RESUMO
Psoriatic arthritis (PsA) is chronic disease that compromises multiple domains and might be associated with progressive joint damage, increased mortality, functional limitation, and considerably impaired quality of life. Our objective was to generate evidence-based recommendations on the management of PsA in Pan American League of Associations for Rheumatology (PANLAR) countries. We used the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE)-ADOLOPMENT approach to adapt the 2019 recommendations of the European Alliance of Associations for Rheumatology. A working group consisting of rheumatologists from various countries in Latin America identified relevant topics for the treatment of PsA in the region. The methodology team updated the evidence and synthesized the information used to generate the final recommendations. These were then discussed and defined by a panel of 31 rheumatologists from 15 countries. Theses guidelines report 15 recommendations addressing therapeutic targets, use of antiinflammatory agents and corticosteroids, treatment with disease-modifying antirheumatic drugs (conventional synthetic, biologic, and targeted synthetic), therapeutic failure, optimization of biologic therapy, nonpharmacological interventions, assessment tools, and follow-up of patients with PsA. Here we present a set of recommendations to guide decision making in the treatment of PsA in Latin America, based on the best evidence available, considering resources, medical expertise, and the patient's values and preferences. The successful implementation of these recommendations should be based on clinical practice conditions, healthcare settings in each country, and a tailored evaluation of patients.
Assuntos
Humanos , Artrite Psoriásica/tratamento farmacológico , Artrite Psoriásica/diagnóstico , Corticosteroides , Antirreumáticos/uso terapêutico , Anti-InflamatóriosRESUMO
OBJECTIVE: Psoriatic arthritis (PsA) is chronic disease that compromises multiple domains and might be associated with progressive joint damage, increased mortality, functional limitation, and considerably impaired quality of life. Our objective was to generate evidence-based recommendations on the management of PsA in Pan American League of Associations for Rheumatology (PANLAR) countries. METHODS: We used the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE)-ADOLOPMENT approach to adapt the 2019 recommendations of the European Alliance of Associations for Rheumatology. A working group consisting of rheumatologists from various countries in Latin America identified relevant topics for the treatment of PsA in the region. The methodology team updated the evidence and synthesized the information used to generate the final recommendations. These were then discussed and defined by a panel of 31 rheumatologists from 15 countries. RESULTS: Theses guidelines report 15 recommendations addressing therapeutic targets, use of antiinflammatory agents and corticosteroids, treatment with disease-modifying antirheumatic drugs (conventional synthetic, biologic, and targeted synthetic), therapeutic failure, optimization of biologic therapy, nonpharmacological interventions, assessment tools, and follow-up of patients with PsA. CONCLUSION: Here we present a set of recommendations to guide decision making in the treatment of PsA in Latin America, based on the best evidence available, considering resources, medical expertise, and the patient's values and preferences. The successful implementation of these recommendations should be based on clinical practice conditions, healthcare settings in each country, and a tailored evaluation of patients.
Assuntos
Antirreumáticos , Artrite Psoriásica , Reumatologia , Artrite Psoriásica/tratamento farmacológico , Artrite Psoriásica/terapia , Humanos , Antirreumáticos/uso terapêutico , Reumatologia/normas , Sociedades Médicas , América Latina , Medicina Baseada em Evidências , Qualidade de Vida , Anti-Inflamatórios/uso terapêutico , Corticosteroides/uso terapêuticoRESUMO
Considerable variability exists in the way health-care providers treat patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in Latin America. The most frequently used treatments for ANCA-associated vasculitis are cyclophosphamide and prolonged glucocorticoid tapers; however, randomised controlled trials conducted over the past 30 years have led to the development of several evidence-based treatment alternatives for these patients. Latin America faces socioeconomic challenges that affect access to care, and the use of certain costly medications with proven efficacy ANCA-associated vasculitis is often restricted. For these reasons, the Pan American League of Associations for Rheumatology developed the first ANCA-associated vasculitis treatment guidelines tailored for Latin America. A panel of local vasculitis experts generated clinically meaningful questions related to the treatment of ANCA-associated vasculitis using the Population, Intervention, Comparator, and Outcome (PICO) format. Following the Grading of Recommendations Assessment, Development, and Evaluation methodology, a team of methodologists conducted a systematic literature review. The panel of vasculitis experts voted on each PICO question and made recommendations, which required at least 70% agreement among the voting members. 21 recommendations and two expert opinion statements for the treatment of ANCA-associated vasculitis were developed, considering the current evidence and the socioeconomic characteristics of the region. These recommendations include guidance for the use of glucocorticoids, non-glucocorticoid immunosuppressants, and plasma exchange.
Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Reumatologia , Humanos , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Anticorpos Anticitoplasma de Neutrófilos , Glucocorticoides/uso terapêutico , Troca Plasmática , PlasmafereseRESUMO
El síndrome POEMS es un trastorno paraneoplásico raro y poco frecuente, que se presenta principalmente en la sexta década de la vida, caracterizado por el compromiso multisistémico con predominio de neuropatía desmielinizante. Abarca diversas y heterogéneas manifestaciones clínicas y su diagnóstico requiere un alto índice de sospecha. Se presentan dos casos de pacientes que consultaron por cuadros poco frecuentes en los que la pérdida de la fuerza orientó al acercamiento de una afectación multisistémica que concluyó con el diagnóstico de esta enfermedad(AU)
POEMS syndrome is a rare and infrequent paraneoplastic syndrome, which occurs mainly in the sixth decade of life, characterized by multisystem involvement with a predominance of demyelinating neuropathy, which encompasses diverse and heterogeneous clinical manifestations and whose diagnosis requires a high index of suspicion. We present two cases of patients who consulted due to unusual symptoms and whose loss of strength led to an approach due to multisystem involvement that concluded with the diagnosis of this disease(AU)
Assuntos
Humanos , Masculino , Feminino , Paraproteinemias , Polineuropatias/epidemiologia , Síndrome POEMS/diagnóstico , Colômbia , Doenças do Sistema Endócrino/epidemiologiaRESUMO
ABSTRACT Brucellosis is a zoonosis that causes a multi-organ granulomatous infection. It has diverse and non-specific clinic features that can make diagnosis difficult. Medical personnel often do not recognize it early. Delayed treatment is associated with high morbidity and even mortality. Its timely diagnosis requires a high index of suspicion. The case is presented of a 35-year-old male zootechnologist, previously healthy, with a progressive picture of two months of evolution of irradiated low back pain to the left hip, nocturnal diaphoresis, and unintentional weight loss. Elevation of acute phase reactants was documented and magnetic resonance imaging found signs of iliopsoas tendonitis and inflammatory changes in the left sacroiliac joint. The IgG and IgM antibodies using an immunoassay forbrucella were positive. After establishing antibiotic treatment, a marked clinical improvement, with resolution of the inflammatory process was evident.
RESUMEN La brucelosis es una zoonosis que genera una infección granulomatosa multiorgánica. Tiene una clínica diversa e inespecífica que puede hacer difícil el diagnóstico. Con frecuencia, el personal médico no la reconoce de forma temprana. El retraso en el tratamiento se asocia con una gran morbilidad e incluso mortalidad. Su diagnóstico oportuno requiere un alto índice de sospecha. Se presenta el caso de un hombre de 35 arios, zootecnista, previamente sano, con un cuadro progresivo de dos meses de evolución de dolor lumbar irradiado a cadera izquierda, diaforesis nocturna y pérdida no intencional de peso. Se documentó elevación de reactantes de fase aguda y en la resonancia magnética se encontraron signos de tendinitis del psoas y cambios inflamatorios en la articulación sacroilíaca izquierda. Los anticuerpos IgG e IgM por inmunoensayo para Brucella fueron positivos, y luego de instaurar tratamiento antibiótico se evidenció marcada mejoría clínica con resolución del proceso inflamatorio.
Assuntos
Humanos , Animais , Adulto , Doenças Musculoesqueléticas , Espondilite , Infecções Bacterianas e Micoses , Doenças Ósseas Infecciosas , Brucelose , Sacroileíte , InfecçõesRESUMO
ABSTRACT Inclusion body myositis is part of the group of inflammatory myopathies, representing 30% of this group of diseases, and is considered an orphan disease because its estimated prevalence is less than 5 per 10,000 inhabitants. It produces weakness and atrophy of the proximal and distal muscles. The pathophysiological mechanisms are mainly autoimmune, inflammatory, and degenerative. The cases are presented of two female patients who came to : the emergency department due to progressive loss of upper and lower limb strength, and progressive asymmetric muscle weakness.
RESUMEN La miositis por cuerpos de inclusión forma parte del grupo de las miopatías inflamatorias, de las que representa el 30%; es considerada una enfermedad huérfana, ya que se estima que su prevalencia es menor a 5 por cada 10.000 habitantes. Produce debilidad y atrofia de los músculos proximales y distales. Los mecanismos fisiopatológicos son principalmente autoinmunes, inflamatorios y degenerativos. Se presentan 2 casos de mujeres, quienes acudieron a urgencias por pérdida progresiva de la fuerza en miembros superiores e inferiores, con debilidad muscular asimétrica de curso progresivo.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Doenças Musculoesqueléticas , Técnicas e Procedimentos Diagnósticos , Diagnóstico , Eletromiografia , Doenças Musculares , MiositeRESUMO
RESUMEN El síndrome de activación macrofágica (SAM) es una grave complicación de varias entidades reumáticas entre las que se encuentran la artritis idiopática juvenil sistémica, enfermedad de Still y lupus eritematoso sistémico. Este síndrome forma parte de las linfohistiocitosis hemofagocíticas adquiridas y constituye una enfermedad potencialmente mortal, con difi cultad en su identificación y carencia de consensos en cuanto a su manejo. Describimos una serie de casos de pacientes con SAM, exponiendo su proceso diagnóstico, su relación con las enfermedades reumáticas de base, su seguimiento y tratamiento, así como los resultados de diferentes esquemas de manejo.
ABSTRACT Macrophage activation syndrome (MAS) is a serious complication of several rheumatic disor ders, among which are the systemic juvenile idiopathic arthritis, Still's disease and systemic lupus erythematosus. This syndrome is part of the Acquired Haemophagocytic Lymphohistiocytoses, and is a potentially fatal disease, with difficulty in its identification and a lack of consensus regarding its management. A series of cases are describe of patients with macrophage activation syndrome, explaining their diagnostic process, their relationship with rheumatic diseases, their monitoring, and treatment, as well as the results of different management schemes.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Doenças da Pele e do Tecido Conjuntivo , Doenças Autoimunes , Síndrome de Ativação Macrofágica , Doenças do Sistema Imunitário , Lúpus Eritematoso Sistêmico , Transtornos LinfoproliferativosRESUMO
OBJETIVO: Generar las recomendaciones para la atención de pacientes con enfermedades reumáticas que reciben terapias inmunomoduladoras e inmunosupresoras (fármacos convencionales, biológicos y moléculas pequeñas) durante la pandemia por COVID-19. MATERIALES Y MÉTODOS: Las recomendaciones se realizaron utilizando el método Delphi como herramienta de acuerdo. Se conformó un panel de expertos con trayectoria académica y experiencia en investigación en reumatología. Se realizó la búsqueda de la literatura y se generó el cuestionario del ejercicio Delphi conformado por 42 preguntas. El grado de acuerdo se logró con el 80% de aprobación de los participantes. RESULTADOS: Se conformó un grupo de 11 reumatólogos de 7 ciudades del país. La tasa de respuesta fue del 100% para las 3 rondas de consulta. En la primera ronda se logró acuerdo en 35 preguntas, en la segunda ronda 37 y en la tercera ronda se logró el acuerdo de las 42 preguntas. CONCLUSIÓN: La recomendación para la mayoría de los tratamientos inmunomoduladores utilizados en reumatología es continuar con las terapias en pacientes que no tengan la infección y suspenderlas en aquellos con diagnóstico de SARS-CoV-2/COVID-19
OBJECTIVE: To produce recommendations for patients with rheumatological diseases receiving immunomodulatory and immunosuppressive therapies (conventional drugs, biologicals, and small molecules) during the COVID-19 pandemic. MATERIALS AND METHODS: The recommendations were determined using the Delphi method as an agreement tool. A panel of experts was formed, with academic backgrounds and research experience in rheumatology. A literature search was conducted and 42 questions were generated. The level of agreement was made with 80% of approval by the participants. RESULTS: A group of eleven rheumatologists from 7 cities in the country participated. The response rate was 100% for the three consultation rounds. In the first round, agreement was reached on 35 questions, on 37 in the second round, and on 42 questions in the third round. CONCLUSION: The recommendation for the majority of the pharmacological treatments used in rheumatology is to continue with immunomodulatory or immunosuppressive therapies in patients who do not have the infection, and to suspend it in patients with a diagnosis of SARS-CoV-2/COVID-19
Assuntos
Humanos , Doenças Reumáticas/tratamento farmacológico , Fatores Imunológicos/administração & dosagem , Antirreumáticos/administração & dosagem , Infecções por Coronavirus/tratamento farmacológico , Doenças Reumáticas/complicações , Padrões de Prática Médica , Suspensão de Tratamento/tendências , Infecções por Coronavirus/epidemiologia , Pandemias/estatística & dados numéricosRESUMO
La neumonía intersticial con características autoinmunes por sus siglas en inglés, es una entidad en la que existe un compromiso pulmonar intersticial y hallazgos clínicos y paraclínicos que sugieren una enfermedad del tejido conectivo, aunque no cumplen criterios diagnósticos para ninguna de estas. Con fines de investigación, en el 2015 se describieron criterios para esta entidad, en los que se incluyeron características de los dominios clínicos, serológicos y morfológicos, con diversos patrones de compromiso pulmonar. En la actualidad, hay un aumento en el interés de esta entidad, pues algunos autores sugieren que se pueda tratar de una enfermedad autoinmune per se, cuyo órgano blanco principal sería el pulmón. Dado su reciente reconocimiento, son pocos los casos descritos en la literatura. Con el propósito de contribuir a la mejor identificación de esa entidad, presentamos el caso de una paciente de 68 años con afectación pulmonar en quien después de descartar otras causas se llegó al diagnóstico de neumonía intersticial con características autoinmunes al cumplir los criterios de cada dominio requerido. Se inició tratamiento con micofenolato mofetilo a dosis de 2,5 mg/día. En su evolución clínica, la paciente presentó mejoría y fue dada de alta con tratamiento ambulatorio(AU)
Interstitial pneumonia with autoimmune features is a condition in which patients can have clinical and serological findings suggesting of a connective tissue disease associated with an interstitial lung disease, nonetheless no criteria for an specific connective tissue disease are meeting. In 2015 classification criteria where proposed, the diagnosis is made in the presence of a combination of features from clinical, serological and morphological domain with various patterns of pulmonary involvement. Currently there is an increase in the interest of this condition, as some authors suggest that it can be an autoimmune pathology per se, whose main target organ would be the lung. Given its recent recognition, there are few cases described in the literature and therefore in order to contribute to the better identification of that entity, we present the case of a 65 year old patient with lung involvement in whom after ruling out other etiological causes reached the diagnosis of I Interstitial pneumonia with autoimmune by meeting criteria of each required domain(AU)
Assuntos
Humanos , Feminino , Idoso , Pesquisa , Doenças Autoimunes/diagnóstico , Evolução Clínica , Doenças Pulmonares Intersticiais/complicações , Doenças do Tecido Conjuntivo Indiferenciado/diagnóstico , Ácido Micofenólico/uso terapêuticoRESUMO
RESUMEN Objetivo: Generar las recomendaciones para la atención de pacientes con enfermedades reumáticas que reciben terapias inmunomoduladoras e inmunosupresoras (fármacos convencionales, biológicos y moléculas pequeñas) durante la pandemia por COVID-19. Materiales y métodos: Las recomendaciones se realizaron utilizando el método Delphi como herramienta de acuerdo. Se conformó un panel de expertos con trayectoria académica y experiencia en investigación en reumatología. Se realizó la búsqueda de la literatura y se generó el cuestionario del ejercicio Delphi conformado por 42 preguntas. El grado de acuerdo se logró con el 80% de aprobación de los participantes. Resultados: Se conformó un grupo de 11 reumatólogos de 7 ciudades del país. La tasa de respuesta fue del 100% para las 3 rondas de consulta. En la primera ronda se logró acuerdo en 35 preguntas, en la segunda ronda 37 y en la tercera ronda se logró el acuerdo de las 42 preguntas. Conclusión: La recomendación para la mayoría de los tratamientos inmunomoduladores utilizados en reumatología es continuar con las terapias en pacientes que no tengan la infección y suspenderlas en aquellos con diagnóstico de SARS-CoV-2/COVID-19.
ABSTRACT Objective: To produce recommendations for patients with rheumatological diseases receiving immunomodulatory and immunosuppressive therapies (conventional drugs, biologicals, and small molecules) during the COVID-19 pandemic. Materials and methods: The recommendations were determined using the Delphi method as an agreement tool. A panel of experts was formed, with academic backgrounds and research experience in rheumatology. A literature search was conducted and 42 questions were generated. The level of agreement was made with 80% of approval by the participants. Results: A group of eleven rheumatologists from 7 cities in the country participated. The response rate was 100% for the three consultation rounds. In the first round, agreement was reached on 35 questions, on 37 in the second round, and on 42 questions in the third round. Conclusion: The recommendation for the majority of the pharmacological treatments used in rheumatology is to continue with immunomodulatory or immunosuppressive therapies in patients who do not have the infection, and to suspend it in patients with a diagnosis of SARS-CoV-2/COVID-19.
Assuntos
Humanos , Pacientes , Doenças Reumáticas , COVID-19 , Terapêutica , Técnica Delphi , AdultoRESUMO
La mastitis granulomatosa idiopática es una enfermedad inflamatoria crónica de causa desconocida y presentación infrecuente, que afecta a mujeres en edad reproductiva. Su presentación clínica similar al carcinoma de mama, sumada al desconocimiento de esta entidad, genera confusión, errores y retrasos en el diagnóstico en gran proporción de los casos, por lo que la histopatología es un requisito fundamental para el diagnóstico correcto de esta enfermedad. Presentamos dos casos de pacientes femeninas con cuadro clínico de mastitis crónica en quienes, tras descartar causas infecciosas y neoplásicas, se estableció el diagnóstico de mastitis granulomatosa idiopática. Se administró terapia inmunosupresora con prednisolona, metotrexate y ácido fólico. Las pacientes tuvieron una adecuada respuesta y se evidenció la resolución del cuadro clínico a las 3 semanas de tratamiento(AU)
Idiopathic granulomatous mastitis is a chronic inflammatory disease of unknown etiology and infrequent presentation. Confusion, delayed and mistaken in diagnosis has been atribuided to the similar clinical manifestation with breast carcinoma, added to the ignorance of this entity, that's why histopathology is a fundamental requirement for the correct diagnosis of this disease. We present two cases of female patients with clinical symptoms of chronic mastitis, in whom a diagnosis of idiopathic granulomatous mastitis was made after ruling out infectious and neoplastic causes(AU)
Assuntos
Humanos , Feminino , Adulto , Prednisolona/uso terapêutico , Mastite Granulomatosa/diagnóstico , Mastite Granulomatosa/tratamento farmacológico , Peru , Resultado do Tratamento , Imunossupressores/uso terapêuticoRESUMO
Resumen Introducción: diversos estudios han buscado factores asociados con el contagio de hepatitis C. La realización de tatuajes ha ganado importancia. Los estudios en los que se ha vinculado el riesgo de hepatitis C con tatuajes no han controlado las condiciones en que se realizó ni medidas de bioseguridad, por lo cual es difícil establecer una relación causal. Objetivo: Evaluar la seroprevalencia de hepatitis C en pacientes adultos con tatuajes realizados en los últimos dos años en el departamento de Risaralda, Colombia. Materiales y métodos: estudio de corte transversal en una muestra por conveniencia de 65 individuos, con uno o más tatuajes realizados entre 8 semanas a 2 años, sin otros factores de riesgo para hepatitis C, mediante una prueba rápida de anticuerpos por inmunocromatografía. Resultados: 57 de 86 sujetos cumplieron los criterios de selección. La prueba rápida de anticuerpos contra hepatitis C fue negativa en todos los participantes. La mayoría de los pacientes era de sexo femenino (59,6 %), con edad promedio de 25 años, 30 estudiantes universitarios, 46 provenientes de Pereira y 40 eran de estrato económico 3 o superior. 46 personas solo tuvieron una sesión de tatuaje. La mayoría tenía 2 a 3 tatuajes y 52 fueron realizados en establecimientos autorizados. 50 participantes refirieron que sus tatuadores cumplían las normas de bioseguridad. Conclusión: los tatuajes realizados bajo condiciones de bioseguridad en establecimientos autorizados y pocas sesiones parecen no aumentar el riesgo de infección por hepatitis C en personas sin otros factores de riesgo. Se requieren estudios adicionales para confirmar dicha hipótesis.
Abstract Introduction: In several studies of factors associated with the spread of hepatitis C, tattooing has gained importance. Studies that link tattooing with a risk of hepatitis C have not controlled for conditions under which it was done nor considered biosecurity measures. This makes it difficult to establish a causal relationship. Objective: This study assessed the seroprevalence of hepatitis C in adult patients who were tattooed within the last two years in the department of Risaralda, Colombia. Materials and Methods: This is a cross-sectional study of a convenience sample of 65 individuals with one or more tattoos that had been done 8 weeks to 2 years prior to the study and who had no other risk factors for hepatitis C. A rapid antibody immunochromatography test was used. Results: Out of 86 subjects, 57 met the selection criteria. The rapid hepatitis C antibody test was negative for all participants. Most were women (59.6%), their average age was 25 years, 30 were university students, 46 came from Pereira, and 40 were from economic stratum 3 or higher. Forty-six had had only one tattoo session. Most had two to three tattoos. Fifty-two had been done in authorized establishments. Fifty participants reported that their tattoo artists met biosafety standards. Conclusion: Tattoos made under biosafety conditions in authorized establishments do not seem to increase the risk of hepatitis C infections in people without other risk factors, especially when there have only been a few sessions. Additional studies are required to confirm this hypothesis.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pacientes , Tatuagem , Estudos Transversais , Hepatite C , Anticorpos , RiscoRESUMO
ABSTRACT Acute mesenteric ischemia is a medical emergency that accounts for less than 1/1000 hospital admissions. The disease affects adults older than 50 years predominantly with cardiac compromise, in whom the presence of acute abdominal pain is the cardinal manifestation, and should make the clinician suspect this entity. Its presentation in adolescents is unusual; therefore, in these cases, the possibility of an underlying thrombophilia should be part of the differential diagnosis. The case is presented here of a young female with a protein C and S deficiency as the cause of mesenteric thrombosis.
RESUMEN La isquemia mesentérica aguda es una urgencia médica que se presenta en menos de 1/1.000 ingresos hospitalarios. Es una entidad clínica infrecuente, predominante en adultos mayores de 50 arios con afectación cardíaca, en quienes la presencia de dolor abdominal agudo es la manifestación cardinal y debería hacer sospechar dicho diagnóstico. La presentación en adolescentes es inusual, por lo que, en estos casos, la posibilidad de una trombofilia subyacente debe formar parte del diagnóstico diferencial. Presentamos el caso de una paciente joven con deficiencia de proteínas C y S como agente causal de trombosis mesentérica.
Assuntos
Humanos , Feminino , Adolescente , Deficiência de Proteína , Trombose , Vasculite , Dor Abdominal , Emergências , Proteína C , Isquemia MesentéricaRESUMO
RESUMEN La enfermedad de Kawasaki forma parte de un grupo heterogéneo de afecciones de baja frecuencia que se caracterizan por la presencia de inflamación y necrosis de la pared vascular, que generan diversas manifestaciones clínicas y patológicas, las cuales comprometen medianos vasos, y que afectan principalmente a la población pediátrica. Presentamos el caso de un hombre de 36 arios de edad, sin antecedentes patológicos previos, con cuadro febril de 4 días de evolución asociado a cefalea, náuseas y odinofagia, con posterior aparición de múltiples lesiones maculopapulares generalizadas, las cuales se resolvieron con descamación en pulpejos, palmas y plantas. Se documenta conjuntivitis no supurativa bilateral, además de compromiso de la mucosa oral y los labios. La biopsia de piel reportó vasculitis linfocítica asociada a compromiso renal, hepático y cardiaco (miocardiopatía dilatada con fracción de eyección deprimida); se configuró el diagnóstico de enfermedad de Kawasaki completa del adulto con manifestaciones atípicas. Recibió tratamiento con metilprednisolona, ácido acetilsalicílico 100mg/día, una dosis única de inmunoglobulina IgG intravenosa 2 g/kg y terapia dialítica interdiaria, logrando una recuperación completa.
ABSTRACT Kawasaki disease is part of a heterogeneous group of low frequency diseases that are characterized by the presence of inflammation and necrosis of the vascular wall, generating various clinical and pathological manifestations, which compromise medium vessels, and mainly affecting the pediatric population. The case is presented of a 36-year-old man with no relevant past medical history, with a febrile episode of 4 days of onset, together with headache, nausea, and odynophagia with subsequent onset of multiple generalized maculopapular lesions. These resolved with subsequent desquamation of fingers, palms and soles of feet. Non-suppurative bilateral conjunctivitis is documented, as well as involvement of oral mucosa and lips. Skin biopsy reported lymphocytic vasculitis, associated with renal, hepatic and cardiac involvement (dilated cardiomyopathy with depressed ejection fraction). A diagnosis of complete adult Kawasaki disease with atypical manifestations was established. He was treated with methylprednisolone, acetylsalicylic acid 100 mg per day, and a single dose of intravenous IgG immunoglobulin 2 g/kg, and daily dialysis therapy to achieve complete recovery.
Assuntos
Humanos , Adulto , Síndrome de Linfonodos Mucocutâneos , Vasculite , Metilprednisolona , ExantemaRESUMO
ABSTRACT Pompe disease, or glycogen storage disease type II, is an autosomal recessive disorder due to the deficiency of lysosomal acid α-glucosidase, the enzyme responsible for degrading glycogen to glucose. The adult-onset form is rare and is characterized, primarily by accumulation of glycogen in striated, cardiac, and smooth muscle tissue. It causes muscle weakness of proximal predominance, so it can be confused with an inflammatory myopathy. The case is presented of a 60 year-old adult with a previous diagnosis of polymyositis in whom Pompe disease was confirmed with a demonstration of the enzymatic deficit in a biological substrate and a genetic identification was obtained.
RESUMEN La enfermedad de Pompe o glucogenosis tipo n es un trastorno autosómico recesivo, debido a la deficiencia de la enzima lisosomal α-glucosidasa ácida encargada de degradar glucógeno a glucosa. La forma de inicio en el adulto es rara y se caracteriza fundamentalmente por acumulación de glucógeno en tejido muscular estriado, cardiaco y liso. Causa debilidad muscular de predominio proximal, por lo que se puede confundir con una miopatía inflamatoria. Se presenta el caso de un adulto de 60 arios con diagnóstico previo de polimiositis, en quien se confirmó una enfermedad de Pompe con demostración del déficit enzimático en sustrato biológico y se logró realizar una identificación genética.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Doença de Depósito de Glicogênio Tipo II , Miosite , Debilidade Muscular , DiagnósticoRESUMO
La polisinovitis aguda edematosa benigna del anciano, también llamada síndrome RS3PE (acrónimo de remitting seronegative symmetrical synovitis with pitting edema), es una enfermedad reumatológica heterogénea que forma parte de las artritis inflamatorias y es de causa desconocida. Se presenta de forma exclusiva en la población de adultos mayores con afección poliarticular asociada a edema, tumefacción y limitación funcional que tiene por lo general buen pronóstico. Se describe el caso de un hombre de 70 años que cumple con seis de los siete criterios diagnósticos para esta enfermedad(AU)
Acute benign edematous polysynovitis in the elderly, also called RS3PE syndrome (acronym for remitting seronegative symmetrical synovitis with pitting edema), is a heterogeneous rheumatologic disease that is part of inflammatory arthritis and whose cause is unknown. It is presented exclusively in the population of aged adults with polyarticular affection associated with edema, swelling and functional limitation and has, in general, a good prognosis. We describe the case of a 70-year-old man who presents six of the seven diagnostic criteria for this disease(AU)
Assuntos
Humanos , Masculino , Idoso , Artrite , Neoplasias da Próstata , Prognóstico , Sinovite/diagnóstico , Saúde do IdosoRESUMO
Resumen La enfermedad de Kikuchi-Fujimoto es un trastorno benigno que afecta principalmente a mujeres jóvenes, se caracteriza por adenopatías de predominio en la región cervical, asociadas a fiebre y leucopenia. Aunque de etiología desconocida, hay evidencia de que una infección viral o una enfermedad autoinmune puede desencadenar la enfermedad. Se reporta un caso infrecuente en Colombia de enfermedad de Kikuchi-Fujimoto en una paciente con lupus eritematoso sistémico.
Abstract Kikuchi-Fujimoto disease is a benign disorder that mainly affects young women, and is characterised by predominantly cervical lymphadenopathy associated with fever and leukopenia. Although of unknown aetiology, there is evidence that a viral infection or autoimmune disease can trigger the disease. An uncommon case in Colombia is presented of Kikuchi-Fujimoto disease in a patient with Systemic Lupus Erythematosus.
Assuntos
Humanos , Feminino , Adulto , Linfadenite Histiocítica Necrosante , Linfadenopatia , Lúpus Eritematoso Sistêmico , Infecções , LinfomaRESUMO
La coleoterapia (consumo de larvas de Ulomoides Dermestoides), ha sido utilizado en medicina alternativa para tratar diferentes patologías crónicas como artritis, diabetes o cáncer. Su uso ha incrementado en Suramérica en los últimos años ganando aceptación. Presentamos el caso de una mujer de 64 años con purpura palpable en extremidades y fiebre que aparece durante el consumo de las larvas. Fue diagnosticado una vasculitis de hipersensibilidad la cual resolvió después de suspender el consumo de las larvas e iniciar corticosteroides. Esta es la primera asociación entre coleoterapia y purpura palpable que se ha descrito en la literatura destacando el potencial riesgo de eventos adversos generados por tratamientos de medicina alternativa
Ingestion of Ulomoides Dermestoides "beetle-peanut" has been used in alternative medicine to treat different chronic diseases such as arthritis, diabetes or cancer. Its use has increased in South America in recent years with acceptance. We present the case of a 64-year-old woman with palpable purpura in limbs and fever that appears during ingestion of the beetles. A hypersensitivity vasculitis was diagnosed which resolved after suspending ingestion of the beetles and initiating corticosteroids. The present case shows the potential risk of adverse events generated by alternative medicine treatments. This is the first association between Ingestion of Ulomoides dermestoides "beetle-peanut" and palpable purpura which has been described in the literature