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1.
Arch. argent. pediatr ; 119(6): e631-e635, dic. 2021. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1353055

ABSTRACT

El síndrome antifosfolipídico (SAF) es infrecuente en la edad pediátrica (3 %) y se presenta como eventos trombóticos de lechos vasculares y/o abortos espontáneos, asociado a la presencia de anticuerpos antifosfolipídicos (aFL). Este síndrome puede ser primario o asociado a alguna enfermedad sistémica subyacente. Se presenta el caso de una niña de 12 años con hemiparesia faciobraquiocrural derecha y alteración en la marcha de aparición aguda, en la cual se confirma un accidente cerebrovascular (ACV) isquémico por trombosis de la arteria cerebral media asociado a aFL positivos (anticuerpo anticardiolipina, anticoagulante lúpico y anticuerpo anti-ß2-glicoproteína). Cumple con los criterios para realizar diagnóstico de síndrome antifosfolipídico. Luego de iniciar el tratamiento, la paciente evoluciona de manera favorable. Se trata de una patología infrecuente y de presentación variable, por lo que requiere un alto sentido de alerta por parte del equipo de salud para evitar retrasos en el diagnóstico y el tratamiento, y disminuir su morbimortalidad


Antiphospholipid syndrome (APS) is infrequent at pediatric age (3 %) and is characterized by venous or arterial thrombosis and/or spontaneous abortions. APS occurs either as a primary condition or in the setting of an underlying disease. This is a case of a 12-year-old girl with a right hemiparesis and acute disturbance in gait, in which an ischemic cerebrovascular accident (CVA) due to middle cerebral artery thrombosis associated with positive antiphospholipid antibodies is confirmed (anticardiolipin antibody, lupus anticoagulant and anti-ß2-glycoprotein antibody), fulfilling the criteria for the diagnosis of antiphospholipid syndrome . After starting treatment accordingly, the patient evolves favorably. As this pathology is infrequent and of variable presentation, it requires a high sense of alert from the health team to avoid delays in diagnosis and treatment


Subject(s)
Humans , Female , Child , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Lupus Coagulation Inhibitor , Antibodies, Antiphospholipid , beta 2-Glycoprotein I
2.
Rev. bras. oftalmol ; 80(1): 56-58, jan.-fev. 2021. graf
Article in English | LILACS | ID: biblio-1251313

ABSTRACT

ABSTRACT Antiphospholipid antibody syndrome (APS) is characterized by the development of venous and/or arterial thrombosis and by gestational morbidity (recurrent fetal losses) in the presence of antiphospholipid antibodies. We report the case of a 38-year-old woman who was diagnosed with primary APS from thromboembolic abnormalities in the retinal periphery that led to retinal ischemia. The aim of this study is to share with physicians and medical undergraduates an atypical manifestation of the syndrome that is the most common acquired thrombophilia, that should be part of the diagnostic routine of all clinical specialties.


RESUMO A síndrome do anticorpo antifosfolipídio (SAAF) caracteriza-se pelo desenvolvimento de tromboses venosas e/ou arteriais e pela morbidade gestacional (perdas fetais recorrentes) na presença de anticorpos antifosfolipídicos. Foi relatado o caso de uma paciente de 38 anos que foi diagnosticada com SAAF primária, a partir de alterações tromboembólicas na periferia da retina, que levaram à isquemia retiniana. O objetivo desse estudo é compartilhar com médicos e acadêmicos de medicina uma manifestação atípica da síndrome que é a trombofilia adquirida mais comum, devendo fazer parte da rotina diagnóstica de todas as especialidades clínicas.


Subject(s)
Humans , Female , Adult , Retinal Diseases/etiology , Antiphospholipid Syndrome/complications , Ischemia/etiology , Retinal Diseases/diagnosis , Retinal Vessels/pathology , Thrombosis , Fluorescein Angiography , Lupus Coagulation Inhibitor , Antiphospholipid Syndrome/diagnosis , Tomography, Optical Coherence , Ischemia/diagnosis
4.
Article in English | WPRIM | ID: wpr-880679

ABSTRACT

Cryptococcal meningitis has become the largest cause for the death of infectious diseases in the central nervous system infectious disease worldwide. Most patients with cryptococcal meningitis have AIDS, autoimmune diseases, hematologic malignancies, and some other relevant diseases. It is mainly caused by infection with


Subject(s)
Antiphospholipid Syndrome/complications , Cryptococcus neoformans , Female , HIV Infections , Humans , Meningitis, Cryptococcal/complications , Middle Aged , Stroke
5.
Rev. cuba. reumatol ; 22(3): e752,
Article in Spanish | LILACS, CUMED | ID: biblio-1144538

ABSTRACT

El síndrome antifosfolipídico o de Hughes, como también se le conoce, puede aparecer de manera aislada o asociado a otras enfermedades autoinmunes como el lupus eritematoso sistémico. La asociación de ambas entidades puede causar varias complicaciones, como el tromboembolismo pulmonar. Se presenta el caso de una paciente de 28 años de edad, con antecedentes de abortos a repetición y óbito fetal, ingresada en esta ocasión, debido a una trombosis venosa profunda del miembro superior derecho, confirmada mediante ecografía Doppler. Se comprobó el diagnóstico de síndrome antifosfolipídico secundario a lupus eritematoso sistémico, sustentado por los elementos clínicos e inmunitarios presentes. La paciente evolucionó satisfactoriamente, con el protocolo terapéutico empleado en fase aguda: heparina de bajo peso molecular del tipo clexane (enoxaparina) 1 mg/kg cada 12 h y dicumarínicos del tipo warfarina 5 mg con una razón normalizada internacional (INR) de 3. Se mantiene actualmente con una dosis de 10 mg/día e hidroxicloroquina 200 mg diarios. Conclusiones: Se resalta la importancia de diagnosticar el síndrome antifosfolipídico, ante toda paciente con abortos espontáneos o muertes perinatales inexplicables. El tratamiento debe ser multidisciplinario y se debe realizar una búsqueda sistemática de afecciones secundarias (particularmente enfermedades difusas del tejido conectivo) antes de calificar al síndrome como primario(AU)


The antiphospholipid or Hughes syndrome, as it is also known, can appear in isolation or in association with other autoimmune diseases such as systemic lupus erythematosus. The association of both entities can cause various complications, such as pulmonary thromboembolism. We present the case of a 28-year-old patient, with a history of repeated abortions and stillbirth, admitted on this occasion due to deep vein thrombosis of the right upper limb, confirmed by Doppler ultrasound. The diagnosis of antiphospholipid syndrome secondary to systemic lupus erythematosus was confirmed, supported by the clinical and immune elements present. The patient evolved satisfactorily, with the therapeutic protocol used in the acute phase, where she initially received treatment with low molecular weight heparin of the type clexane (enoxaparin) 1 mg x kg every 12 hours, and discoumarin drugs of the warfarin type, which she currently maintains at a 5mg dose with an INR of 3. Initially prednisone was placed at a dose of 1mg x kg with good therapeutic response, currently maintaining a 10mg dose. He is also currently on hydroxychloroquine 200 mg daily. Conclusions: The importance of diagnosing the antiphospholipid syndrome is highlighted in all patients with spontaneous abortions or unexplained perinatal deaths. Treatment should be multidisciplinary and a systematic search for secondary conditions (particularly diffuse connective tissue diseases) should be conducted before qualifying the syndrome as primary(AU)


Subject(s)
Humans , Female , Adult , Abortion, Spontaneous/etiology , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Hydroxychloroquine , Lupus Erythematosus, Systemic/complications , Pulmonary Embolism/prevention & control , Hydroxychloroquine/therapeutic use
6.
Rev. bras. ginecol. obstet ; 41(10): 621-627, Oct. 2019. tab
Article in English | LILACS | ID: biblio-1042317

ABSTRACT

Abstract Antiphospholipid antibody syndrome (APS) is a systemic, autoimmune, prothrombotic disease characterized by persistent antiphospholipid antibodies (aPLs), thrombosis, recurrent abortion, complications during pregnancy, and occasionally thrombocytopenia. The objective of the present study was to review the pathophysiology of APS and its association with female infertility. A bibliographic review of articles of the past 20 yearswas performed at the PubMed, Scielo, and Bireme databases. Antiphospholipid antibody syndrome may be associated with primary infertility, interfering with endometrial decidualization and with decreased ovarian reserve. Antiphospholipid antibodies also have direct negative effects on placentation, when they bind to the trophoblast, reducing their capacity for invasion, and proinflammatory effects, such as complement activation and neutrophil recruitment, contributing to placental insufficiency, restricted intrauterine growth, and fetal loss. In relation to thrombosis, APS results in a diffuse thrombotic diathesis, with global and diffuse dysregulation of the homeostatic balance. Knowing the pathophysiology of APS, which is closely linked to female infertility, is essential for new therapeutic approaches, specialized in immunomodulation andinflammatory signaling pathways, to provide important advances in its treatment.


Resumo A Síndrome do anticorpo antifosfolípide (SAF) é uma doença sistêmica, autoimune e prótrombótica caracterizada por anticorpos antifosfolípides, trombose, aborto recorrente, complicações durante a gestação, e, ocasionalmente, trombocitopenia. O objetivo do presente estudo foi revisar a fisiopatologia da SAF e sua associação com a infertilidade feminina. Foi feita uma revisão bibliográfica dos últimos 20 anos nas bases de dados PubMed, Scielo e Bireme. A SAF pode estar associada à infertilidade primária, interferindo na decidualização endometrial e combaixas reservas ovarianas. Os anticorpos antifosfolípides também apresentam efeito negativo direto na placentação, se ligando ao trofoblasto e diminuindo sua capacidade de invasão, além de efeitos pró-inflamatórios, tais como ativação do sistema de complemento e recrutamento de neutrófilos, contribuindo para a insuficiência placentária, crescimento intrauterino restrito e perda fetal.Quanto a trombose, a SAF resulta em distúrbios trombóticos difusos, com uma desregulação do balanço homeostático. Conhecer a fisiopatologia da SAF, que apresenta associação importante com a infertilidade feminina, é essencial para novas abordagens terapêuticas, principalmente no que tange imunomodulação e os caminhos de ativação inflamatórios.


Subject(s)
Humans , Female , Pregnancy , Adult , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/physiopathology , Infertility, Female/complications , Infertility, Female/physiopathology , Abortion, Habitual , Antibodies, Antiphospholipid/blood , Middle Aged
7.
Arq. bras. oftalmol ; 82(2): 155-157, Mar.-Apr. 2019. tab
Article in English | LILACS | ID: biblio-989400

ABSTRACT

ABSTRACT - Numerous studies have reported on structural vascular anomalies and ischemia associated with neurofibromatosis type 1 that are thought to stem from dysfunction of neurofibromin, the neurofibromatosis type 1 protein. Documented cases of associated antiphospholipid syndrome fulfilling the accepted diagnostic criteria are exceptionally rare, with only three cases reported in the literature. Here, we report on a patient with neurofibromatosis type 1 and a history of spontaneous abortions presenting with sudden vision loss in the right eye and swelling of the optic nerve head. Fluorescein angiography indicated anterior ischemic optic neuropathy. Brain magnetic resonance imaging revealed findings consistent with left cavernous sinus meningioma. Serologic testing demonstrated persistently elevated anti-b2-glycoprotein antibodies. Her findings suggested antiphospholipid syndrome with concomitant clinical and laboratory evidence of antiphospholipid syndrome: frequent abortions, a vaso-occlusive episode, and persistently elevated antiphospholipid syndrome antibodies. To our knowledge, this case represents the first neuro-ophthalmic manifestation of antiphospholipid syndrome associated with neurofibromatosis type 1.


RESUMO - Inúmeros estudos têm relatado anomalias vasculares estruturais e isquemia associada com à neurofibromatose tipo 1 que, acredita-se, resultam da disfunção da neurofibromina, a proteína tipo 1 da neurofibromatose. Casos documentados de síndrome antifosfolípide associada que atendem aos critérios diagnósticos aceitos são excepcionalmente raros, com apenas três casos relatados na literatura. Aqui, relatamos um paciente com neurofibromatose tipo 1 e histórico de abortos espontâneos apresentando perda repentina de visão no olho direito e edema de cabeça do nervo óptico. A angiofluoresceínografia indicou neuropatia óptica isquêmica anterior. Ressonância magnética cerebral revelou achados compatíveis com meningioma do seio cavernoso esquerdo. O teste sorológico demonstrou anticorpos anti-b2 glicoproteína persistentemente elevados. Seus achados sugerem síndrome antifosfolípide com evidências clínicas e laboratoriais concomitantes de síndrome antifosfolipídica: abortos frequentes, episódio vaso-oclusivo e anticorpos antifosfolípides persistentemente elevados. Pelo nosso conhecimento, este caso pode representar a primeira manifestação neuro-oftálmica da síndrome antifosfolípide associada à neurofibromatose tipo 1.


Subject(s)
Humans , Female , Adult , Antiphospholipid Syndrome/complications , Neurofibromatosis 1/complications , Fluorescein Angiography/methods , Abortion, Spontaneous/etiology , Antiphospholipid Syndrome/diagnosis , Neurofibromatosis 1/diagnosis , Tomography, Optical Coherence/methods
10.
An. bras. dermatol ; 93(3): 397-404, May-June 2018. tab, graf
Article in English | LILACS | ID: biblio-949898

ABSTRACT

Abstract: Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes.


Subject(s)
Humans , Pigmentation Disorders/diagnosis , Purpura/diagnosis , Skin Diseases, Vascular/diagnosis , Purpura/etiology , Purpura/pathology , Skin/blood supply , Syndrome , Calciphylaxis/pathology , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/pathology , Skin Diseases, Vascular/pathology , Diagnosis, Differential , Purpura Fulminans/pathology
12.
Arch. argent. pediatr ; 115(6): 412-415, dic. 2017. ilus, tab
Article in Spanish | LILACS, BINACIS | ID: biblio-887403

ABSTRACT

Elsíndromeantifosfolipídicoes unaenfermedadautoinmunitaria en la cual se producen de forma persistente autoanticuerpos contra una variedad de fosfolípidos y proteínas transportadoras de estos. Ocurre en el 1, 8% de la población y solo el 2% de los casos son pediátricos. El espectro de manifestaciones clínicas es amplio: desde pacientes asintomáticos hasta una enfermedad amenazante para la vida como es el síndrome antifosfolipídico catastrófico. Cualquier órgano puede verse afectado como consecuencia de la trombosis a nivel de los grandes vasos o la microcirculación. Las manifestaciones más frecuentes en pediatría corresponden a trombosis venosas en el 60% de los pacientes, trombosis arterial en el 32%, alteraciones hematológicas en el 38% (plaquetopenia, leucopenia), alteraciones en la piel en el 18% (livedo reticularis, fenómeno de Raynaud) y alteraciones neurológicas en el 16%. Se presenta el caso clínico de una paciente pediátrica por la baja incidencia a esta edad.


The antiphospholipid syndrome is a multisystem autoimmune disease in which autoantibodies against a variety of phospholipids and phospholipid binding proteins are produced. It occurs in 1.8% of the population and only 2% of the cases are pediatric. The spectrum of clinical manifestations is wide from asymptomatic patients to a life-threatening disease like the catastrophic antiphospholipid syndrome. Any organ can be affected. The most frequent manifestations in pediatrics correspond to venous thrombosis in 60% of patients, arterial thrombosis in 32%, hematological disease in 38% (thrombocytopenia, leucopenia), skin alterations in 18% (livedo reticularis, Raynaud's phenomenon) and neurological signs in 16%. We describe the case of a previously healthy 14-year-old female patient diagnosed with antiphospholipid syndrome.


Subject(s)
Humans , Female , Adolescent , Antiphospholipid Syndrome/diagnosis , Pulmonary Embolism/etiology , Pulmonary Embolism/diagnostic imaging , Antiphospholipid Syndrome/complications
13.
Rev. Assoc. Med. Bras. (1992) ; 63(11): 994-999, Nov. 2017. tab, graf
Article in English | LILACS | ID: biblio-896312

ABSTRACT

Summary Antiphospholipid syndrome (APS) is an autoimmune disease characterized by antiphospholipid antibodies (aPL) associated with thrombosis and/or pregnancy morbidity. Most APS events are directly related to thrombotic events, which may affect small, medium or large vessels. Other clinical features like thrombocytopenia, nephropathy, cardiac valve disease, cognitive dysfunction and skin ulcers (called non-criteria manifestations) add significant morbidity to this syndrome and represent clinical situations that are challenging. APS was initially described in patients with systemic lupus erythematosus (SLE) but it can occur in patients without any other autoimmune disease. Despite the autoimmune nature of this syndrome, APS treatment is still based on anticoagulation and antiplatelet therapy.


Resumo A síndrome antifosfolipídide (APS) é uma doença autoimune caracterizada por tromboses e morbidade gestacional associadas à positividade de antiphospholipid antibodies (aPL). A maioria das manifestações da APS está diretamente relacionada aos eventos trombóticos, que podem afetar pequenos, médios ou grandes vasos. Outras manifestações como trombocitopenia, nefropatia, valvulopatia, disfunção cognitiva e úlceras cutâneas (chamadas de manifestações não critérios) agregam significativa morbidade e muitas vezes são refratárias ao tratamento convencional. Embora tenha sido inicialmente descrita em pacientes com lúpus eritematoso sistêmico (LES), a síndrome antifosfolípide também pode ocorrer em pacientes sem outras doenças autoimunes associadas. Apesar do caráter autoimune dessa síndrome, o tratamento da APS ainda é baseado na anticoagulação e na antiagregação plaquetária.


Subject(s)
Humans , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Antiphospholipid Syndrome/therapy , Diagnosis, Differential
14.
Med. interna (Caracas) ; 33(2): 72-83, 2017. ilus, tab
Article in Spanish | LILACS, LIVECS | ID: biblio-1009158

ABSTRACT

El síndrome antifosfolípido está definido por la combinación de manifestaciones clínicas trombóticas y/u obstétricas y un título persistentemente alto y significativo de anticuerpos antifosfolípidos. La presencia de múltiples trombos en lechos vasculares pequeños que lleva a falla multiorgánica, simultáneamente o en menos de 1 semana, define al síndrome antifosfolípido catastrófico el cual conlleva alta mortalidad; sin embargo, la sospecha diagnóstica y la institución temprana del tratamiento, definitivamente inciden en el pronóstico de éstos pacientes(AU)


Antiphospholipid syndrome is defined by the combination of thrombotic and/or obstetric clinical manifestations and a persistently high and significant title of antiphospholipid antibodies. The presence of multiple thrombi in small vascular beds leading to multi-organ failure that occurs simultaneously or in less than 1 week, and defines the catastrophic antiphospholipid syndrome which carries high mortality, The suspected diagnosis and early treatment affects the prognosis of these patients(AU)


Subject(s)
Humans , Male , Female , Heparin/administration & dosage , Antiphospholipid Syndrome/complications , Antibodies, Antiphospholipid/chemistry , Venous Thrombosis/etiology , Cardiovascular Diseases , Internal Medicine
15.
Rev. chil. reumatol ; 33(2): 58-64, 2017. ilus
Article in Spanish | LILACS | ID: biblio-1253716

ABSTRACT

El síndrome antifosfolípido (SAF) fue descrito y caracterizado durante la segunda mi-tad del siglo XX inicialmente como un fenómeno protrombótico secundario en con-texto de otras enfermedades del tejido conectivo, principalmente lupus. Sin embargo, el estudio de pacientes con enfermedad primaria impulsó a distintos consensos, tan-to clínicos como de laboratorio para su correcta identificación. Entre los pacientes con SAF destaca la forma de presentación catastrófica, de baja prevalencia, pero impor-tante por su mal pronóstico, caracterizada por el compromiso de múltiples sistemas en corto tiempo. Presentamos el caso de una paciente del Hospital Clínico San Borja-Arriarán con diag-nóstico de SAF primario, que presentó en su evolución la forma catastrófica. Este caso sirve de base para una revisión del proceso diagnóstico del SAF en relación a otras patologías reumatológicas y las características propias del SAF catastrófico.


Antiphospholipid syndrome (APS) was described and characterized during the second half of the 20th century initially as a secondary prothrombotic phenome-non in the context of other connective tissue diseases, mainly lupus. However, the study of patients with primary disease prompted different consensus, both clin-ical and laboratory for their correct identification. Among patients with APS, the catastrophic presentation is of low prevalence, but important because of its poor prognosis, characterized by the commitment of multiple systems in a short time. We present the case of a patient from the San Borja-Arriaran Clinic Hospital with di-agnosis of primary APS, which presented the catastrophic form in its evolution. This case serves as a basis for a review of the diagnostic process of APS in relation to other rheumatologic pathologies and the characteristics of catastrophic APS.


Subject(s)
Humans , Female , Middle Aged , Thrombosis/etiology , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/therapy , Tomography, X-Ray Computed , Antiphospholipid Syndrome/mortality , Antiphospholipid Syndrome/diagnostic imaging , Stroke , Ischemia
16.
Rev. centroam. obstet. ginecol ; 21(3): 69-76, jul.-sept. 2016. tab, ilus
Article in Spanish | LILACS | ID: biblio-869641

ABSTRACT

El síndrome antifosfolipídico fue definido por Hughes en 1983, describiendo episodios de trombocitopenia y abortos, cuya prevalencia aún es desconocida y puede ser detectado en el 15% de las...


The antiphospholipid syndrome was defined by Hughes in 1983, describing episodes of thrombocytopenia and abortions, whose prevalence is still unknown and can be detected in 15% of women of...


Subject(s)
Humans , Female , Abortion , Antibodies, Antiphospholipid , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/etiology , Thrombocytopenia
17.
Rev. bras. reumatol ; 56(1): 2-7, jan.-fev. 2016. tab
Article in English | LILACS | ID: lil-775214

ABSTRACT

Resumo Introdução: A crioglobulinemia é uma vasculite de pequenos vasos mediada por imunocomplexos que normalmente envolvem a pele, os rins e os nervos periféricos. A síndrome antifosfolipídica (SAF) é um transtorno da hipercoagulabilidade autoimune que provoca trombose dos vasos sanguíneos. Pode se manifestar como um distúrbio microtrombótico que afeta múltiplos órgãos, denominado SAF catastrófica. Objetivo: Esta série de casos objetiva descrever os desafios de diagnóstico e tratamento que surgem quando esses dois graves transtornos estão presentes simultaneamente no mesmo paciente. Métodos: Foram descritos quatro pacientes internados em nosso hospital em decorrência de danos graves a múltiplos órgãos mediados pela vasculite crioglobulinêmica com SAF concomitante. Resultados: As manifestações clínicas incluíram úlceras de perna, livedo reticular, insuficiência renal e neuropatia periférica. As etiologias sugeridas para a combinação de síndromes foram a hepatite C, o lúpus eritematoso sistêmico e a doença mieloproliferativa retal associada a linfoma de zona marginal tipo células B. Todos os pacientes foram tratados com anticoagulantes, altas doses de corticosteroides, rituximabe, gamaglobulinas intravenosas e troca de plasma. Conclusão: A rara associação entre a SAF grave ou catastrófica e a crioglobulinemia deve ser considerada por médicos que atendem pacientes com isquemia ou necrose de múltiplos órgãos.


Abstract Background: Cryoglobulinemia is an immune-complex-mediated small vessel vasculitis that classically involves the skin, kidneys and peripheral nerves. Antiphospholipid syndrome (APS) is an autoimmune hypercoagulable disorder which causes blood vessel thrombosis. It can present as a multi-organ microthrombotic disorder which is called catastrophic APS. Objective: In this case series we aim to describe the diagnostic and management challenges that arise when these two severe disorders simultaneously present in the same patient. Methods: We describe four patients who were admitted to our hospital due to multi-organ life threatening damage mediated by cryoglobulinemic vasculitis with concurrent APS. Results: Clinical manifestations included leg ulcers, livedo reticularis, renal failure, and peripheral neuropathy. Suggested etiologies for the combined syndromes were hepatitis C, systemic lupus erythematosus and myeloproliferative disease rectal maltoma. All of our patients were treated with anticoagulation, high-dose corticosteroids, rituximab, intravenous gammaglobulins and plasma exchange. Conclusion The rare association of severe or catastrophic APS with cryoglobulinemia in patients should be considered by physicians who treat patients with multi-organ ischemia or necrosis.


Subject(s)
Humans , Antiphospholipid Syndrome/complications , Cryoglobulinemia/complications , Immunosuppressive Agents/therapeutic use , Plasma Exchange , Antiphospholipid Syndrome/prevention & control , Cryoglobulinemia/prevention & control , Rituximab
18.
Med. interna (Caracas) ; 32(2): 124-128, 2016. ilus
Article in Spanish | LILACS, LIVECS | ID: biblio-1009548

ABSTRACT

El Síndrome Antifosfolípido (SAF) es una enfermedad auto-inmune que puede afectar vasos sanguíneos en todos los segmentos vasculares generando manifestaciones, incluyendo oculares, relacionadas con hipercoagulabilidad. La forma más frecuente de presentación ocular es trombosis retiniana, cuyos mecanismos desencadenantes aún no están totalmente claros. Los niveles elevados de anticuerpos antifosfolípidos (aPL) son el mecanismo principal de la trombosis en este síndrome. La incidencia de manifestaciones oculares está entre 14-18%, y por eso requiere gran atención en el abordaje de pacientes con SAF primario o secundario. Se presenta caso de paciente femenino de 32 años de edad con diagnóstico de SAF primario en tratamiento, refiere inicio de enfermedad actual en febrero 2016 cuando presenta rash eritematoso en cara, cuello y región superior del tórax, concomitante artralgias generalizadas; se asocia fiebre cuantificada en 39°C y evacuaciones líquidas por lo cual acude e ingresa al Hospital Universitario de Caracas. Durante la hospitalización, refiere escotomas y súbitamente pérdida de visión en ojo derecho, por lo que se realiza examen físico ocular, campimetría y fondo de ojo, los cuales resultan anormales. Este caso es presentado con el fin de realizar abordaje diagnóstico y terapéutico de acuerdo a lo reportado en la literatura(AU)


Antiphospholipid Syndrome (APS) is an autoimmune disease that can affect a wide range of blood vessels presenting clinical features, including ophtalmologic, linked to hypercoagulability. A retinal thrombosis is the commonest presentation in the ocular spectrum but the whose trigger mechanisms are not yet clear. A high level of antiphospholipid antibodies is the main mechanism proposed to explain thrombosis in this syndrome. The accumulated incidence of ocular manifestations range from 14-18%, which makes the ophthalmologic evaluation of great importance when assessing patients with either primary or secondary APS. We present the case of a 32 year-old feminine patient with previous APS diagnosis, who consulted for a rash in face, neck and chest, plus universal arthralgies, fever and liquid stools. During her hospitalization, she refered sudden visual loss of her right eye; the medical team made a complete physical evaluation, visual field test and funduscopy were done and the results were abnormal(AU)


Subject(s)
Humans , Female , Adult , Retinal Vein Occlusion/etiology , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/physiopathology , Blindness , Immune System Diseases , Internal Medicine
19.
Rev. bras. reumatol ; 55(2): 159-166, Mar-Apr/2015. tab, graf
Article in Portuguese | LILACS | ID: lil-746140

ABSTRACT

Introdução: A discussão sobre a intensidade de varfarina em pacientes com síndrome antifosfolípide (SAF) permanece presente nos dias atuais. Objetivos: Avaliar qual intensidade de anticoagulação com varfarina está associada com maior redução de eventos tromboembólicos no tratamento de pacientes com SAF, assim como avaliar o risco de hemorragia nas diferentes modalidades de tratamento. Metodologia: Foi realizada uma revisão sistemática da literatura a partir de busca nas bases de dados eletrônicos: PubMed, LILACS e SciELO, sendo utilizadas as palavras-chave: treatment, warfarin, antiphospholipid syndrome, antiphospholipid antibody syndrome e suas respectivas traduções para o português, em diferentes combinações. Também foi realizada uma meta-análise com auxílio do programa Review Manager 5.2 da Cochrane. Resultados: Apenas dois artigos preencheram os critérios para inclusão neste estudo. Em relação ao principal desfecho avaliado neste trabalho, os dois estudos apresentaram valores similares, demonstrando maior frequência de eventos trombóticos nos grupos de alta intensidade. A análise comparativa dos ensaio clínicos randomizados avaliados demonstrou um risco trombótico aumentado para aqueles pacientes que receberam intervenção com varfarina em alta intensidade. Outro achado da meta-análise foi a maior ocorrência de hemorragia menor também no grupo experimental, que recebeu varfarina mantendo Razão Normalizada Internacional (RNI) > 3. Conclusão: Nos indivíduos com SAF e predominância de eventos venosos, o uso de anticoagulação em moderada intensidade (MI) (RNI: 2-3) está mais indicado. Por outro lado, essa evidência ainda não pode ser estendida aos pacientes com eventos arteriais, pela limitada representação dessa amostra de sujeitos nos dois estudos clínicos incluídos nesta meta-análise. .


Introduction: Discussion about the intensity of warfarin in patients with antiphospholipid syndrome (APS) remains present in our days. Objectives: To evaluate which intensity of anticoagulation with warfarin is associated with a greater reduction of thromboembolic events in the treatment of patients with APS, as well as assess the risk of bleeding in the different treatment modalities. Methodology: A systematic review of the literature was carried out with search from electronic databases: PubMed, LILACS and SciELO, with the use of the key-words: treatment, warfarin, antiphospholipid syndrome, antiphospholipid antibody syndrome and their respective translations into Portuguese, in different combinations. In addition, a meta-analysis with the aid of Review Manager 5.2 software by Cochrane was performed. Results: Only two articles met the inclusion criteria for this study. Regarding the main outcome assessed in this study, the two studies showed similar values, indicating higher frequency of thrombotic events in high-intensity groups. The comparative analysis of the randomized clinical trial evaluated showed an increased thrombotic risk for those patients who received intervention with high-intensity warfarin. Another finding of the meta-analysis was the higher incidence of minor bleeding, also in the experimental group, that received warfarin keeping International Normalized Ratio (INR) > 3. Conclusion: In individuals with APS and prevalence of venous events, the use of moderate intensity (MI) anticoagulation (INR: 2-3) is the most suitable. However, this evidence cannot yet be extended to patients with arterial events, due to the limited representation of this sample of subjects in the two clinical trials included in this meta-analysis. .


Subject(s)
Humans , Anticoagulants/administration & dosage , Antiphospholipid Syndrome/complications , Thrombosis/drug therapy , Thrombosis/etiology , Warfarin/administration & dosage , Anticoagulants/adverse effects , Hemorrhage/chemically induced , Hemorrhage/epidemiology , Risk Assessment , Warfarin/adverse effects
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