A Case Report. Catastrophic Antiphospholipid Syndrome
Article Sidebar
How to Cite
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Downloads
Societal impact
Main Article Content
Abstract
Twenty-six-year-old, female patient with a clinical history of Systemic Lupus Erythematosus, Lupus Panniculitis, and Antiphospholipid Syndrome. She was admitted to the emergency room after two days of symptoms of asthenia, fever, and profuse bleeding from ulcers in the lower limbs. Physical examination revealed generalized edema and tachycardia. The requested tests showed coagulation abnormalities, alterations in the complement and liver enzymes. Presence of Streptococcus spp bacteremia was also determined. Patient was hospitalized and antibiotic treatment was started. While she was in the hospital, she required close monitoring, active and multidisciplinary management by the medical staff of the institution due to severe and rapid health deterioration. However, despite treatment, the patient developed multiple organ failure leading to death within only seven days of the beginning of the symptomatology.
Article Details
References
Piette J-C, Cervera R, Levy RA, Nasonov EL, Triplett DA, Shoenfeld Y. The catastrophic antiphospholipid syndrome—Asherson’s syndrome. Ann Med Internet. 2003;154:195-6.
Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530-4.
Asherson RA. The catastrophic antiphospholipid syndrome. J Rheumatol. 1992;19:508–12.
Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid Syndrome Clinical and Immunologic manifestations and Patterns of Disease Expression in a Cohort of 1,000 Patients. Arthritis Rheum. 2002 Apr;46(4):1019-27.
Asherson RA, Cervera R, Piette JC, Shoenfeld Y, Espinosa G,Petri MA, et al. Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore). 2001;80:355–77.
Erkan D, Cervera R, Asherson RA. Catastrophic antiphospholipid syndrome: where do we stand? Arthritis Rheum. 2003 Dec;48(12):3320-7.
Asherson RA, Espinosa G, Menahem S, Yinh J, Bucciarelli S, Bosch X, et al. Relapsing catastrophic Antiphospholipid Syndrome: report of three cases. Semin Arthritis Rheum. 2008 Jun;37(6):366- 72.
Asherson R. Multiorgan failure and antiphospholipid antibodies: the catastrophic antiphospholipid (Asherson’s) syndrome. Immunobiology. 2005;210(10):727-33.
Ortega-Hernandez OD, Agmon-Levin N, Blank M, Asherson R, Shoenfeld Y. The physiopathology of the catastrophic antiphospholipid (Asherson’s) syndrome: compelling evidence. J Autoimmun. 2009;32:1–6.
Asherson R. The catastrophic antiphospholipid (Asherson’s) syndrome. Autoimmun Rev. 2006 Dec;6(2):64-7.
Asherson RA, Shoenfeld Y. The role of infection in the pathogenesis of the catastrophic antiphospholipid syndrome-molecular mimicry? J Rheumatol. 2000;27:12–5.
Kitchens CS. Thrombotic storm: when thrombosis begets thrombosis. Am J Med.1998;104:381–5.
Raschi E, Testoni C, Borghi MO, Fineschi S, Meroni PL. Endothelium activation in the antiphospholipid syndrome. Biomed Pharmacother. 2003;7:282–6.
Pierangeli SS, Girardi G, Vega-Ostertag M, Liu X, Espinola RG,Salmon J. Requirement of activation of complement C3 and C5 for antiphospholipid antibody mediated thrombosis. Arthritis Rheum. 2005;52:2120–4.
Hart ML, Ceonzo KA, Shaffer LA, Takahashi K, Rother RP, Reenstra WR, et al. Gastrointestinal ischemia–reperfusion is lectin complement pathway dependent without involving C1q. J Immunol. 2004 Dec 1;173(11):7055-61.
Fleming SD, Egan RE, Chai C, Girardi G, Holers VM, Salmon J, et al. Anti-phospholipid antibodies restore mesenteric ischemia reperfusion-induced injury in complement receptor 2/complement receptor 1-deficient mice. J Immunol. 2005;173:7055–61.
Asherson RA. Acute respiratory distress syndrome and other unusual manifestations of the catastrophic antiphospholipid (Asherson’s) syndrome. Isr Med Assoc J. 2004 Jun;6(6):360-3.
Espinosa G, Cervera R, Asherson RA. Catastrophic antiphospholipid syndrome and sepsis. A common link? J Rheumatol. 2007 May;34(5):923-6.
Asherson RA. Multiorgan failure and antiphospholipid antibodies: the catastrophic antiphospholipid (Asherson’s) syndrome. Immunobiology.2005;210(10):727-33.
Bucciarelli S, Espinosa G, Cervera R, Erkan D, Gómez-Puerta JA, Ramos-Casals M, et al. Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum. 2006 Aug;54(8):2568-76.
Espinosa G, Bucciarelli S, Cervera R, Lozano M, Reverter JC, De la Red G, et al. Thrombotic microangiopathic hemolytic anaemia and antiphospholipid antibodies. Ann Rheum Dis. 2004;63:730– 6
Asherson RA, Espinosa G, Cervera R, Gómez-Puerta JA, Musuruana J, Bucciarelli S, et al. Disseminated intravascular coagulation in catastrophic antiphospholipid syndrome: clinical and haematological characteristics of 23 patients. Ann Rheum Dis. 2005 Jun;64(6):943-6.
Cervera R, Gómez-Puerta JA, Cucho M, Espinosa G, Font J, De la Red G, et al. Catastrophic antiphospholipid syndrome (CAPS): analysis of the international consensus statement on preliminary classification criteria for CAPS using the ‘‘CAPS Registry’’. Ann Rheum Dis. 2003;62(Suppl 1):88
Asherson RA, Cervera R, Piette JC, Font J, Lie JT, Burcoglu A, et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Ann Rheum Dis.1998 May;77(3):195- 207.
Asherson RA, Cervera R, Piette JC, Shoenfeld Y, Espinosa G, Petri MA, et al. Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Ann Rheum Dis.2001 Nov;80(6):355-77.
Amital H, Levy Y, Davidson C, Lundberg I, Harju A, Kosach Y, et al. Catastrophic antiphospholipid syndrome: remission following leg amputation in 2 cases. Semin Arthritis Rheum. 2001 Oct;31(2):127-32.
Spinnato JA, Clark AL, Pierangeli SS, Harris EN. Intravenous immunoglobulin therapy for the antiphospholipid syndrome in pregnancy. Am J Obstet Gynecol. 1995;172:690–4.
Erkan D, Asherson RA, Espinosa G, Cervera R, Font J, Piette JC, et al. Long term outcome of catastrophic antiphospholipid syndrome survivors. Ann Rheum Dis. 2003 Jun;62(6):530-3.