RESUMEN
Anti-glomerular basement membrane (GBM) disease is a rare and severe vasculitis that affects the glomerular and pulmonary capillaries and has an incidence of less than 2 cases per million individuals per year. Anti-GBM disease is mediated by autoantibodies against the α3 chain of type IV collagen. In the majority of cases, the autoantibodies are of the immunoglobulin G (IgG) class, with rare cases being mediated by immunoglobulin M (IgM) or immunoglobulin A (IgA); there are less than 15 IgA-mediated cases reported in the literature worldwide. The classic form of this disease manifests with rapidly progressive glomerulonephritis (RPGN), with or without pulmonary hemorrhage, and the diagnosis consists of identifying high titers of autoantibodies in the serum and/or deposited in the tissues. IgA antibodies are not identified in routine immunoassay tests, and renal biopsy with immunofluorescence is essential for diagnosis. We present a case of RPGN due to anti-GBM disease with linear IgA deposition, whose diagnosis was made exclusively by renal biopsy and with an unfavorable prognosis.
Asunto(s)
Enfermedad por Anticuerpos Antimembrana Basal Glomerular , Autoanticuerpos , Glomerulonefritis , Inmunoglobulina A , Humanos , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/inmunología , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/complicaciones , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico , Inmunoglobulina A/sangre , Inmunoglobulina A/inmunología , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Glomerulonefritis/inmunología , Glomerulonefritis/patología , Glomerulonefritis/diagnóstico , Biopsia , Masculino , FemeninoRESUMEN
Introducción: El síndrome de Goodpasture es una rara enfermedad autoinmune que forma parte del espectro de síndrome pulmón-riñón Objetivo: Presentar un paciente pediátrico con síndrome de Goodpasture atípico. Presentación del caso: Paciente de seis años seguida por el servicio de nefrología; ingresó a los cuatro años de edad por presentar hematuria macroscópica asociada a manifestaciones respiratorias y antecedente de títulos elevados de anticuerpos antimembrana basal glomerular. Fue motivo de investigación y se diagnosticó el síndrome de Goodpasture. Después de tratamiento inmunosupresor con ciclofosfamida y metilprednisolona seguido de prednisona oral, la paciente presentó descenso de los títulos de anticuerpos antimembrana basal glomerular, así como mejoría de los síntomas respiratorios, sin embargo, la proteinuria se mantuvo con incremento en los últimos meses. Para el diagnóstico se tuvieron en cuenta las manifestaciones clínicas en la niña, título elevado de anticuerpos antimembrana basal glomerular y la confirmación por biopsia renal de glomerulopatía. El tratamiento fue rápidamente efectivo con una disminución inmediata en los títulos de anticuerpos antimembrana basal y mejoría evidente de la condición clínica de la paciente. Se trata del primer caso pediátrico con síndrome de Goodpasture publicado en Cuba. Conclusiones: Por tratarse de una entidad rara en pediatría se requiere un diagnóstico temprano y tratamiento agresivo para mejorar el pronóstico del paciente. En su seguimiento son necesarias una terapia farmacológica prolongada, una adecuada adherencia al tratamiento propuesto y un estrecho monitoreo clínico y analítico de lo cual dependerá la progresión de la injuria renal y pulmonar(AU)
Introduction: Goodpasture´s syndrome is a rare autoimmune disease that is part of the lung-kidney syndrome's sprectrum. Objective: To present the case of a pediatric patient with an atypical Goodpasture´s syndrome. Case presentation: Six years old female patient under follow-up in the Nephrology service. She was admitted when she was four years old by presenting macroscopic hematuria, respiratory symptoms and a history of high titers of anti-glomerular basement membrane antibodies. She was under research and was diagnosed with the Goodpasture´s symdrome. After being under immunosupressive treatment with cyclophosphamide and methylprednisolone followed by oral prednisone, the patient presented a decrease in the titers of anti-glomerular basement membrane antibodies, and an improvement of the respiratory symptoms; however, proteinuria kept increasing in the last months. For the diagnosis, there were taken into account the clinical manifestations of the girl, the high titers of anti-glomerular basement membrane antibodies and the confirmations of glumerulopathy by renal biopsy. The treatment was effective quickly with an inmmediate decrease of the titers of anti-glomerular basement membrane antibodies and an evident improvement of the clinical condition of the patient. This is the first pediatric case presenting Goodpasture´s syndrome that has been published in Cuba. Conclusions: As this is a rare entity in Pediatrics, it is required an early diagnosis and an aggressive treatment to improve the patient's prognosis. In the follow-up are needed a prolonged pharmacological therapy, an adequate adherence to the proposed treatment and a close clinical and analytic monitoring from which will depend the progression of the lung and renal injury(AU)
Asunto(s)
Humanos , Femenino , Preescolar , Niño , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/epidemiologíaRESUMEN
Goodpasture Syndrome is described as a single episode disease entity. It is diagnosed with the demonstration of antiglomerular basement (anti-GBM) antibodies in plasma or renal tissue. Although the recurrence of anti-GBM disease is rare, it has been reported in up to 3% of cases. Recurrence with negative anti-GBM antibodies in plasma is even less frequent We report a 63 years old male in whom anti-GBM disease recurred without detectable anti-GBM antibodies in plasma, despite having positive antibodies at the onset.
Asunto(s)
Enfermedad por Anticuerpos Antimembrana Basal Glomerular/patología , Autoanticuerpos/análisis , Antibacterianos/uso terapéutico , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico por imagen , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/tratamiento farmacológico , Biopsia , Ciclofosfamida/uso terapéutico , Técnica del Anticuerpo Fluorescente , Humanos , Enfermedades Renales/patología , Glomérulos Renales/patología , Masculino , Persona de Mediana Edad , Prednisona/uso terapéutico , Recurrencia , Combinación Trimetoprim y Sulfametoxazol/uso terapéuticoRESUMEN
El síndrome pulmón-riñón es una entidad infrecuente, que comprende un gran espectro de patologías, como las vasculitis asociadas a ANCA y la enfermedad por anticuerpos antimembrana basal glomerular entre otras. Se describen en esta serie 12 casos donde las entidades más prevalentes fueron las antes mencionadas, observándose además un caso de lupus y uno de granulomatosis con poliangeítis, que se encuentran dentro de las causas menos frecuentes. La forma de presentación clínica inicial fue simultánea renal y pulmonar en 5/12 pacientes y renal en 7/12 de los mismos. El diagnóstico temprano de dichas patologías basándose en criterios clínicos, radiológicos, de laboratorio e histológicos, permite instaurar terapéuticas tempranas como la inmunosupresión y plasmaféresis, pudiendo prevenir complicaciones tales como las infecciones y la insuficiencia renal crónica terminal, siendo las primeras la principal causa de muerte (AU)
Pulmonary-renal syndrome is an infrequent condition. It includes a wide variety of conditions such as ANCA (antineutro-phil cytoplasmic autoantibody) associated with systemic vasculitis and anti-GBM (anti-glomerular basement membrane) disease among others. In this series we describe twelve cases, in which the most prevalent diseases were the above mentioned as well as one case of lupus and one of granulomatosis with polyangiitis (these being less frequent causes). The clinical presentation was both renal and pulmonary simultaneously in five of twelve patients and renal in seven of twelve patients. Early diagnosis of this condition on the basis of clinical, radiological, histological and analytic criteria allows early treatments such as immunosuppression and plasma exchange, thus avoiding complications such as infections (the main cause of death) and terminal chronic renal failure (AU)
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/terapia , Glomerulonefritis/diagnóstico , Glomerulonefritis/terapia , Granulomatosis con Poliangitis , Terapia de Inmunosupresión , Plasmaféresis , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico , Insuficiencia Renal Crónica , Lupus Eritematoso SistémicoRESUMEN
ABSTRACT Background and objectives: Anti-neutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis is a small vessel vasculitis with insufficient epidemiologic estimates in the United States. We aimed to determine demographic and clinical features of ANCA associated vasculitis patients presenting to a large tertiary care referral center in Upstate New York. Design, setting, participants, and measurements: A retrospective analysis of cases with pauci-immune GN on renal biopsy and clinical diagnosis of ANCA vasculitis presenting over 11 years was conducted. Outcomes of interest were: demographics, ANCA antibody positivity, patient and renal survival, and regional trends. Results: 986 biopsies were reviewed, 41 cases met the criteria for inclusion: 18 GPA, 19 MPA, and 4 double positive (anti-GBM disease plus ANCA vasculitis). Mean age at presentation was 52.4 years (SD 23.7), 23 (56%) were male and median creatinine was 2.6 mg/dL. The median patient follow up was 77 weeks (IQR 10 - 263 weeks), with a 3-month mortality rate of 5.7% and a 1-year estimated mortality rate of 12%. Thirteen patients required hemodialysis at the time of diagnosis; 7 patients came off dialysis, with median time to renal recovery of 4.86 weeks (IQR 1.57 - 23.85 weeks). C-ANCA positivity (p < 0.001) and C-ANCA plus PR3 antibody pairing (p = 0.005) was statistically significant in GPA versus MPA. P-ANCA positivity was observed in MPA versus GPA (p = 0.02) and double positive versus GPA (p = 0.002), with P-ANCA and MPO antibody pairing in MPA versus GPA (p = 0.044). Thirty-seven of the 41 cases were referred locally, 16 cases were from within a 15-mile radius of Albany, Schenectady, and Saratoga counties. Conclusions: ANCA vasculitis is associated with end stage renal disease and increased mortality. Our study suggests the possibility of higher regional incidence of pauci-immune GN in Upstate New York. Further studies should investigate the causes of clustering of cases to specific regions.
RESUMO Introdução e objetivos: A vasculite associada a anticorpos anticitoplasma de neutrófilo (ANCA) é uma vasculite de pequenos vasos com estimativas epidemiológicas insuficientes nos Estados Unidos. Nosso objetivo foi determinar características demográficas e clínicas de pacientes com vasculite associada à ANCA, apresentando-se a um grande centro de referência de atendimento terciário em Upstate New York. Formato, cenário, participantes e medidas: Foi realizada uma análise retrospectiva dos casos de GN pauci-imune em biópsias renais e diagnóstico clínico de vasculite ANCA por mais de 11 anos. Os resultados de interesse foram: dados demográficos, positividade de anticorpos ANCA, sobrevidas renal e de pacientes e tendências regionais. Resultados: 986 biópsias foram revisadas, 41 casos preencheram os critérios de inclusão: 18 GPA, 19 PAM, e 4 duplo-positivos (doença anti-MBG com vasculite ANCA). A média de idade na apresentação foi de 52,4 anos (DP 23,7), 23 (56%) eram do sexo masculino e mediana de creatinina de 2,6 mg/dL. O acompanhamento mediano dos pacientes foi de 77 semanas (IQR 10 - 263 semanas), com uma taxa de mortalidade de 3 meses de 5,7% e uma taxa de mortalidade estimada em 1 ano de 12%. Treze pacientes necessitaram de hemodiálise no momento do diagnóstico; 7 pacientes saíram da diálise, com tempo médio para recuperação renal de 4,86 semanas (IQR 1,57 - 23,85 semanas). A positividade para C-ANCA (p < 0,001) e o pareamento de anticorpos C-ANCA mais PR3 (p = 0,005) foram estatisticamente significantes em GPA versus PAM. A positividade de P-ANCA foi observada em PAM versus GPA (p = 0,02) e duplo positivo versus GPA (p = 0,002), com pareamento de anticorpos P-ANCA e MPO em PAM versus GPA (p = 0,044). Trinta e sete dos 41 casos foram encaminhados localmente, 16 casos foram de dentro de um raio de 15 milhas dos condados de Albany, Schenectady e Saratoga. Conclusões: A vasculite por ANCA está associada à doença renal terminal e aumento da mortalidade. Nosso estudo sugere a possibilidade de maior incidência regional de GN pauci-imune no norte do estado de Nova York. Novos estudos devem investigar as causas do acúmulo de casos em regiões específicas.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Atención Terciaria de Salud , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/epidemiología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/mortalidad , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/epidemiología , Fallo Renal Crónico/epidemiología , Biopsia , Comorbilidad , New York/epidemiología , Incidencia , Estudios Retrospectivos , Estudios de Seguimiento , Mortalidad/tendencias , Diálisis Renal , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/sangre , Creatinina/sangre , Estimación de Kaplan-Meier , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/sangre , Riñón/patología , Fallo Renal Crónico/sangreRESUMEN
Goodpasture Syndrome is described as a single episode disease entity. It is diagnosed with the demonstration of antiglomerular basement (anti-GBM) antibodies in plasma or renal tissue. Although the recurrence of anti-GBM disease is rare, it has been reported in up to 3% of cases. Recurrence with negative anti-GBM antibodies in plasma is even less frequent We report a 63 years old male in whom anti-GBM disease recurred without detectable anti-GBM antibodies in plasma, despite having positive antibodies at the onset.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Autoanticuerpos/análisis , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/patología , Recurrencia , Biopsia , Prednisona/uso terapéutico , Combinación Trimetoprim y Sulfametoxazol/uso terapéutico , Técnica del Anticuerpo Fluorescente , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/tratamiento farmacológico , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico por imagen , Ciclofosfamida/uso terapéutico , Enfermedades Renales/patología , Glomérulos Renales/patología , Antibacterianos/uso terapéuticoRESUMEN
BACKGROUND AND OBJECTIVES: Anti-neutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis is a small vessel vasculitis with insufficient epidemiologic estimates in the United States. We aimed to determine demographic and clinical features of ANCA associated vasculitis patients presenting to a large tertiary care referral center in Upstate New York. Design, setting, participants, and measurements: A retrospective analysis of cases with pauci-immune GN on renal biopsy and clinical diagnosis of ANCA vasculitis presenting over 11 years was conducted. Outcomes of interest were: demographics, ANCA antibody positivity, patient and renal survival, and regional trends. RESULTS: 986 biopsies were reviewed, 41 cases met the criteria for inclusion: 18 GPA, 19 MPA, and 4 double positive (anti-GBM disease plus ANCA vasculitis). Mean age at presentation was 52.4 years (SD 23.7), 23 (56%) were male and median creatinine was 2.6 mg/dL. The median patient follow up was 77 weeks (IQR 10 - 263 weeks), with a 3-month mortality rate of 5.7% and a 1-year estimated mortality rate of 12%. Thirteen patients required hemodialysis at the time of diagnosis; 7 patients came off dialysis, with median time to renal recovery of 4.86 weeks (IQR 1.57 - 23.85 weeks). C-ANCA positivity (p < 0.001) and C-ANCA plus PR3 antibody pairing (p = 0.005) was statistically significant in GPA versus MPA. P-ANCA positivity was observed in MPA versus GPA (p = 0.02) and double positive versus GPA (p = 0.002), with P-ANCA and MPO antibody pairing in MPA versus GPA (p = 0.044). Thirty-seven of the 41 cases were referred locally, 16 cases were from within a 15-mile radius of Albany, Schenectady, and Saratoga counties. CONCLUSIONS: ANCA vasculitis is associated with end stage renal disease and increased mortality. Our study suggests the possibility of higher regional incidence of pauci-immune GN in Upstate New York. Further studies should investigate the causes of clustering of cases to specific regions.
Asunto(s)
Enfermedad por Anticuerpos Antimembrana Basal Glomerular/epidemiología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/epidemiología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/mortalidad , Fallo Renal Crónico/epidemiología , Atención Terciaria de Salud , Adulto , Anciano , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/sangre , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/sangre , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Biopsia , Comorbilidad , Creatinina/sangre , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Estimación de Kaplan-Meier , Riñón/patología , Fallo Renal Crónico/sangre , Masculino , Persona de Mediana Edad , Mortalidad/tendencias , New York/epidemiología , Diálisis Renal , Estudios RetrospectivosRESUMEN
Basement membranes form an anatomic barrier that contains connective tissue. They are composed of type IV collagen, laminin and proteoglycans. Anti-basement membrane antibodies bind to the non-collagen site of the α3 chain of type IV collagen. A group of renal diseases, pulmonary diseases and perhaps others affecting different organs have long been associated with the presence of antibodies directed against glomerular basement membrane (GBM), alveolar basement membrane and tubular basement membrane. Goodpasture disease has a frequency of 0.5 to 1 case by million/year, and is responsible for up to 20% of crescentic glomerulonephritis in renal biopsy. It has been associated with genetic and immune abnormalities and there are usually environmental triggers preceding clinical onset. Renal disease can occur isolated or in association with pulmonary hemorrhage. In general, renal disease has a rapid progression that determines severe compromise, with rare spontaneous resolution. The diagnosis of Goodpasture disease requires the presence of the anti-GBM antibody, either in circulation or in renal tissue. The prognosis of non-treated patients is poor. The standard of care is plasma exchange combined with prednisone and cyclophosphamide. Anti-GBM antibody levels must be monitored frequently until their disappearance, and then every 6 months to confirm sustained remission in the absence of clinical signs of recurrence. Prognosis of the disease is strongly associated with its initial presentation. Survival rates are related to the degree of renal compromise at onset of the disease. Recurrence of the disease post-transplantation is low.
Asunto(s)
Enfermedad por Anticuerpos Antimembrana Basal Glomerular , Autoanticuerpos/sangre , Membrana Basal Glomerular , Inmunosupresores/uso terapéutico , Intercambio Plasmático/métodos , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/inmunología , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/fisiopatología , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/terapia , Ciclofosfamida/uso terapéutico , Progresión de la Enfermedad , Membrana Basal Glomerular/inmunología , Membrana Basal Glomerular/patología , Humanos , Pruebas de Función Renal , Monitorización Inmunológica/métodos , Prednisona/uso terapéutico , PronósticoRESUMEN
La granulomatosis con poliangitis como causa del síndrome pulmón-riñón es infrecuente en niños. Presentamos el caso de un paciente de 13 años con hemorragia alveolar, glomerulonefritis rápidamente progresiva, escleritis y anticuerpos anti-PR3 (proteinasa 3). Hacemos referencia a las características de esta vasculitis asociada a anticuerpos contra el citoplasma de los neutrófilos (ANCA) en los niños y a su enfoque terapéutico.
Granulomatosis with polyangiitis as a cause of kidney-lung syndrome is uncommon in children. We report the case of a 13 year old patient with alveolar hemorrhage, rapidly progressive glomerulonephritis, episcleritis and anti PR3. We refer to features of the ANCA-associated vasculitis in children and to its therapeutic approach.
Asunto(s)
Masculino , Adolescente , Granulomatosis con Poliangitis , Enfermedad por Anticuerpos Antimembrana Basal Glomerular , PediatríaRESUMEN
We describe a female patient with Alport disease who developed antiglomerular basement membrane nephritis late after kidney transplantation during the treatment of an acute bacterial pyelonephritis and discuss the potential role of the infection as a trigger for the development of this nephritis.
Asunto(s)
Enfermedad por Anticuerpos Antimembrana Basal Glomerular/etiología , Infecciones por Escherichia coli/microbiología , Fallo Renal Crónico/cirugía , Trasplante de Riñón/efectos adversos , Nefritis Hereditaria/complicaciones , Pielonefritis/microbiología , Adolescente , Antibacterianos/uso terapéutico , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/inmunología , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/terapia , Autoanticuerpos/sangre , Membrana Basal/inmunología , Ceftriaxona/uso terapéutico , Sustitución de Medicamentos , Infecciones por Escherichia coli/tratamiento farmacológico , Femenino , Humanos , Inmunosupresores/administración & dosificación , Fallo Renal Crónico/etiología , Glomérulos Renales/inmunología , Plasmaféresis , Quimioterapia por Pulso , Pielonefritis/tratamiento farmacológico , Resultado del TratamientoRESUMEN
CONTEXT: anti-glomerular basement membrane (anti-GBM) antibody syndrome is characterized by deposition of anti-GBM antibodies on affected tissues, associated with glomerulonephritis and/or pulmonary involvement. This syndrome has been described in association with other autoimmune disorders, but as far as we know, it has not been described in association with dermatomyositis and psoriasis. CASE REPORT: a 51-year-old man with a history of dermatomyositis and vulgar psoriasis presented with a condition of sensitive-motor polyneuropathy of the hands and feet, weight loss of 4 kg, malaise and fever. On admission, he had been making chronic use of cyclosporin and antihypertensive drugs for three months because of mild arterial hypertension. Laboratory tests showed anemia and leukocytosis, elevated serum urea and creatinine and urine presenting proteinuria, hematuria, leukocyturia and granular casts. The 24-hour proteinuria was 2.3 g. Renal biopsy showed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG) deposits on the glomerular basement membrane by means of direct immunofluorescence, which were suggestive of anti-GBM antibodies. The patient was then treated initially with methylprednisolone and with monthly cyclophosphamide in the form of pulse therapy.
Asunto(s)
Enfermedad por Anticuerpos Antimembrana Basal Glomerular/complicaciones , Psoriasis/complicaciones , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Dermatomiositis/complicaciones , Dermatomiositis/patología , Humanos , Riñón/patología , Masculino , Persona de Mediana EdadRESUMEN
CONTEXT: Anti-glomerular basement membrane (anti-GBM) antibody syndrome is characterized by deposition of anti-GBM antibodies on affected tissues, associated with glomerulonephritis and/or pulmonary involvement. This syndrome has been described in association with other autoimmune disorders, but as far as we know, it has not been described in association with dermatomyositis and psoriasis. CASE REPORT: A 51-year-old man with a history of dermatomyositis and vulgar psoriasis presented with a condition of sensitive-motor polyneuropathy of the hands and feet, weight loss of 4 kg, malaise and fever. On admission, he had been making chronic use of cyclosporin and antihypertensive drugs for three months because of mild arterial hypertension. Laboratory tests showed anemia and leukocytosis, elevated serum urea and creatinine and urine presenting proteinuria, hematuria, leukocyturia and granular casts. The 24-hour proteinuria was 2.3 g. Renal biopsy showed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG) deposits on the glomerular basement membrane by means of direct immunofluorescence, which were suggestive of anti-GBM antibodies. The patient was then treated initially with methylprednisolone and with monthly cyclophosphamide in the form of pulse therapy.
CONTEXTO: A síndrome do anticorpo anti-membrana basal glomerular (anti-MBG) é caracterizada pela deposição de anticorpos anti-MBG em tecidos afetados, associada à glomerulonefrite e/ou ao envolvimento pulmonar. Essa síndrome já foi descrita em associação a outras doenças autoimunes, mas até onde conhecemos, não há relatos de sua associação com dermatomiosite e psoríase. RELATO DE CASO: Um homem de 51 anos com antecedentes de dermatomiosite e psoríase vulgar apresentou quadro de polineuropatia sensitivo-motora de mãos e pés, perda de 4 kg, adinamia e febre. À admissão estava em uso crônico de ciclosporina e de anti-hipertensivos há três meses devido a hipertensão arterial leve. Exames laboratoriais mostraram anemia e leucocitose, creatinina e ureia séricas elevadas e urina com proteinúria, hematúria, leucocitúria e cilindros granulosos. A proteinúria de 24 horas foi de 2,3 g. A biópsia renal revelou uma glomerulonefrite crescêntica necrotizante com depósitos lineares de imunoglobulina G (IgG) na MBG à imunofluorescência, sugestivos de anticorpos anti-MBG. O paciente foi então tratado inicialmente com metilprednisolona e com ciclofosfamida mensalmente na forma de pulsoterapia.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/complicaciones , Psoriasis/complicaciones , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Dermatomiositis/complicaciones , Dermatomiositis/patología , Riñón/patologíaRESUMEN
La enfermedad antimembrana basal glomerular (anti-MBG) es una condición que se manifiesta clínicamente como glomerulonefritis rápidamente progresiva y hemorragia alveolar, también llamada Síndrome Riñón- Pulmón. Se asocia a la presencia de autoanticuerpos dirigidos contra el colágeno tipo IV de la membrana basal glomerular. Las vasculitis sistémicas asociadas a ANCA también pueden manifestarse como Síndrome Riñón-Pulmón, cuadro clínico a veces indistinguible de la enfermedad anti-MBG. La concomitancia de ANCA y anticuerpos anti-MBG en el Síndrome Riñón-Pulmón es del orden de un 30 por ciento, según distintos reportes de la literatura. El perfil clínico, el pronóstico y el rol fisiopatológico de cada anticuerpo en este grupo de pacientes todavía son materia de investigación. El mecanismo patogénico inicial parece ser el daño mediado por ANCA, que puede inducir la aparición de anticuerpos anti-MBG, los que perpetúan el daño en el glomérulo.
Anti-glomerular basement membrane (anti-MBG) disease is a condition that is manifested clinically as rapidly progressive glomerulonephritis and alveolar hemorrhage, also known as Pulmonary-Renal Syndrome. It is associated with the presence of autoantibodies directed against type IV collagen of the glomerular basement membrane. Systemic vasculitis associated with ANCA may also manifest as Pulmonary-Renal Syndrome, sometimes clinically indistinguishable from the anti-MBG disease.The concomitance of ANCA and anti-MBG antibodies in the Pulmonary-Renal Syndrome is about 30 percent, according to various reports in literature. The clinical profile, prognosis and physiopathologic roles of each antibody in this group of patients is still under investigation. The pathogenic mechanism appears to be the initial damage mediated by ANCA, which may induce the appearance of anti-MBG, those who perpetuate the glomerulus damage.
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/complicaciones , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/inmunología , Enfermedades Pulmonares/complicaciones , Enfermedades Pulmonares/inmunología , Enfermedades Renales/complicaciones , Enfermedades Renales/inmunología , Anticuerpos Anticitoplasma de Neutrófilos , Glomérulos Renales/inmunología , Glomérulos Renales/patología , Pulmón/inmunología , Pulmón/patología , SíndromeRESUMEN
Objetivos: determinar las glomerulopatías más frecuentes y su curso clínico en mujeres gestantes. Hacer una revisión de la literatura del tema. Materiales y métodos: serie de casos retrospectivos de pacientes gestantes con enfermedad glomerular activa diagnosticada por biopsia, entre enero de 2000 y septiembre de 2007, atendidas en el Hospital San Vicente de Paúl de Medellín, Colombia. No hubo exclusiones. Se determinaron sus características clínicas, la evolución de la enfermedad glomerular y el resultado materno perinatal. Resultados: se hizo el diagnóstico en 11 pacientes. Cuatro casos de nefritis lúpica, dos de glomerulonefritis rápidamente progresiva (GNRP) por GN extracapilar y granulomatosis de Wegener, respectivamente; tres con síndrome nefrótico por glomeruloesclerosis focal y segmentaria (GEFyS), una con GN membrana proliferativa tipo I, y una con GN proliferativa mediada por complejos inmunes. En dos casos se presentó eclampsia. En tres casos hubo muerte materna: dos por eclampsia y una por granulomatosis de Wegener; y en tres casos muerte fetal: dos por eclampsia y una en materna con nefritis lúpica y síndrome antifosfolípido. Las glomerulopatías primarias no mostraron empeoramiento durante la gestación. Conclusiones: en casos de GN durante la gestación hay riesgo incrementado de complicaciones materno-fetales.
Asunto(s)
Adulto , Humanos , Femenino , Embarazo , Enfermedad por Anticuerpos Antimembrana Basal Glomerular , Biopsia , EmbarazoRESUMEN
Goodpasture s syndrome (GS) is an auto-immune disease that is part of the pulmonary-renal syndrome spectrum. It is characterized by the linear deposition of anti-glomerular basement membrane antibodies (anti-GBM) in glomerular and alveolar basement membrane resulting in alveolar hemorrhage and progressive glomerulonephritis. An early diagnosis is important to decrease clinical morbidity. In the present work we illustrate a GS case initially diagnosed as Wegener s granulomatosis. The patient showed favorable clinical evolution with corticosteroid therapy associated with plasmapheresis and cyclophosphamide.