RESUMO
ABSTRACT Case report of a patient with an immunodeficiency who demands regular replacement of intravenous immunoglobulin. She presented an episode of transfusion-related acute lung injury shortly after using an immunoglobulin product different than the one she usually received. The patient evolved with respiratory changes (hypoxia, dyspnea, change in pulmonary auscultation) minutes after the end of the infusion, and received non-invasive respiratory support. She was discharged after 36 hours with good outcome. The patient achieved full recovery, showing no further reactions in subsequent immunoglobulin infusions (no longer receiving the product that was used when she had the episode of transfusion-related acute lung injury). Although rare, this reaction is potentially serious and has no specific treatment other than supportive therapy. The literature is scarce regarding the risk of recurrence. The decision on whether to proceed with immunoglobulin therapy after this adverse effect should be analyzed individually, assessing the possible risks and benefits for the patient.
RESUMO Relato de caso de paciente com imunodeficiência que necessitava de reposição regular de imunoglobulina endovenosa. Ela apresentou um episódio de lesão pulmonar aguda relacionada à transfusão após uso de produto de imunoglobulina diferente daquele que recebia habitualmente. Evoluiu com alterações respiratórias (hipóxia, dispneia e alteração de ausculta pulmonar) minutos após o fim da infusão, necessitando de suporte respiratório não invasivo. A paciente recebeu alta hospitalar após 36 horas, com boa evolução. Obteve recuperação total dos sintomas, sem mais reações nas infusões subsequentes de imunoglobulina (sendo optado por não mais prescrever o produto que foi usado quando ocorreu o episódio de lesão pulmonar aguda relacionada à transfusão). Apesar de rara, essa reação é potencialmente grave, não possui tratamento específico além de terapia de suporte, e há pouca informação na literatura sobre o risco de recorrência. A decisão sobre o seguimento da terapia com imunoglobulina após esse efeito adverso deve ser analisada individualmente, avaliando os possíveis riscos e benefícios para o paciente.
Assuntos
Humanos , Masculino , Feminino , Adulto , Idoso , Lesão Pulmonar Aguda Relacionada à Transfusão , Síndromes de Imunodeficiência , Pneumopatias , Infusões Intravenosas , Imunoglobulinas Intravenosas/efeitos adversos , Pessoa de Meia-IdadeRESUMO
ABSTRACT In the last few years, new primary immunodeficiencies and genetic defects have been described. Recently, immunoglobulin products with improved compositions and for subcutaneous use have become available in Brazil. In order to guide physicians on the use of human immunoglobulin to treat primary immunodeficiencies, based on a narrative literature review and their professional experience, the members of the Primary Immunodeficiency Group of the Brazilian Society of Allergy and Immunology prepared an updated document of the 1st Brazilian Consensus, published in 2010. The document presents new knowledge about the indications and efficacy of immunoglobulin therapy in primary immunodeficiencies, relevant production-related aspects, mode of use (routes of administration, pharmacokinetics, doses and intervals), adverse events (major, prevention, treatment and reporting), patient monitoring, presentations available and how to have access to this therapeutic resource in Brazil.
RESUMO Nos últimos anos, novas imunodeficiências primárias e defeitos genéticos têm sido descritos. Recentemente, produtos de imunoglobulina, com aprimoramento em sua composição e para uso por via subcutânea, tornaram-se disponíveis em nosso meio. Com o objetivo de orientar o médico no uso da imunoglobulina humana para o tratamento das imunodeficiências primárias, os membros do Grupo de Assessoria em Imunodeficiências da Associação Brasileira de Alergia e Imunologia produziram um documento que teve por base uma revisão narrativa da literatura e sua experiência profissional, atualizando o I Consenso Brasileiro publicado em 2010. Apresentam-se novos conhecimentos sobre indicações e eficácia do tratamento com imunoglobulina nas imunodeficiências primárias, aspectos relevantes sobre a produção, forma de utilização (vias de administração, farmacocinética, doses e intervalos), efeitos adversos (principais efeitos, prevenção, tratamento e notificação), monitorização do paciente, apresentações disponíveis e forma de obtenção deste recurso terapêutico em nosso meio.
Assuntos
Humanos , Imunoglobulinas/uso terapêutico , Consenso , Fatores Imunológicos/uso terapêutico , Administração Cutânea , Brasil , Resultado do Tratamento , Administração Intravenosa , Síndromes de Imunodeficiência , Fatores Imunológicos/provisão & distribuiçãoRESUMO
Resumo A combinação de imunossupressores faz parte do protocolo de tratamento de pacientes submetidos a um transplante renal (TR). A Thymoglobuline®, imunoglobulina policlonal de coelho dirigida contra timócitos humanos, é o agente mais usado como terapia de indução no TR nos Estados Unidos. No Brasil, a Thymoglobuline® está aprovada para uso em pacientes que foram submetidos a transplante e, apesar de ser amplamente utilizada, ainda existem controvérsias em relação ao seu modo de uso. Realizamos uma revisão sistemática da literatura avaliando os estudos que utilizaram a Thymoglobuline® na indução e no tratamento de rejeição em pacientes submetidos ao TR. A revisão utilizou os bancos de dados computadorizados da EMBASE, LILACS e MedLine e dos trabalhos selecionados foram extraídas informações sobre os dados gerais dos pacientes, as características metodológicas e as variáveis analisadas em cada estudo. Dos resultados obtidos, desenvolvemos um guia prático sobre o uso de Thymoglobuline® em pacientes transplantados renais.
Abstract The combination of immunosuppressive drugs is part of the treatment regimen of patients undergoing kidney transplantation (RT). Thymoglobulin®, a rabbit immunoglobulin directed against human thymocytes, is the most commonly agent used for induction therapy in RT in the US. In Brazil, Thymoglobulin® is approved by ANVISA for the use in patients who underwent kidney transplantation and despite being widely used, there are controversies regarding the drug administration. We prepared a systematic review of the literature, evaluating studies that used Thymoglobulin® for induction and for acute rejection treatment in patients undergoing RT. The review used the computadorized databases of EMBASE, LILACS and MedLine. Data were extracted from the studies concerning general features, methodological characteristics and variables analyzed in each study. From the results, a practical guide was prepared analyzing various aspects on the use of Thymoglobulin® in patients submitted to RT.
Assuntos
Humanos , Soro Antilinfocitário/uso terapêutico , Rejeição de Enxerto/tratamento farmacológico , Rejeição de Enxerto/prevenção & controle , Imunossupressores/uso terapêutico , Transplante de Rim , Guias de Prática Clínica como AssuntoRESUMO
Fundamento: el Síndrome de Stevens-Johnson (SSJ) es una enfermedad grave, a menudo fatal, que ha sido considerada como un tipo de eritema multiforme, es causada generalmente por fármacos y de no ser diagnosticado y tratado de forma oportuna puede asociarse a secuelas importantes y la muerte. Objetivo: describir y reseñar el tratamiento y evolución clínica del cuadro clínico de tres casos con diagnóstico de Síndrome de Stevens-Johnson, ingresados en Cuidados Intensivos. Presentación de casos: se presentan tres enfermos, dos pertenecientes al sexo femenino y uno al masculino, con diagnóstico de SSJ asociados al empleo de fármacos. En los tres casos el tratamiento consistió en medidas de cuidados generales para la profilaxis y el tratamiento de complicaciones, esteroides sistémicos y además IgG intravenosa de producción nacional (intacglobin). Los tres pacientes evolucionaron de forma satisfactoria. Resultados: se destaca la utilidad del uso de inmunoglobulina G intravenosa en el tratamiento de estos pacientes, así como su importancia en una unidad de cuidados para enfermos graves. Conclusiones: en la casuística predominó el sexo femenino y el empleo precoz del Intacglobin (IgG IV) que contribuyó a una mejor evolución de los pacientes al detener la progresión de la enfermedad, evitar complicaciones y disminuir la estadía en las unidades de enfermos graves.
Background: Stevens-Johnsons syndrome (SJS) is a serious disease, fatal most of the time, which has been considered as a type of erythema multiforme. It is generally caused by medicaments. If it is not diagnosed and treated at appropriate time it can be associated to considerable sequelae and death. Objective: to describe the treatment and evolution of the clinical manifestations of three cases with the diagnosis of Stevens-Johnsons syndrome admitted in the intensive care unit. Cases presentation: the cases of two female patients and a male patient with the diagnosis of SJS, associated to the use of medicaments, are briefly presented. The treatment consisted of measures of general care for the prophylaxis and treatment of complications for the three cases. The patients were treated with intravenous IgG of national production (intacglobin) and systemic steroids. The three patients improved their condition satisfactorily. Results: the use of intravenous immunoglobulin G in the treatment of these patients stands out; as well as the importance of the treatment of seriously ill patients in an intensive care unit. Conclusions: female sex predominated in the casuistics. The early use of Intacglobin (IgG IV) contributed to a better improvement of the patients condition arresting the development of the disease, avoiding complications and decreasing the hospital stay of seriously ill patients.
RESUMO
Background: Guillain-Barré syndrome (GBS) is the commonest cause of acute flaccid paralysis worldwide, with an incidence of 0.6-4 per 100.000 inhabitants per year. It affects all age groups and carries an incapacity burden of up to 20%. Aim: To describe the features of GBS in adult Chilean patients admitted to a tertiary care hospital. Material and Methods: Review of medical records of 41 patients aged 17 to 81 years (30 males) admitted to a public hospital with the diagnosis of GBS between 2003 and 2009. According to clinical and electrophysiological criteria, the patients were classified into different varieties of GBS. Results: The incidence of GBS was higher in males (2.7:1) and the demyelinated GBS variety was found in 66% of cases. According to the Hughes disability score, patients treated with plasmapheresis, showed non-statistically significant better outcomes than those treated with intravenous immunoglobulin. Conclusions: In this group of patients the demyelinated variety of GBS was more common than the axonal type. Although not statistically significant, the better response to plasmapheresis is encouraging and should prompt a controlled study.
Assuntos
Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Dislipidemias/epidemiologia , Síndrome Metabólica/epidemiologia , Insuficiência Renal Crônica/epidemiologia , Classe Social , Pessoal Administrativo , Fatores Etários , Índice de Massa Corporal , Estudos de Coortes , Comorbidade , Estudos Transversais , Taxa de Filtração Glomerular , Obesidade , Obesidade Abdominal/epidemiologia , Insuficiência Renal Crônica/fisiopatologiaRESUMO
A anemia é frequente em pacientes após o transplante renal (TxR) e sua prevalência varia conforme o tempo pós-transplante e os critérios diagnósticos empregados. A infecção pelo Parvovírus B19 (PV B19) é causa subdiagnosticada de anemia nesta população. Para ilustrar a epidemiologia e espectro clínico, apresentamos caso de PV B19 que evoluiu com aplasia pura de série vermelha (APSV), ressaltando as dificuldades do diagnóstico e tratamento. O emprego da detecção do DNA viral pela reação em cadeia da polimerase e do diagnóstico das alterações da morfologia da medula óssea são particularmente úteis para o diagnóstico no paciente transplantado imunossuprimido que falha na produção da resposta humoral contra o PV B19.
Anemia is frequent in kidney transplant patients, and its prevalence varies according to posttransplant time and the adopted diagnostic criteria. Parvovirus B19 (PV B19) infection is an underdiagnosed cause of anemia in this particular population. To illustrate epidemiologic and clinical data regarding it, we present a case of PV B19 infection complicated by pure red cell aplasia (PRCA), pointing out the pitfalls we encountered in diagnosis and treatment. The use of viral DNA detection by polymerase chain reaction (PCR), and correct interpretation of morphological features of bone marrow histology are particularly important for the diagnosis of this condition in kidney transplant patients, who fail to develop a proper humoral response against PV B19, thus importantly decreasing the sensitivity of serological methods in this setting.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Transplante de Rim/efeitos adversos , Infecções por Parvoviridae/complicações , Aplasia Pura de Série Vermelha/virologia , Doença Crônica , Infecções por Parvoviridae/etiologiaRESUMO
Stevens-Johnson's syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatoses, that lead to keratinocyte apoptosis induced by interactions between Fas (cell death receptor) and soluble Fas-ligand, present in serum of Stevens-Johnson's syndrome / toxic epidermal necrolysis patients. Anti-Fas antibodies in intravenous immunoglobulin (IVIG) would block the apoptosis cascade. Three cases of toxic epidermal necrolysis occurred in one male and two female patients, after use of allopurinol, leprosy multidrug therapy concomitant with dipyrone, and diclofenac. The cases were treated with intravenous immunoglobulin 2-3 mg/kg and prednisone 20-50 mg/day. The interruption of new lesions outbreak and reepithelization were extremely fast after the use of intravenous immunoglobulin, without adverse effects. Controlled studies are needed to confirm the efficacy of intravenous immunoglobulin in Stevens-Johnson's syndrome / toxic epidermal necrolysis, but the results seem promising.
A Síndrome de Stevens-Johnson e a Necrólise Epidérmica Tóxica são dermatoses graves, que levam à apoptose dos queratinócitos induzida pela interação entre Fas (receptor de morte celular) e Fasligante solúvel, presente no soro de pacientes com Síndrome de Stevens-Johnson e Necrólise Epidérmica Tóxica. Anticorpos anti-Fas contidos na imunoglobulina endovenosa podem bloquear esta cascata apoptótica. Três casos de Necrólise Epidérmica Tóxica são descritos, ocorrendo após uso de alopurinol, diclofenaco e poliquimioterapia para hanseníase concomitante com dipirona. Os três casos foram tratados com imunoglobulina endovenosa 2-3 mg/kg, divididos em 4 ou 5 dias e prednisona 20-50 mg/dia. A interrupção no surgimento de novas lesões e a repitelização foram extremamente rápidas, sem ocorrência de efeitos adversos. Estudos controlados são necessários para confirmar a eficácia da imunoglobulina endovenosa na Síndrome de Stevens-Johnson e Necrólise Epidérmica Tóxica, porém, seus resultados parecem ser promissores.
Assuntos
Feminino , Humanos , Pessoa de Meia-Idade , Glucocorticoides/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Prednisona/uso terapêutico , Síndrome de Stevens-Johnson , Quimioterapia Combinada/métodos , Resultado do TratamentoRESUMO
A Doença de Kawasaki é vasculite sistêmica febril de etiologia desconhecida e importante causa cardiopatia adquirida na infância, principalmente no primeiro ano de vida, onde são mais comuns os casos atípicos. Os casos de Doença de Kawasaki Atípica (DKA) ou incompleta podem não preencher o número de critérios diagnósticos clássicos e apresentar sintomas pouco frequentes, atrasando o diagnóstico, o que aumenta o risco de doença coronariana. Descrevemos aqui um caso de DKA no qual sintomas oftalmológicos e neurológicos foram observados, além de um aumento importante de transaminases, alertando que os critérios clássicos podem ser restritivos, atrasar o diagnóstico e o tratamento precoce e efetivo dos casos atípicos.(AU)
Kawasaki disease is a systemic febrile vasculitis which etiology remains unknown and is an important cause of acquired heart disease during childhood, mainly in the first year of life, when atypical cases are more frequent. Atypical Kawasaki Disease (AKD) or incomplete cases may not fulfill the classic diagnostic criteria and present anusual symptoms, delaying the diagnosis and increasing the risk of coronary damage. Herein we report a case of atypical Kawasaki Disease, in which ophthalmologic and neurologic symptoms were observed besides an important rise in transaminases levels. We report warns that over reliance on classical criteria may be restrictive, may delay the diagnosis and prevent the effective and early treatment in atypical cases.(AU)
Assuntos
Humanos , Feminino , Lactente , Imunoglobulinas Intravenosas/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/diagnósticoRESUMO
Human immunoglobulin (Ig) began to be applied in the clinical practice with the treatment of primary immunodeficiencies. Quickly, applications of Ig increased, as its anti-inflammatory and immunomodulatory functions were elucidated. Currently, Ig is the most commonly used blood product. Ig is obtained by processing plasma; methods, in particular, techniques to reduce plasma viral loads have been evolving over the years and include: pasteurization, solvent/ detergent treatment, caprylic acid treatment and nanofiltration. These methods contribute to increased safety and quality of blood products. The mechanisms of action of Ig not only involve the blockade of Fc receptors of phagocytes, but also control complement pathways, idiotype-anti-idiotype dimer formation, blockage of superantigen binding to T cells, inhibition of dendritic cells and stimulation of regulatory T cells (Tregs). There are several formulations of Ig available, each one with its own peculiar characteristics. In Brazil, there is stringent legislation regulating the quality of Ig. Only Ig products that completely fulfill the quality control criteria are released for use. These standards involve different tests from visual inspection to determination of anti-complementary activity. This paper will further review the history and current status of Ig, including its production and mechanisms of action. The formulations available in Brazil and also the criteria of quality control currently applied will be presented.
Assuntos
Hemoderivados , Sistema Imunitário , Imunoglobulinas , Imunoglobulinas Intravenosas/uso terapêutico , Farmacocinética , PlasmaRESUMO
Human immunoglobulin (Ig) began to be applied in the clinical practice with the treatment of primary immunodeficiencies. Quickly, applications of Ig increased, as its anti-inflammatory and immunomodulatory functions were elucidated. Currently, Ig is the most commonly used blood product. Ig is obtained by processing plasma; methods, in particular, techniques to reduce plasma viral loads have been evolving over the years and include: pasteurization, solvent/ detergent treatment, caprylic acid treatment and nanofiltration. These methods contribute to increased safety and quality of blood products. The mechanisms of action of Ig not only involve the blockade of Fc receptors of phagocytes, but also control complement pathways, idiotype-anti-idiotype dimer formation, blockage of superantigen binding to T cells, inhibition of dendritic cells and stimulation of regulatory T cells (Tregs). There are several formulations of Ig available, each one with its own peculiar characteristics. In Brazil, there is stringent legislation regulating the quality of Ig. Only Ig products that completely fulfill the quality control criteria are released for use. These standards involve different tests from visual inspection to determination of anti-complementary activity. This paper will further review the history and current status of Ig, including its production and mechanisms of action. The formulations available in Brazil and also the criteria of quality control currently applied will be presented.
RESUMO
A epidermólise bolhosa adquirida é doença bolhosa subepidérmica crônica e rara. Geralmente, inicia-se na fase adulta, sendo a etiologia desconhecida, embora vinculada à presença de anticorpos contra o colágeno tipo VII. Há formação de bolhas, espontaneamente ou após trauma, podendo causar complicações graves. O tratamento é desapontador e difícil. Além da terapia convencional com corticoides sistêmicos, recentemente, novas modalidades terapêuticas promissoras estão sendo utilizadas, dentre elas, a imunoglobulina intravenosa. Destaca-se, neste relato, o difícil manejo clínico desta doença, e a melhora importante com a imunoglobulina intravenosa.
Acquired bullous epidermolysis is a chronic and rare bullous subepidermal disease. It usually begins in adulthood and its etiology is unknown although it is associated with antibodies against type VII collagen. There are spontaneous and trauma induced formation of blisters that may cause serious complications. Treatment is disappointing and difficult. Apart from conventional therapy with systemic corticosteroid, new therapeutic modalities such as intravenous immunoglobulin are currently being used. This report highlights the extremely difficult clinical management of this rare disease and the important improvement provided by intravenous immunoglobulin.