ABSTRACT
La enfermedad antimembrana basal glomerular (EMBG) es un trastorno autoinmune caracterizado por la presencia de anticuerpos antimembrana basal glomerular (AMBG), hemorragia pulmonar, glomerulonefritis necrotizante y depósito lineal de inmunoglobulinas en inmunofluorescencia directa. La predisposición genética, entre otros factores, posee un papel importante en el desarrollo de la enfermedad. Estudios previos han demostrado que el antígeno leucocitario humano (HLA), HLA-DR15 y HLA- DR4, se asocian con mayor riesgo de presentarla, mientras que el HLA-DR1 y HLA-DR7 han demostrado ser factor de protección frente a su desarrollo.Describimos el primer caso de dos hermanos no gemelos con EMBG con tipaje HLA idéntico, con factor de riesgo HLA-DR4 y factor de protección HLA-DR7. Planteamos la importancia de analizar el tipaje de histocompatibilidad en hermanos de pacientes con EMBG, para determinar el grado de susceptibilidad genética y plantear en ellos un seguimiento estrecho, con el objetivo de lograr un diagnóstico y tratamiento precoces en caso de presentar la enfermedad. (AU)
Anti-glomerular basement membrane disease (AGBM) is an autoinmune disorder characterized by the presence of anti-glomerular basement membrane (anti-GBM) antibodies, alveolar hemorrhage, necrotizing glomerulonephritis, and linear deposition of immunoglobulins through direct inmunofluorescence. Genetic predisposition, among other factors, plays an important role in the development of the disease. Previous studies have shown that HLA-DR15 and HLA-DR4 increase the risk of presenting it, while HLA-DR1 and HLA-DR7 protect against its development.We describe the first case of two non-twin siblings with AGBM and identical HLA, with HLA-DR4 as risk factor and HLA-DR7 as protection factor. We propose the importance of analyzing HLA in siblings of patients with AGBM, to determine the degree of genetic susceptibility and to carry out a close follow-up on them, with the aim of achieving an early diagnosis and treatment in case of presenting the disease. (AU)
Subject(s)
Humans , Male , Female , Young Adult , Anti-Glomerular Basement Membrane Disease , Genetic Predisposition to Disease , HLA-DR1 Antigen , HLA-DR4 Antigen , HLA-DR7 AntigenABSTRACT
Anti glomerular basement membrane disease (AGBM) is an autoinmune disorder characterised by the presence of anti-glomerular basement membrane (Anti-GBM) antibodies, alveolar hemorrhage, necrotizing glomerulonephritis, and linear deposition of immunoglobulins through direct inmunofluorescence. Genetic predisposition, among other factors, plays an important role in the development of the disease. Previous studies have shown that HLA-DR15 and HLA-DR4 increase the risk of presenting it, while HLA-DR1 and HLA-DR7 protect against its development. We describe the first case of two non-twin siblings with AGBM and identical HLA, with HLA-DR4 as risk factor and HLA-DR7 as protection factor. We propose the importance of analysing HLA in siblings of patients with AGBM, to determine the degree of genetic susceptibility and to carry out a close follow-up on them, with the aim of achieving an early diagnosis and treatment in case of presenting the disease.
Subject(s)
Anti-Glomerular Basement Membrane Disease , Glomerulonephritis , Humans , Anti-Glomerular Basement Membrane Disease/diagnosis , HLA-DR7 Antigen , HLA-DR4 Antigen , SiblingsABSTRACT
Anti-glomerular basement membrane disease (AGBM) is an autoinmune disorder characterized by the presence of anti-glomerular basement membrane (anti-GBM) antibodies, alveolar hemorrhage, necrotizing glomerulonephritis, and linear deposition of immunoglobulins through direct inmunofluorescence. Genetic predisposition, among other factors, plays an important role in the development of the disease. Previous studies have shown that HLA-DR15 and HLA-DR4 increase the risk of presenting it, while HLA-DR1 and HLA-DR7 protect against its development. We describe the first case of two non-twin siblings with AGBM and identical HLA, with HLA-DR4 as risk factor and HLA-DR7 as protection factor. We propose the importance of analyzing HLA in siblings of patients with AGBM, to determine the degree of genetic susceptibility and to carry out a close follow-up on them, with the aim of achieving an early diagnosis and treatment in case of presenting the disease.
ABSTRACT
INTRODUCTION: Chlorambucil is an alkylating agent used in combination with prednisolone for the treatment of idiopathic membranous nephropathy. Although chlorambucil is generally well-tolerated, it is a myelosuppresive drug that can cause several infections. CASE PRESENTATION: We report the case of an 81-year-old Caucasian male presenting with idiopathic membranous nephropathy who developed fever, cough, dyspnea, pulmonary infiltrates, and abdominal pain shortly after the initiation of treatment with chlorambucil and corticosteroids for nephropathy. Virology tests for infectious diseases revealed a recent cytomegalovirus infection. Antiviral treatment (ganciclovir) resulted in full remission. CONCLUSIONS: Cytomegalovirus infection should be considered in the differential diagnosis of respiratory symptoms and pulmonary infiltrates in patients treated with chlorambucil for nephrotic syndrome.
Subject(s)
Alkylating Agents/adverse effects , Chlorambucil/adverse effects , Cytomegalovirus Infections/chemically induced , Aged, 80 and over , Alkylating Agents/therapeutic use , Chlorambucil/therapeutic use , Cytomegalovirus Infections/diagnosis , Glomerulonephritis, Membranous/drug therapy , Humans , MaleABSTRACT
La espondilodiscitis es una infección del cuerpo vertebral y los discos adyacentes, cuya forma de diseminación más frecuente es la vía hematógena. Se trata de una entidad rara pero con una incidencia global en aumento, debido principalmente al incremento de bacteriemias en portadores de dispositivos intravasculares, como el utilizado en pacientes en tratamiento renal sustitutivo con hemodiálisis. Su diagnóstico es difícil por presentarse con síntomas inespecíficos, por lo que para un diagnóstico precoz y preciso es necesario el uso de la resonancia magnética. El tratamiento antibiótico empírico temprano y el abordaje multidisciplinario mejoran el pronóstico de esta infección potencialmente grave. Presentamos una serie de casos diagnosticados de espondilodiscitis en nuestra Sección de Nefrología (AU)
Spondylodiscitis is an infection of the vertebral body and adjacent intervertebral discs, in which hematogenous spread is by far the most common cause. This is a rare disease but its overall incidence is increasing, due mainly to increased bacteremia in patients with intravascular catheter as it is used in hemodialysis, being Staphylococcus aureus the most common organism. The diagnosis is difficult because of nonspecific symptoms. The use of magnetic resonance imaging allows an early and accurate diagnosis. The early empirical antibiotic treatment may improve the outcome of this potentially catastrophic infection, so it requires the collaboration of a multidisciplinary team of physicians to optimal therapy. We report a case series of spondylodiscitis diagnosed in our Nephrology Unit (AU)
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Aged, 80 and over , Discitis/diagnosis , Low Back Pain/etiology , Renal Dialysis/adverse effects , Catheter-Related Infections/complications , /adverse effects , Renal Insufficiency, Chronic/complications , Risk FactorsABSTRACT
Spondylodiscitis is an infection of the vertebral body and adjacent intervertebral discs, whose most common means of spreading is the haematogenous route. It is a rare disease but its overall incidence is increasing, mainly due to increased bacteraemia in patients with intravascular devices, such as that used in patients on renal replacement therapy with haemodialysis. Its diagnosis is difficult due to non-specific symptoms and as such, for early and accurate diagnosis, MRI is necessary. The early empirical antibiotic treatment and the multidisciplinary approach may improve the prognosis of this potentially serious infection. We report a series of cases diagnosed with spondylodiscitis in our Nephrology Unit.
