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      Debut de lupus eritematoso sistémico con síndrome antifosfolipídico catastrófico: beneficio del tratamiento precoz Translated title: Debut of systemic lupus eritematous with catastrophic antiphospholipid syndrome: benefit of the early treatment

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          Abstract

          La denominación de Síndrome Antifosfolipídico (SAF) "catastrófico" fue empleada para definir una forma grave y rápidamente evolutiva de SAF que conduce a fallo multiorgánico. Todos desarrollan accidentes oclusivos en un corto periodo (días a semanas), siendo el índice de mortalidad del 50%. En su tratamiento se han utilizado glucocorticoides, heparina, recambio plasmático, ciclofosfamida y gammaglobulinas intravenosas. El presente caso es de una paciente de sexo femenino, con diagnóstico de lupus eritematoso sistémico, con anticuerpos anticardiolipina, anti ribonucleoproteína (anti RNP) y afectación multiorgánica que sobrevivió gracias al tratamiento precoz y multidisciplinario.

          Translated abstract

          The Catastrophic Antiphospholipid Syndrome (CAS) results in multiorgan system failure. All patients develop occlusive accident in a short period (days to weeks), with a mortality of 50%. Treatment can include glucocorticoids, heparin, plasmatic exchange, ciclofosfamide and intravenous globulin. The present article treat of female patient lairs Systemic Eritematous Lupus (SLE), anticardiolipine antibodies, anti ribonucleoprotein antibodies (antiRNP) and multiorgan systemic failure, she survived thanks to the multidisciplinary a early treatment.

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          The antiphospholipid syndrome.

          Jerrold S Levine, D Ware Branch, Joyce Rauch (2002)
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            Management of antiphospholipid antibody syndrome: a systematic review.

            Caroline Crowther, Wendy Lim, J W Eikelboom (2006)
            Antiphospholipid antibodies are autoantibodies directed against proteins that bind to phospholipid. Antiphospholipid antibody syndrome (APS) refers to the association between antiphospholipid antibodies and thrombosis risk or pregnancy morbidity. Patients with APS may be at increased risk of recurrent arterial or venous thrombosis or pregnancy loss. To systematically review the evidence for treatment of thrombosis risk in patients with antiphospholipid antibodies or APS. Search of MEDLINE (1966 to November 2005) and Cochrane Library electronic databases (2005) and reference lists for randomized trials, meta-analyses of randomized trials, and prospective cohort studies of the treatment of thrombosis risk in patients with antiphospholipid antibodies or APS. Studies were selected on the basis of clinical relevance. Among patients with antiphospholipid antibodies, the absolute risk of developing new thrombosis is low ( 10% in the first year) in patients with a history of venous thrombosis who have discontinued anticoagulant drugs within 6 months. Compared with placebo or untreated control, anticoagulation with moderate-intensity warfarin (adjusted to a target international normalized ratio [INR] of 2.0-3.0) reduces the risk of recurrent venous thrombosis by 80% to 90% irrespective of the presence of antiphospholipid antibodies and may be effective for preventing recurrent arterial thrombosis. No evidence exists that high-intensity warfarin (target INR, >3.0) is more effective than moderate-intensity warfarin. For patients with a single positive antiphospholipid antibody test result and prior stroke, aspirin and moderate-intensity warfarin appear equally effective for preventing recurrent stroke. Treatment issues that have not been addressed in clinical trials, or for which the evidence is conflicting, include the role of antithrombotic prophylaxis in patients with antiphospholipid antibodies without prior thrombosis, the optimal treatment of noncerebrovascular arterial thrombosis, recurrent thrombosis despite warfarin therapy, and treatment of women with antiphospholipid antibodies and recurrent fetal loss. In patients with APS, moderate-intensity warfarin is effective for preventing recurrent venous thrombosis and perhaps also arterial thrombosis. Aspirin appears to be as effective as moderate-intensity warfarin for preventing recurrent stroke in patients with prior stroke and a single positive test result for antiphospholipid antibody. The optimal treatment of other thrombotic aspects of APS needs to be addressed in well-designed prospective studies.
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              Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome.

              S Bucciarelli, J Font, J Piette … (2005)
              To describe the characteristics of patients with catastrophic antiphospholipid syndrome (APS) included in the International Registry of patients with this condition (CAPS registry) and to analyse the value of the recently proposed preliminary criteria for the classification of catastrophic APS. A review of the first 220 patients included in the website based CAPS registry was undertaken and the preliminary criteria for their classification were tested; 175 unselected patients with systemic lupus erythematosus or APS, or both, acted as controls. The mean age of the patients was 38 (14) years (range 7 to 74), with a female preponderance (F/M, 153/67). The main clinical manifestations included renal involvement in 154 (70%), pulmonary in 146 (66%), cerebral in 133 (60%), cardiac in 115 (52%), and cutaneous in 104 (47%); 114 patients (52%) recovered after the catastrophic APS event (mortality 48%). Patients who received the combination of anticoagulation plus steroids plus plasma exchange or intravenous immunoglobulins had the best survival rate (63%, p = 0.09). Sufficient data could be analysed for application of the classification criteria in 176 patients. According to the preliminary criteria, 89 patients (51%) could be classified as having "definite" and 70 (40%) as having "probable" catastrophic APS, thus given a sensitivity of 90.3% with a specificity of 99.4%. Positive and negative predictive values were 99.4% and 91.1%, respectively. The preliminary criteria for the classification of catastrophic APS and the CAPS registry are useful tools for epidemiological studies.
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                Author and article information

                Contributors
                R. Ruiz Domínguez: Role: ND
                I. Huanca Mamani: Role: ND
                Journal
                Journal ID (publisher-id): ami
                Title: Anales de Medicina Interna
                Abbreviated Title: An. Med. Interna (Madrid)
                Publisher: Arán Ediciones, S. L. (, , Spain )
                ISSN (Print): 0212-7199
                Publication date (Print and electronic): July 2007
                Volume: 24
                Issue: 7
                Pages: 335-338
                Affiliations
                [01] orgnameCaja Nacional de Salud orgdiv1Hospital Materno Infantil orgdiv2Departamento de Medicina Interna
                [02] orgnameCaja Nacional de Salud orgdiv1Hospital Materno Infantil orgdiv2Servicio de Nefrología Perú
                Article
                Publisher ID: S0212-71992007000700008
                DOI: 10.4321/s0212-71992007000700008
                SO-VID: 83834d3c-a47a-4bd4-afeb-fd2f57145a9b
                License:

                This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 International License.

                History
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                Figures: 0, Tables: 0, Equations: 0, References: 25, Pages: 4
                Product

                SciELO Spain


                Keywords: Lupus eritematoso sistémico,Síndrome antifosfolipídico catastrófico,Anticuerpos anti U1-RNP,Systemic Lupus Eritematous,Catastrophic Antiphospholipid Syndrome,Anti U1-RNP antibodies
                Data availability:
                Keywords: Lupus eritematoso sistémico, Síndrome antifosfolipídico catastrófico, Anticuerpos anti U1-RNP, Systemic Lupus Eritematous, Catastrophic Antiphospholipid Syndrome, Anti U1-RNP antibodies

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