Membranoproliferative Glomerulonephritis and Mixed Cryoglobulinemia as a Form of Presentation of Visceral Leishmaniasis.

BACKGROUND Visceral leishmaniasis (VL) is an endemic systemic disease in the Mediterranean countries, including Spain. This vector-borne infection can present with several clinical presentations, from asymptomatic to severe forms. Renal impairment is frequently described in VL but is usually mild and related to interstitial nephritis, being that glomerular involvement is rarely found. CASE REPORT We describe a case of a 69-year-old Spanish male presenting with subacute renal failure due to membranoproliferative glomerulonephritis and mixed cryoglobulinemia accompanied by other autoimmune features (hypocomplementemia, antinuclear and antiDNA antibodies). No hepatosplenomegaly was found with abdominal ultrasound. Hepatotropic viruses and human immunodeficiency virus serological markers were negatives. We initially suspect the presence of an autoimmune disease and the patient was treated with steroids without improvement. After an extensive study including renal and bone marrow biopsy, a correct diagnosis of visceral leishmaniasis was made, and treatment with liposomal amphotericin B was initiated, achieving renal function recovery and normalization of immunological manifestations. CONCLUSIONS Renal involvement can be an important feature of VL and it might be associated with increased morbidity and mortality. The association between mixed cryoglobulinemia and renal involvement in VL have rarely been described. VL is frequently associated with diverse autoimmune manifestations and it can be initially misdiagnosed, which could lead to fatal consequences. The role of the immune system in the formation of cryoglobulins are discussed. In our case, an autoimmune disease was initially suspected, and starting treatment with steroids pulses was initiated. However, the presence of mixed cryoglobulinemia in this patient who was hepatitis C serological marker negative and who had poor renal function recovery after immunosuppressive treatment made us suspect other pathologies. The presence of cryoglobulinemia with renal disease in endemic areas of Leishmania should make us exclude this infection before starting immunosuppressive treatment.

IgG4-related disease and idiopathic membranous nephropathy: "The clothes do not make the man"
.

This case report describes a patient with a previous history of autoimmune pancreatitis secondary to IgG4-related disease, who developed an overt nephrotic syndrome due to membranous nephropathy, surprisingly idiopathic. In all the previously described cases with both concurrent diseases, membranous nephropathy was considered to be secondary to the IgG4-related disease based on the absence of anti-PLA2R1 autoantibodies, and nephrotic syndrome usually remitted after treatment with steroids alone. However, in our patient positivity of serum anti-PLA2R1 autoantibodies together with a normal serum IgG4 level, and the absence of the other most commonly associated diseases were compatible with an idiopathic membranous nephropathy. Combination treatment with steroids and cyclophosphamide was successful. We hypothesize about causality or coincidental diseases and the importance of a correct classification.
.

Estudio evolutivo de las glomerulonefritis en Castilla-La Mancha (GLOMANCHA) en el periodo 1994-2008 / Study of glomerulonephritis evolution in Castilla-La Mancha (GLOMANCHA) between 1994 and 2008

Antecedentes: Los registros de biopsias renales permiten la recogida de datos histopatológicos que, puestos en su conjunto, ayudan a comprender enfermedades y su historia natural. Objetivos: Analizar los datos del Registro de Glomerulonefritis de Castilla-La Mancha (GLOMANCHA) y la evolución de las diferentes enfermedades biopsiadas (1994-2008). Métodos: Se recogieron las 6 enfermedades biopsiadas más prevalentes durante los 14 años (941 biopsias) en 5 hospitales de la comunidad autónoma. En 2008 se evaluó la situación vital y renal de los pacientes y se analizaron los factores asociados a dicha situación en cada enfermedad. Resultados: De las 941 biopsias, el 59% fueron de varones con una edad media de 48±18 años. En el momento de la biopsia la mediana de filtrado glomerular (FG) era de 50,3 (25,5-76,3) ml/min/1,73 m2 y la de proteinuria de 3,4 (1,5-6,4) g al día. La enfermedad más frecuente fue la nefropatía lúpica, seguida de la glomeruloesclerosis focal y segmentaria, y de la membranosa. El mejor pronóstico renal durante el seguimiento (media 7,3±4,8 años) fue el de la nefropatía lúpica y la nefropatía por cambios mínimos; la glomeruloesclerosis focal y segmentaria y las glomerulonefritis rápidamente progresivas de tipo 3 tuvieron el peor pronóstico renal. Esta última, además, tuvo el peor pronóstico vital. Conclusiones: GLOMANCHA demuestra el mal pronóstico de las glomerulonefritis rápidamente progresivas de tipo 3, a diferencia de la nefropatía por cambios mínimos y lúpica. La función renal es un predictor independiente de supervivencia renal y de mortalidad en nuestra población (AU)

Background: Renal biopsy registries allow histopathological data to be collected to improve knowledge of different pathologies and their natural history. Aim: To analyse the data of the Castilla La Mancha Glomerulonephritis Registry (GLOMANCHA) and the evolution of the different biopsy-proven pathologies between 1994 and 2008. Methods: The 6 most common biopsy-proven pathologies were collected during the 14 years of the study (941 biopsies) in the 5 participant centres of the autonomous community. In 2008, we assessed patient renal survival and mortality and we evaluated associated factors to each situation for each pathology. Results: Of the 941 biopsies, 59% belonged to men, with a mean age of 48±18 years. At the time of the biopsy, the median glomerular filtration rate was 50.3 (25.5-76.3) ml/min/1,73 m2 and median proteinuria was 3.4 (1.5-6.4) grams per day. The most common pathology were lupus nephropathy, followed by focal segmental glomerulosclerosis and membranous nephropathy. Lupus nephropathy and minimal change disease achieved the best renal prognosis during follow-up (mean 7.3±4.8 years). Rapidly progressive glomerulonephritis type 3 and focal segmental glomerulosclerosis had the worst renal prognosis. In addition, rapidly progressive glomerulonephritis type 3 presented the worst vital prognosis. Conclusions: In GLOMANCHA, we demonstrate the poor prognosis of rapidly progressive glomerulonephritis type 3, in contrast to minimal change disease or lupus nephropathy. Renal function is an independent predictor of renal survival and mortality in this study (AU)

Study of glomerulonephritis evolution in Castilla-La Mancha (GLOMANCHA) between 1994 and 2008. / Estudio evolutivo de las glomerulonefritis en Castilla-La Mancha (GLOMANCHA) en el periodo 1994-2008.

BACKGROUND: Renal biopsy registries allow histopathological data to be collected to improve knowledge of different pathologies and their natural history. AIM: To analyse the data of the Castilla La Mancha Glomerulonephritis Registry (GLOMANCHA) and the evolution of the different biopsy-proven pathologies between 1994 and 2008. METHODS: The 6 most common biopsy-proven pathologies were collected during the 14 years of the study (941 biopsies) in the 5 participant centres of the autonomous community. In 2008, we assessed patient renal survival and mortality and we evaluated associated factors to each situation for each pathology. RESULTS: Of the 941 biopsies, 59% belonged to men, with a mean age of 48±18 years. At the time of the biopsy, the median glomerular filtration rate was 50.3 (25.5-76.3) ml/min/1,73 m(2) and median proteinuria was 3.4 (1.5-6.4) grams per day. The most common pathology were lupus nephropathy, followed by focal segmental glomerulosclerosis and membranous nephropathy. Lupus nephropathy and minimal change disease achieved the best renal prognosis during follow-up (mean 7.3±4.8 years). Rapidly progressive glomerulonephritis type 3 and focal segmental glomerulosclerosis had the worst renal prognosis. In addition, rapidly progressive glomerulonephritis type 3 presented the worst vital prognosis. CONCLUSIONS: In GLOMANCHA, we demonstrate the poor prognosis of rapidly progressive glomerulonephritis type 3, in contrast to minimal change disease or lupus nephropathy. Renal function is an independent predictor of renal survival and mortality in this study.