IgG4-related kidney disease: experience from a Mexican cohort.

To evaluate the clinical/serological phenotype and outcomes of IgG4-related kidney disease. Case series of IgG4-related kidney disease from a cohort of 69 patients with IgG4-related disease. We defined kidney involvement as the presence of at least one of the following conditions: (A) laboratory parameters of kidney injury (proteinuria and/or elevated creatinine levels and/or hematuria); and/or (B) contrast-enhanced computed tomography features (multiple low-density lesions and/or nephromegaly and/or hypovascular solitary mass and/or renal pelvic lesion and/or perinephric lesions). We identified 17 patients with kidney involvement (24.6%), with a mean age of 53.6 ± 11.3 years; thirteen (76.5%) were male. Six patients fulfilled the laboratory criteria, six the imaging criteria, and five both. Five patients had a renal biopsy, the main histopathological diagnosis being IgG4 tubulointerstitial nephritis. Sixteen patients received glucocorticoids and 12 also immunosuppressors and/or biologics. Sixteen patients presented either total or partial renal remission at a median follow-up of 26 months, while one patient developed end-stage renal disease. Patients with kidney disease, as opposed to patients without kidney involvement, had a higher number of involved organs, higher IgG4-related disease responder index and IgG4 and IgG1 serum levels, higher prevalence of rheumatoid factor, and lower C3 and C4 levels. Our study emphasizes the systemic nature of IgG4-related disease, highlighting that renal involvement is usually present in a subset of patients with multisystemic disease, high IgG1 and IgG4 levels, and hypocomplementemia. Key Points • IgG4-RKD presents at a younger age in Mexican mestizo patients. • IgG4-RKD presents with proteinuria and kidney injury or as an asymptomatic imaging finding. • IgG4-RKD presents in the context of multisystemic disease, hypocomplementemia, and high IgG1 and IgG4 levels.

Calcineurin Inhibitors With Reduced-Dose Steroids as First-Line Therapy for Focal Segmental Glomerulosclerosis.

INTRODUCTION: High-dose corticosteroids remain the first-line therapy for focal and segmental glomerulosclerosis (FSGS), whereas calcineurin inhibitors (CNIs) are reserved for those patients resistant to corticosteroid therapy. METHODS: This is a retrospective cohort analysis in patients with primary FSGS diagnosed between 2007 and 2014. According to the administered treatment, patients were segregated into 3 groups: high-dose prednisone, first-line CNIs plus low-dose prednisone, and rescue CNIs. Cumulative corticosteroid doses were compared as well as response to therapy and long-term renal survival by Cox regression analysis. RESULTS: A total of 66 patients were included (39 treated with high-dose prednisone, 11 treated with first-line CNI, 16 treated with high-dose prednisone followed by rescue CNI). Cumulative doses of prednisone in the high-dose group were 9.3 g (interquartile range [IQR] = 7.5-12.5 g), compared to 2.5 g (IQR = 1.82-3.12 g) in the first-line CNI plus low-dose corticosteroid group and 13.8 g (IQR = 9.2-15.8 g) rescue CNI groups, respectively (P < 0.001). Time under corticosteroid management was also higher in the high-dose prednisone group compared to the first-line CNI group. There was a response to treatment in 76.9%, 72.7%, and 87.5% of high-dose prednisone, first-line CNI and rescue CNI groups, with complete remission in 48.7%, 36.4%, and 31.3% respectively. There was no difference in relapse incidence after treatment (48.4%, 44.4%, and 46.7%) or in 5-year renal survival (87.2%, 81.8%, and 87.5%). Baseline proteinuria, biopsy chronicity score, and response to therapy were independent predictors of renal survival. CONCLUSION: An initial CNI plus low-dose corticosteroid approach in primary FSGS reduces corticosteroid exposure with a response-to-therapy rate similar to that of the currently recommended high-dose corticosteroid regimen. These findings justify a randomized trial to formally test this hypothesis.

Ibero⁻American Consensus on Low- and No-Calorie Sweeteners: Safety, Nutritional Aspects and Benefits in Food and Beverages.

International scientific experts in food, nutrition, dietetics, endocrinology, physical activity, paediatrics, nursing, toxicology and public health met in Lisbon on 2⁻4 July 2017 to develop a Consensus on the use of low- and no-calorie sweeteners (LNCS) as substitutes for sugars and other caloric sweeteners. LNCS are food additives that are broadly used as sugar substitutes to sweeten foods and beverages with the addition of fewer or no calories. They are also used in medicines, health-care products, such as toothpaste, and food supplements. The goal of this Consensus was to provide a useful, evidence-based, point of reference to assist in efforts to reduce free sugars consumption in line with current international public health recommendations. Participating experts in the Lisbon Consensus analysed and evaluated the evidence in relation to the role of LNCS in food safety, their regulation and the nutritional and dietary aspects of their use in foods and beverages. The conclusions of this Consensus were: (1) LNCS are some of the most extensively evaluated dietary constituents, and their safety has been reviewed and confirmed by regulatory bodies globally including the World Health Organisation, the US Food and Drug Administration and the European Food Safety Authority; (2) Consumer education, which is based on the most robust scientific evidence and regulatory processes, on the use of products containing LNCS should be strengthened in a comprehensive and objective way; (3) The use of LNCS in weight reduction programmes that involve replacing caloric sweeteners with LNCS in the context of structured diet plans may favour sustainable weight reduction. Furthermore, their use in diabetes management programmes may contribute to a better glycaemic control in patients, albeit with modest results. LNCS also provide dental health benefits when used in place of free sugars; (4) It is proposed that foods and beverages with LNCS could be included in dietary guidelines as alternative options to products sweetened with free sugars; (5) Continued education of health professionals is required, since they are a key source of information on issues related to food and health for both the general population and patients. With this in mind, the publication of position statements and consensus documents in the academic literature are extremely desirable.

Glomerulonefritis rápidamente progresiva asociada con vasculitis por ANCA / Rapidly progressive glomerulonephritis associated with ANCA-vasculitis

Resumen El glomérulo renal, estructura altamente vascularizada, se ve afectado por un grupo de vasculitis de pequeño vaso asociadas con anticuerpos anticitoplasma de neutrófilo (ANCA), descritas por su relativa escasez de depósitos inmunes como pauciinmunes. Estas vasculitis se clasifican según el consenso internacional de Chapel-Hill en poliangeítis microscópica, granulomatosis con poliangeítis, granulomatosis eosinofílica y la llamada vasculitis limitada al riñón. Desde el punto de vista anatomopatológico, la glomerulonefritis rápidamente progresiva puede dividirse en pauciinmune, antimembrana basal glomerular (GBM GN) y mediada por inmunocomplejos. El tratamiento, en general, es con inmunosupresor y terapia renal de soporte desde medidas conservadoras hasta tratamiento de sustitución renal. Se comunica un caso de glomerulonefritis rápidamente progresiva asociada con ANCA por confirmación anatomopatológica.

Abstract The renal glomerulus highly vascularized structure is affected by a small vessel vasculitis group associated with neutrophil cytoplasmic antibodies (ANCA) described by its relative scarcity of pauci-immune deposits as immune. This vasculitis is classified according to international consensus in Chapel-Hill microscopic poliangetitis, granulomatosis with polyangiitis, eosinophilic granulomatosis and the called vasculitis limited to the kidney. From the anatomopathological point of view, the rapidly progressive glomerulonephritis can be divided into pauci-immune, anti glomerular basement membrane and mediated by immune complexes. The treatment is generally based on immunosuppressive therapy and renal therapy support from conservative measures to renal replacement therapy. This paper reports a case of rapidly progressive glomerulonephritis associated with ANCA pathological confirmation.

Hidratación: importancia en algunas condiciones patológicas en adultos / Hydration: Importance in some pathological conditions in adults

Resumen Estar bien hidratado se relaciona con un estado adecuado de salud y bienestar; sin embargo ¿qué pasa en los pacientes adultos que tienen algún padecimiento como obesidad, diabetes mellitus tipo 2, hipertensión arterial, cardiopatía isquémica e insuficiencia cardiaca, alteraciones nefrológicas (insuficiencia, poliquistosis y litiasis renal), enfermedad pulmonar obstructiva crónica, dislipidemia, hiperuricemia o, bien, en adultos mayores y en el periodo perioperatorio, en donde hay pérdida del estado de salud o una necesidad diferente de hidratación y que requieren consumir bebidas no alcohólicas para tener un buen estado de hidratación sin alterar la evolución natural de estas condiciones? Algunos puntos y recomendaciones son: la carbonatación de las bebidas ofrece el beneficio de aumentar la saciedad y disminuir la ingesta energética, lo que puede contribuir a la pérdida de peso; el agua simple es la mejor fuente de hidratación en los pacientes diabéticos, sin embargo, otras fuentes de hidratación pueden ser el agua mineralizada, el agua mineral, la leche (de preferencia descremada), café y té sin azúcar o con edulcorantes no calóricos o bajos en calorías, así como cualquier bebida que los contenga; en pacientes con litiasis renal se recomienda ingerir 2.5 a 4 L de agua al día; las bebidas para deportistas pueden ser consumidas por pacientes hipertensos, siempre y cuando no excedan la cantidad de sodio recomendada por la Organización Mundial de la Salud. En conclusión, la hidratación juega un papel importante en la evolución de las enfermedades mencionadas.

Abstract Being well hydrated is related to an adequate state of health and well-being; however, what happens in those adult patients having some pathological conditions such as obesity, type 2 diabetes mellitus, high blood pressure, ischemic heart disease and heart failure, kidney diseases (renal failure, polycystic renal disease and renal lithiasis), chronic obstructive pulmonary disease, dyslipidemia, hyperuricemia, or in the elderly and in the perioperative period, where there is loss of health or a different need for hydration and require the use of non-alcoholic beverages in order to have a good state of hydration without altering the natural evolution of these conditions? Some key points and recommendations are: carbonation of beverages offers the benefit of increasing satiety and decreasing energy intake, which can contribute to weight loss; simple water is the best source of hydration in diabetic patients; however, other sources of hydration may be mineralized water, mineral water, milk (preferably non-fat), coffee and tea without sugar or non-caloric sweeteners or low-calorie, as well as any beverage containing them; in patients with renal lithiasis it is recommended to take 2.5 to 4 L of water per day; sports drinks can be consumed by hypertensive patients as long as they do not exceed the amount of sodium recommended by the World Health Organization. In conclusion, hydration plays an important role in the evolution of the pathologic conditions mentioned above.