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INTRODUCTION: Because of their rarity in men, systemic lupus erythematous and lupus nephritis (LN) are poorly understood in men. Our aim was to analyze the clinical presentation and course of histology-proven systemic lupus erythematous and LN in males and to determine the risk factors for progression to end-stage renal disease. METHODS: Fifty patients from 2 historical cohorts in Spain (Hospital 12 de Octubre) and Uruguay were retrospectively analyzed and compared with a female cohort matched for age and disease characteristics. RESULTS: The median age at the time of renal biopsy was 27 years (range, 8-79 years). The main forms of presentation were nephrotic syndrome in 26 of 50 patients (52%), and class IV LN in 34 of 50 (68%). After treatment, 21 patients (45.6%) achieved complete renal remission. During follow-up, 12 patients required renal replacement therapy, and 3 patients died of infectious causes. When patients who required renal replacement therapy were compared with those who did not require it, several parameters showed significant differences (P < 0.05) at the time of renal biopsy: estimated glomerular filtration rate < 60 ml/min, hypertension, hypoalbuminemia, and concomitant visceral involvement (neurologic, cardiovascular, and/or pulmonary). In the multivariate analysis, only estimated glomerular filtration rate < 60 ml/min persisted as a risk factor for progression to end-stage renal disease. When compared with a cohort of female patients with LN, there were no significant differences in remission or renal survival. DISCUSSION: LN in males usually presents as nephrotic syndrome, and type IV LN is the most frequent form. An estimated glomerular filtration rate < 60 ml/min at the time of renal biopsy is associated with poor renal outcomes. There were no differences in remission or progression of LN in males when compared with a cohort of female patients with LN.
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Resumen: Este documento de recomendaciones tiene como objetivo orientar a médicos nefrólogos y no nefrólogos que asisten a pacientes con enfermedad renal crónica (ERC) en todas las etapas de la misma, en el proceso de vacunación contra el SARS-CoV-2. Como consecuencia de la situación epidemiológica y de los tiempos del proceso de elaboración de las vacunas disponibles, no se ha generado evidencia lo suficientemente potente, por lo que las recomendaciones no se acompañan del nivel de evidencia. Se fundamenta la necesidad de priorizar la vacunación en este grupo de pacientes en el mayor riesgo de adquirir la infección por SARS-CoV-2, desarrollar la enfermedad COVID-19 con mayor gravedad y presentar una mortalidad más elevada que la población general. Las recomendaciones se organizan por grupos de pacientes considerando pacientes con ERC no dialítica, diálisis y trasplante renal, y pacientes bajo tratamiento inmunosupresor.
Summary The objective of this document containing recommendations is to provide guidelines for nephrologists and non-nephrologists who assist patients with chronic kidney disease (CKD) at all stages of the disease on the vaccination process against SARS-CoV-2. As a consequence of the current epidemiological situation and the timing of the COVID-19 vaccine development -for available vaccines- there is no solid evidence, and thus, recommendations are not accompanied by the due medical proof. The need to prioritize vaccination in this group of patients is based on the increased risk of acquiring the SARS-CoV-2 infection, developing the COVID-19 disease with greater severity and presenting higher mortality rates than the general population. The recommendations are organized by groups of patients, considering patients with non-dialytic CKD, dialysis and kidney transplantation, and patients under immunosuppressive treatment.
Resumo: O objetivo deste documento de recomendações é orientar os nefrologistas e não nefrologistas que atendem pacientes com doença renal crônica (DRC) em todas as fases da doença, no processo de vacinação contra a SARS-CoV-2. Como consequência da situação epidemiológica e do momento do processo de produção das vacinas disponíveis, não foram geradas evidências suficientemente potentes, de modo que as recomendações não são acompanhadas de seu nível de evidência. A necessidade de priorizar a vacinação neste grupo de pacientes baseia-se no maior risco de adquirir a infecção pelo SARS-CoV-2, desenvolver a doença COVID-19 com maior gravidade e apresentar mortalidade superior à da população em geral. As recomendações são organizadas por grupos de pacientes, considerando pacientes com DRC não dialítica, em diálise, com transplante renal, e pacientes em tratamento imunossupressor.
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Diálisis Renal , Trasplante de Riñón , Insuficiencia Renal Crónica , Vacunas contra la COVID-19RESUMEN
Clq nephropathy (Clq N) is an infrequent disease and only about 100 cases have been reported. It is defined by a pattern of immunofluorescence (IF) with dominant or co-dominant complement Clq with electron-dense deposits in the mesangium, without clinical or serological features of Lupus Nephritis. The most common histopathological findings of ClqN are focal segmental glomerulosclerosis and Minimal Change Disease. We report a 17 year-old male patient with an isolated selective proteinuria found in a routine study. He had normal renal function and urine culture was negative. Serum lipids, liver enzymes an complement were all normal. Serum antinuclear and anti-DNA antibodies, antineutrophil cytoplasmic antibodies (ANCA), HIV, Hepatitis B and C serology, were negative. Renal and abdominal ultrasonography was normal. The histopathological study revealed segmental glomerular sclerosis, moderate increase of mesangial matrix, Bowmann capsule adhesions and fucsinophil deposits in mesangium. The IF was positive (dominant) for Clq (+++) and IgA, IgG, IgM, C3++, all of them with a granular mesangial distribution. Ultrastructural findings were pedicelar effacement and paramesangial electron-dense deposits. Tubular reticular inclusions (TRI) were not found. Remission of proteinuria was reached after 18 months of treatment with enalapril and losartan. The patient remains with normal renal function. Clinical findings, negative serology for Lupus, light microscopy IF with dominant positivity for Clq, absence of TRI and paramesangial electron-dense deposits in electron microscopy lead us to the diagnosis of ClqN. A poor response to steroid therapy was described in ClqN. Thus it was worthwhile to differentiate it from lupus nephritis, that is responsive to steroids.
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Complemento C1q/análisis , Glomerulonefritis/patología , Riñón/patología , Adolescente , Biopsia , Diagnóstico Diferencial , Humanos , Nefritis Lúpica/patología , Masculino , Microscopía ElectrónicaRESUMEN
Clq nephropathy (Clq N) is an infrequent disease and only about 100 cases have been reponed. It is defined by a pattern of immunofluorescense (IF) with dominant or co-dominant complement Clq with electrondense deposits in the mesangium, without clinical or serological features of Lupus Nephritis. The most common histopathological findings of ClqN are focal segmental glomerulosclerosis and Minimal Change Disease. We repon a 17 year-old male patient with an isolated selective proteinuría found in a routine study. He had normal renal function and uriñe culture was negative. Serum lipids, liver enzymes an complement were all normal. Serum antinuclear and anti-DNA antibodies, antineutrophil cytoplasmic antibodies (ANCA), HIV, Hepatitis B and C serology, were negative. Renal and abdominal ultrasonography was normal. The histopathological study revealed segmental glomerular sderosis, modérate increase of mesangial matrix, Bowmann capsule adhesions and fucsinophil deposits in mesangium. The IF was positive (dominant) for Clq (+++) and IgA, IgG, IgM, C3++, all of them with a granular mesangial distribution. Ultrastructural findings were pedicelar effacement and paramesangial electrondense deposits. Tubular reticular inclusions (TRI) were not found. Remission of proteinuría was reached after 18 months of treatment with enalapril and losartan. The patient remains with normal renal function. Clinical findings, negative serology for Lupus, light microscopy IFwith dominant positivity for Clq, absence of TRI and paramesangial electrondense deposits in electron microscopy lead us to the diagnosis of ClqN. A poor response to steroid therapy was described in ClqN. Thus it was worthwhile to differentiate it from lupus nephritis, that is responsive to steroids.