”) was created in 2000 by the European Forum on Antiphospholipid Antibodies (www.med.ub.es/MIMMUN/FORUM/CAPS.HTM). Currently, this database documents the entire clinical, laboratory, and therapeutic data of more than 350 fully registered patients.

 
  • References

  • 1 Asherson RA. The catastrophic antiphospholipid syndrome. J Rheumatol 1992; 19 (4) 508-512
  • 2 Piette JC, Cervera R, Levy RA, Nasonov EL, Triplett DA, Shoenfeld Y. The catastrophic antiphospholipid syndrome—Asherson's syndrome. Ann Med Interne (Paris) 2003; 154 (4) 195-196
  • 3 Asherson RA, Cervera R, Piette JC , et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore) 1998; 77 (3) 195-207
  • 4 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 (6) 355-377
  • 5 Bucciarelli S, Espinosa G, Cervera R , et al; European Forum on Antiphospholipid Antibodies. Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum 2006; 54 (8) 2568-2576
  • 6 Cervera R, Piette JC, Font J , et al; Euro-Phospholipid Project Group. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002; 46 (4) 1019-1027
  • 7 Asherson RA, Cervera R, de Groot PG , et al; Catastrophic Antiphospholipid Syndrome Registry Project Group. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003; 12 (7) 530-534
  • 8 Cervera R, Font J, Gómez-Puerta JA , et al; Catastrophic Antiphospholipid Syndrome Registry Project Group. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis 2005; 64 (8) 1205-1209
  • 9 Cervera R, Bucciarelli S, Plasín MA , et al; Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group (European Forum On Antiphospholipid Antibodies). Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009; 32 (3–4) 240-245
  • 10 Espinosa G, Cervera R, Asherson RA. Catastrophic antiphospholipid syndrome and sepsis. A common link?. J Rheumatol 2007; 34 (5) 923-926
  • 11 Cervera R, Font J, Tincani A, Boffa MC. V meeting of the European forum on antiphospholipid antibodies. Autoimmun Rev 2006; 5: 499-507
  • 12 Cervera R, Tektonidou MG, Espinosa G , et al. Task Force on catastrophic antiphospholipid syndrome (APS) and non-criteria APS manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions. Lupus 2011; 20 (2) 165-173
  • 13 Erkan D, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: updated diagnostic algorithms. Autoimmun Rev 2010; 10 (2) 74-79
  • 14 Asherson RA, Espinosa G, Menahem S , et al. Relapsing catastrophic antiphospholipid syndrome: report of three cases. Semin Arthritis Rheum 2008; 37 (6) 366-372
  • 15 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 (6) 740-746
  • 16 Cervera R, Espinosa G, Cordero A , et al; Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group. 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 (5) 287-296
  • 17 Bucciarelli S, Espinosa G, Asherson RA , et al; Catastrophic Antiphospholipid Syndrome Registry Project Group. The acute respiratory distress syndrome in catastrophic antiphospholipid syndrome: analysis of a series of 47 patients. Ann Rheum Dis 2006; 65 (1) 81-86
  • 18 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 (6) 943-946
  • 19 Espinosa G, Bucciarelli S, Cervera R , et al. Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies. Ann Rheum Dis 2004; 63 (6) 730-736
  • 20 Uthman I, Shamseddine A, Taher A. The role of therapeutic plasma exchange in the catastrophic antiphospholipid syndrome. Transfus Apheresis Sci 2005; 33 (1) 11-17
  • 21 Orbach H, Katz U, Sherer Y, Shoenfeld Y. Intravenous immunoglobulin: adverse effects and safe administration. Clin Rev Allergy Immunol 2005; 29 (3) 173-184
  • 22 Bayraktar UD, Erkan D, Bucciarelli S, Espinosa G, Asherson RA ; Catastrophic Antiphospholipid Syndrome Project Group. The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. J Rheumatol 2007; 34 (2) 346-352
  • 23 Tenedios R, Erkan D, Lockshin MD. Rituximab in the primary antiphospholipid syndrome. Arthritis Rheum 2005; 52: 4078 [abstract]
  • 24 Erdozain JG, Ruiz-Irastorza G, Egurbide MV, Aguirre C. Sustained response to rituximab of autoimmune hemolytic anemia associated with antiphospholipid syndrome. Haematologica 2004; 89 (9) ECR34 [abstract]
  • 25 Rubenstein E, Arkfeld DG, Metyas S, Shinada S, Ehresmann S, Liebman HA. Rituximab treatment for resistant antiphospholipid syndrome. J Rheumatol 2006; 33 (2) 355-357
  • 26 Manner H, Jung B, Tonassi L , et al. Successful treatment of catastrophic antiphospholipid syndrome (CAPS) associated with splenic marginal-zone lymphoma with low-molecular weight heparin, rituximab and bendamustine. Am J Med Sci 2008; 335 (5) 394-397
  • 27 Nageswara Rao, Arteaga GM, Reed AM, Gloor JM, Rodriguez V.. Rituximab for successful management of probable pediatric catastrophic antiphospholipid syndrome. Pediatr Blood Cancer. 2009; 52 (4) 536-538
  • 28 Vora SK, Asherson RA, Erkan D. Catastrophic antiphospholipid syndrome. J Intensive Care Med 2006; 21 (3) 144-159
  • 29 Burcoglu-O'Ral A, Erkan D, Asherson RA. Treatment of catastrophic antiphospholipid syndrome with defibrotide, a proposed vascular endothelial cell modulator. J Rheumatol 2002; 29 (9) 2006-2011
  • 30 Bucciarelli S, Espinosa G, Bové A, Cervera R, Asherson RA. Relapsing catastrophic antiphospholipid syndrome. Ann Rheum Dis 2007; 66 (Suppl II) 304 [abstract]
  • 31 Miyakis S, Lockshin MD, Atsumi T , et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4 (2) 295-306