Colangitis biliar primaria. Parte 1. Actualización: generalidades, epidemiología, factores involucrados, fisiopatología y manifestaciones clínicas / Primary biliary cholangitis. Part 1. State of the art, epidemiology, physiopathology and clinical manifestations

La colangitis biliar primaria (CBP), es una colangiopatía crónica caracterizada por la destrucción selectiva de las células epiteliales biliares de conductos hepáticos de pequeño y mediano calibre, que afecta principalmente a mujeres. Los principales síntomas son la fatiga y el prurito, sin embargo, gran porcentaje de los pacientes pueden ser asintomáticos. El diagnóstico se basa en anticuerpos antimitocondriales (AMA) con títulos >1:40, fosfatasa alcalina >1,5 veces del límite superior normal por más de 24 semanas e histología hepática compatible con la patología. Se asocia con múltiples enfermedades principalmente de carácter autoinmune extra hepáticas, enfermedades tiroideas, óseas, entre otras. El tratamiento de primera línea es el ácido ursodesoxicólico (AUDC) que a pesar que no cura la enfermedad, mejora las pruebas del perfil hepático, así como el retraso en la progresión a cirrosis. Actualmente se encuentran en estudio nuevos tratamientos y terapias adyuvantes. El propósito de esta revisión es ofrecer una actualización de este tema que se presenta en los servicios de medicina interna y gastroenterología; para su realización se conformó un equipo interdisciplinario que desarrolló una búsqueda en la base Medline a través de PubMed con las palabras claves correspondientes y se procedió a una lectura crítica y analítica de títulos, resúmenes y textos completos para el filtro, extracción y síntesis de la información encontrada

Primary biliary cholangitis (PBC) is a chronic autoimmune cholangiopathy characterized by a selective destruction of biliary epithelial cells of small and medium caliber hepatic ducts, which mainly affects women. The main symptoms are fatigue and pruritus, however, a large proportion of patients may be asymptomatic. The diagnosis is based on AMA titers >1:40, alkaline phosphatase >1.5 times the upper limit for more than 24 weeks and compatible liver histology. It is associated with multiple autoimmune diseases mainly extrahepatic, thyroid diseases, bone diseases, among others. The first line treatment is ursodeoxycholic acid (UDCA), that improves liver function tests and delay the progression to cirrhosis. Currently, there are new treatments and adjuvant therapies on study. The purpose of this review is to offer an update in this topic, which is very important in gastroenterology and internal medicine. We formed an interdisciplinary team to search in the database Medline thorough PubMed with the key words describe below, we made a critical lecture of the titles and abstracts of each article to write this paper

Cirrosis biliar primaria: aspectos clinico- epidemiológicos en una población uruguaya / [Primary biliary cirrhosis: clinical and epidemiological features in an Uruguayan population].

INTRODUCTION: Primary biliary cirrhosis (PBC) is a chronic cholestatic, autoimmune, liver disease produced by inflammation and destruction of the interlobular bile ducts. It is more frequent among female patients and is usually diagnosed in the fifth decade of life. OBJECTIVE: Our objective was to describe the clinical and epidemiological characteristics of patients with PBC in Uruguay. MATERIAL AND METHODS: This descriptive study included patients from 3 medical centers diagnosed with PBC in the period January 2002 to September 2011. The diagnosis was based on the presence of at least two of the following requirements: cholestasis, antimitochondrial antibodies (AMA) (or AMA subtype 2) or positive antinuclear antibodies (ANA) (anticentromere pattern) and compatible biopsy. Data recorded were sex, age, symptoms, related illness, laboratory results, images and histology at the moment of the diagnosis. RESULTS: We included 81 patients, 94

were women and the mean age was 56 years old (range: 31 to 79 years old). Symptoms were present in 59 patients (73

) and pruritus, found in 51 of them (86

), was the most frequent symptom. Positive AMA was found in 84

of cases. Histological study was available in 35 patients (43

) and 13 of them (37

) had cirrhosis. The mean survival according to the presence or absence of cirrhosis was 9.17 years (95

confidence interval: 6.79-11.56) and 10.7 years (95

confidence interval: 9.27-12.14), respectively (P = 0.03). CONCLUSIONS: Female predominance and frequent association with other autoimmune diseases were confirmed in this group. Although there was a high percentage of symptomatic and cirrhotic patients at diagnosis, only the presence of cirrhosis was associated with a lower survival.

Negative conversion of antimitochondrial antibody in primary biliary cirrhosis: a case of autoimmune cholangitis

Autoimmune cholangitis is a clinical constellation of chronic cholestasis, histological changes of chronic nonsuppurative cholangitis and the presence of autoantibodies other than antimitochondrial antibody (AMA). It is uncertain whether this entity is definitely different from AMA positive primary biliary cirrhosis (PBC), though it shows some differences. We report a case of autoimmune cholangitis in a 59-year-old woman, who had been previously diagnosed as AMA-positive PBC associated with rheumatoid arthritis, has been converted to an AMA-negative and anticentromere antibody-positive PBC during follow-up. The response to ursodeoxycholic acid treatment is poor except within the first few months, but prednisolone was dropping the biochemical laboratory data.

Anticuerpos contra mitocondrias, corazón y perinúcleo. ¿Asociación con el título de anticuerpos anticardiolipina en el síndrome antifosfolípido primario?: estudio exploratorio / Seric antibodies as antiheart, antimithocondrial and antiperinuclear. These antibodies es related to the anticardiolipin antibodies level. Exploratory study

En el suero de pacientes con síndrome antifosfolípido primario se han descrito anticuerpos antimitocondriales (AM), anticorazón (ACOR) y contra perinúcleo (AP); algunos se nagativizan al inhibir los sueros con micelas de cardilipina. Decidimos evaluar si la fecuencia de AM, ACOR y AP tiende a aumentar en proporción directa al título de anticuerpos anticardiolipina (aCL). Se incluyeron sueros de pacientes: 12 con SAFP y 3 con lupus eritematoso sistémico. Se distribuyeron en 3 grupos de 5 sueros cada uno según el título de aCL (ensayo inmunoenzimático, punto de corte densidad óptica 0.21). Grupo bajo (DO de 0.816 a 1.360) y grupo alto (DO de 1.850 a 3.000). Detección de AM, ACOR y AP por inmunofluorescencia indirecta. Los resultados fueron: positivos, grupo bajo (O), grupo medio MA y ACOR en 1/5 y AP en 1/5 respectivamente, grupo alto ACOR y AP en el 20 por ciento (1/5), ACOR en el 20 por ciento (1/5), y AP en el 40 por ciento (2/5) y AM en 0 por ciento. Observamos tendencia a mayor frecuencia de ACOR y AP en relación directa a título de aCL y aunque el tamaño de la muestra no permite ser concluyente, los resultados justifican estudios posteriores para dilucidar este aspecto

Un nuevo sustrato para la deteccón de anticuerpos antimicondriales en suero humano / A new substrate for detection of antimitochondrial antibodies in human serum

This work describes a new model to detect antimitochondrial antibodies using indirect immunofluorescence on mouse sperm as substrate. As controls conventional substrates and mitochondrial protein immunoblots were esed. An intense fluorescent reaction was visualized in the mitochondrial sheet of mouse sperms allowing a straightforward diagnosis of positive sera. Sera coming from 10 patients with progressive systemic sclerosis, 12 patients with systemic lupus erythematosus and 17 patients with primary biliary cirrhosis were tested with this method, confirming results obtained with conventional tests that use indirect immunofluorescence and rat frozen kidney slices as substrate. The new method is simpler, more accurate and has a lower margin of error