Hematoma intraparenquimatoso asociado a vasculitis ANCA positiva / Intraparenchymal haematoma associated with an ANCA-positive vasculitis

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Patogênese e tratamento da glomerulonefrite - uma atualização / Pathogenesis and treatment of glomerulonephritis-an update

Resumo A presente revisão traz os conceitos mais atuais acerca dos fatores de risco genéticos, eventos etiológicos, respostas nefritogênicas e tratamento dos principais tipos de glomerulonefrite (GN) imunomediada. Tais patologias incluem GN pós-infecciosa, nefropatia por IgA, doença por anticorpo antimembrana basal glomerular (anti-MBG), vasculite associada a ANCA (VAA) e nefrite lúpica. Apesar da(s) etiologia(s) da maioria dos casos de GN permanecer indefinida, acredita-se que seu início se deva, em grande parte, a insultos ambientais, particularmente na forma de processos infecciosos que deflagram respostas de hospedeiro em indivíduos geneticamente suscetíveis, levando assim a quadros de GN. A concepção mecanicista em torno dessas patologias evoluiu a partir da visão mais antiga de que a maioria seria consequência do aprisionamento glomerular de complexos imunes pré-formados para a percepção atual de que as mesmas, em sua maioria, são doenças autoimunes por natureza mediadas por anticorpos e linfócitos T reativos a auto-antígenos. O tratamento da GN não tem acompanhado os progressos na compreensão de sua patogênese. Os papéis recentemente atribuídos a mediadores mais antigos como complemento e proteínas reguladoras do complemento lançam luz sobre novos alvos terapêuticos.

Abstract This review updates current concepts of the genetic risk factors, etiologic events, nephtitogenic responses and treatment of the major immunologically mediated types of glomerulonephritis (GN). These include post-infectious GN, IgA nephropathy, anti-glomerular basement membrane (GBM) antibody disease, ANCA-associated vasculitis (AAV) and lupus nephritis. Although the etiology(s) of most GNs remain undefined, many are now believed to be initiated by environmental insults, particularly infectious processes, that trigger host responses in genetically susceptible individuals which lead to GN. Mechanistic concepts of these diseases have evolved from earlier views that most were consequent to glomerular trapping of preformed immune complexes to the current view that most of these diseases are auto-immune in nature mediated by both antibodies and T cells reactive with self-antigens. Therapy of GN has lagged behind advances in understanding pathogenesis. Newly appreciated roles for older mediators like complement and complement regulatory proteins offer new therapeutic targets.

Paquimeningitis hipertrófica relacionada con IgG4 y MPO-ANCA / IgG4- and MPO-ANCA-associated hypertrophic pachymeningitis

Introducción. La paquimeningitis hipertrófica idiopática es una enfermedad fibroinflamatoria de la duramadre. Su diagnóstico requiere la exclusión de enfermedades infecciosas, tumorales y otras enfermedades inflamatorias. En los últimos años se han descrito nuevas entidades que pueden presentarse con paquimeningitis hipertrófica: la enfermedad relacionada con IgG4 y la paquimeningitis MPO-ANCA+ como forma de vasculitis limitada al sistema nervioso central. Caso clínico. Varón de 64 años con cefalea y cervicalgia de predominio nocturno y clínica de compresión medular. Tras el diagnóstico de paquimeningitis hipertrófica craneocervical facilitado por el estudio de resonancia magnética, se realizó un estudio etiológico. Se descartaron enfermedades infecciosas y tumorales. La clínica no mostraba afectación sistémica y en la analítica presentaba IgG4 elevada y MPO-ANCA+. Tras tratamiento con corticoides presentó una rápida mejoría de la clínica. Conclusiones. La enfermedad relacionada con IgG4 y la vasculitis asociada a MPO-ANCA limitada al sistema nervioso central pueden representar un alto porcentaje de las paquimeningitis hipertróficas que se consideraban idiopáticas, y su diagnóstico requiere biopsia y estudio histológico (AU)

Introduction. Idiopathic hypertrophic pachymeningitis is a fibroinflammatory immune-mediated disease of the dura mater. Its diagnosis requires the preclusion of infectious, tumoral and other inflammatory diseases. In recent years new entities have been reported that can present with hypertrophic pachymeningitis, such as IgG4-associated disease and MPO-ANCA+ pachymeningitis, as a form of vasculitis limited to the central nervous system. Case report. We describe the case of a 64 years-old male with headaches and cervicalgia, predominantly at night, and clinical signs and symptoms of spinal cord compression. Following the diagnosis of craniocervical hypertrophic pachymeningitis provided by the magnetic resonance imaging study, an aetiological study was conducted. Infectious and tumoral diseases were precluded. The clinical features did not show any systemic involvement and high levels of IgG4 and MPO-ANCA+ were found in the results of the analyses. The clinical signs and symptoms quickly improved following treatment with corticoids. Conclusions. IgG4-related disease and MPO-ANCA-associated vasculitis limited to the central nervous system can account for a high percentage of the cases of hypertrophic pachymeningitis that were considered idiopathic, and their diagnosis requires a biopsy and a histological study (AU)

Necrotizing crescentic glomerulonephritis in a patient with positive serologies for lupus and antineutrophil cytoplasmic antibodies

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Granulomatosis de Wegener con anticuerpos anticitoplasma de neutrófilo frente a catepsina G / Wegener's granulomatosis with anti-neutrophil cytoplasmic antibodies against anti-cathepsin G antigen

La granulomatosis de Wegener pertenece al grupo de vasculitis de vaso pequeño asociadas a anticuerpos anticitoplasma de neutrófilo. Se caracteriza por inflamación granulomatosa y vasculitis necrosante que puede afectar a diversos órganos, principalmente cursa con afectación renal y de las vías respiratorias. Es una enfermedad rara en la infancia y el diagnóstico precoz es fundamental para el pronóstico a largo plazo de la enfermedad. La presencia de anticuerpos anticitoplasma de neutrófilo con patrón citoplasmático o de títulos elevados de anticuerpos antiproteinasa-3 ha sido actualmente incluida en los criterios diagnósticos de vasculitis en niños. En este artículo se presenta un caso de granulomatosis de Wegener en la infancia, con presencia de anticuerpos anticitoplasma de neutrófilo con patrón citoplasmático con ausencia de anticuerpos antiproteinasa-3 y presencia de niveles elevados de anticuerpos anticatepsina G, raramente asociados a granulomatosis de Wegener (AU)

Wegener’s granulomatosis belongs to the group of small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies characterized by granulomatous inflammation andnecrotising vasculitis in various organs with particular involvement of the upper and lower respiratory tracts and kidneys. Wegener’s granulomatosis is a rare disorder in childhood and early diagnosis of this disease is critical to the long-term prognosis of the disease. The presence of positive cytoplasmic antineutrophil cytoplasmic antibody staining or a high titre of proteinase 3 antibodies were added as new criteria of vasculitis in childhood. This article presents a case of Wegener’s granulomatosis, with the presence of anti-neutrophil cytoplasm antibodies with cytoplasmic pattern with absence of anti-proteinase 3 antibodies and presence of high levels of anti-cathepsin G antibodies, rarely described in Wegener’s granulomatosis (AU)

Vasculitis ANCA positivo en un paciente con esclerosis sistémica / No disponible

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Síndrome de Sjögren y vasculitis asociada a anticuerpos anticitoplasma de neutrófilo / Sjögren's syndrome and vasculitis associated with antineutrophil cytoplasmic antibodies

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Anticuerpos frente al citoplasma de los neutrófilos: más allá de las vasculitis / Anti-neutrophil cytoplasmic antibodies: beyond vasculitis

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Glomerulonefritis extracapilar tipo I con coexistencia de anticuerpos anti-MBG y ANCA-p positivos / No disponible

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