Colangitis biliar primaria: experiencia de cinco años en el Hospital Clínico de la Universidad de Chile / Primary biliary cholangitis. Experience in 179 patients

BACKGROUND: Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic disease, which can progress to cirrhosis. It mainly affects middle-aged women. Its most frequent form of presentation is asymptomatic with biochemical cholestasis and the presence of antimitochondrial antibodies (AMA). AIM: To describe the epidemiological characteristics, clinical presentation and treatment for patients with PBC at a clinical hospital. MATERIAL AND METHODS: Descriptive, observational, retrospective study, carried out between January 2015 and December 2020. Results: 179 patients (158 women) were cared in the study period. At the time of diagnosis, the median age was 54 years (range 24-76), 55% of them were asymptomatic, 45% had fatigue and 28% had pruritus. Positive AMA were present in 65% of patients, antinuclear antibodies (ANA) in 51%, and anti-smooth muscle antibodies (ASMA) in 9%. Immunoglobulin M (IgM) was elevated in 30% of the patients and 50% of patients were biopsied. Splenomegaly and esophageal varices were present in 24 and 22% of patients, respectively. PBC was associated with Sjogren's syndrome in 15%, hypothyroidism in 14%, osteoporosis in 13%, and scleroderma in 8%. CONCLUSIONS: The epidemiological characteristics of our patients agree with those published abroad. Laboratory cholestasis associated with the presence of AMA, currently allows diagnosis without the need for histological study. Ursodeoxycholic acid (UDCA) is the first-line treatment for patients with PBC. The use of biochemical response criteria is essential to identify patients who require other UDCA alternatives for isolated or combined treatment.

Association between three autoimmune diseases: vitiligo, primary biliary cirrhosis, and Sjögren's syndrome

Abstract Although the association of multiple autoimmune diseases has already been widely described, no reports of the association between vitiligo, primary biliary cirrhosis and Sjogren's syndrome were retrieved in the SciELO and PubMed databases. The authors describe the case of a female patient who was diagnosed with primary biliary cirrhosis and Sjogren's syndrome at age 54. At age 58, she developed vitiligo restricted to the face, associated with significant impairment of self-esteem and quality of life. Antinuclear antibody was negative at the onset of the condition, but became positive after phototherapy initiation. In general, the occurrence of multiple autoimmune diseases in the same patient is known as a mosaic of autoimmunity. However, specific mechanisms appear to interconnect primary biliary cirrhosis and Sjogren's syndrome, such as PDC-E2-mediated generalized epithelitis.

Novedades en enfermedades colestásicas / Latest advances in cholestatic diseases

Cholestatic liver diseases arise from impaired hepatobiliary production and excretion of bile. They have some common clinical manifestations and pathogenic features, but at the same time differences that require a special approach. Intrahepatic cholestasis of pregnancy and drug induced liver cholestasis are the most common reversible intrahepatic cholestasis. Primary biliary cirrhosis and primary sclerosing cholangitis are chronic cholestatic diseases. This review will be focused on these two types, in particular their clinical and therapeutic management and complications. While in recent years there has not been much change in the basic clinical approach of these diseases, every day we receive more information from both the basic and clinical science studies, which has enabled to develop new therapeutic lines and reject others that have not confirmed effectiveness.

Las enfermedades colestásicas se caracterizan por la disminución de la formación o excreción del flujo de bilis. Ellas tienen ciertas manifestaciones clínicas y mecanismos patogénicos comunes, pero a su vez diferencias que requieren un enfrentamiento no siempre similar. La colestasia intrahepática del embarazo(CIE) y la colestasia secundaria a fármacos son las más frecuentes dentro de las colestasias intrahepáticas reversibles. La cirrosis biliar primaria (CBP) y la colangitis esclerosante primaria (CEP), son las enfermedades colestásicas crónicas en que centramos esta revisión, en particular en los aspectos clínicos y de manejo terapéutico tanto de éstas, como de sus complicaciones. Si bien en los últimos años no ha habido un cambio significativo en el manejo fundamental de estas enfermedades, cada vez tenemos más información tanto en el área de ciencias básicas como en aspectos clínicos, lo que ha permitido ir desarrollando nuevas líneas terapéuticas y descartando otras que no han confirmado efectividad.

Pseudocisto pancreático associado à doença hepática na gestação: relato de caso / Pancreatic pseudocyst associated with liver disorder in pregnancy: case report

As doenças hepáticas, de vias biliares e pancreáticas podem causar grande morbimortalidade materno-fetal. Nesses casos, o diagnóstico e o tratamento imediatos são essenciais para reverter esse processo e melhorar o prognóstico materno-fetal. Apresentamos um caso clínico de paciente primigesta de 17 anos que foi internada por disfunção hepática severa. Ela foi submetida a cesariana e a duas laparotomias subsequentes decorrentes de sangramento e evisceração. Posteriormente, a paciente apresentou pseudocisto pancreático com manejo conservador e boa evolução. Concluímos que o conhecimento das doenças hepáticas, de vias biliares e pancreáticas na gestação e suas complicações é fundamental para reduzir a morbimortalidade materno-fetal.

Pregnancy-associated liver, biliary and pancreatic disorders are conditions that can cause high maternal and fetal morbidity and mortality. In such cases, prompt diagnosis and immediate treatment are essential for the reversal of the underlying process and for maternal and fetal survival. We presented a clinical case of a 17-year-old primigravid woman hospitalized with severe liver dysfunction. She subsequently evolved to cesarean delivery and two laparotomies secondary to bleeding and evisceration. Afterwards, she presented with pancreatic pseudocyst. She was submitted to conservative management and had a good evolution. We concluded that the knowledge of the pregnancy-associated liver, biliary and pancreatic disorders and their complications is fundamental to reduce maternal and fetal morbidity and mortality.

Afecciones hepatobiliares autoinmunes / Auto-immune hepatobiliary diseases

Las afecciones hepato-biliares autoinmunes son relativamente infrecuentes en la práctica clínica y es posible que una cierta proporción de ellas pasen inadvertida o se incluyan en el grupo de las hepatopatías criptogénicas. Histológicamente se caracterizan por una inflamación crónica, persistente y progresiva de las células y conductos intrahepáticos que es característica sólo en las etapas iniciales ya que en las avanzadas son ocultadas por la fibrosis propia de la cirrosis. Clínicamente se destacan las formas colestásicas con prurito, ictericia, compromiso del estado general. En las formas avanzadas aparecen las manifestaciones de insuficiencia hepática; la hipertensión portal y las neoplasias (hepatocarcinoma y colangiocarcinomas) que son menos frecuentes en estas hepatologías autoinmunes que en las por virus C. El tratamiento médico se basa en el empleo de corticoides y/o azatioprina en el caso de las hepatitis autoinmunes y de colestiramina o ácido ursodeoxicólico en la cirrosis biliar primaria y en la colangitis esclerosante.

Xantomatosis eruptiva: a propósito de un caso / Eruptive xanthomata

Se presenta el caso de un paciente de sexo masculino y 41 años, diabético tipo II, que manifiesta la aparición en forma abrupta de múltiples lesiones diagnosticadas como xantomas eruptivos. Debido a que la hiperlipidemia puede presentarse con xantomas, los dermatólogos pueden ser los primeros en diagnosticar éstas lesiones cutáneas asociadas con anormalidades lipídicas

Colangite esclerosante primária / Primary Sclerosing Cholangitis

Colangite esclerosante primária (CEP) é uma doença colástica crônica e progressiva, de etiologia desconhecida, caracterizada por inflamação e fibrose das biliares intra e extra- hepáticas. Dois terços dos pacientes são portadores, também, de retocolite ulcerativa inespecífica (RCUI), por outro lado, CEP é a hepatopatia crônica mais comum entre os portadores de doença inflamatória intestinal (DII)...

Osteopenia en cirrosis biliar primaria: estudio en 20 pacientes / Osteopenia in primary biliary cirrhosis: study in 20 patients

We studied 20 female patients, aged 51 ñ 13.6 years old, with the diagnosis of Primary Biliary Cirrhosis (PBC) to assess osteopenia, main involved sites and its relation to menopause, some parameters of mineral metabolism and the degree of histological liver involvement. The diagnosis of PBC was based in histological, clinical and laboratory features. Bone densitometry was measured with dual-photon densitometer and compared with values of a normal female population from the laboratory. When compared to controls, studied patients had a significantly lower lumbar spine bonedensity and total bone mineral content and a non significantly lower density in femoral neck and total body. Serum calcium, phosphorus, magnesium, PTH and urinary calcium/creatinine ratio were within normal limits. Lumbar spine density was no significantly lower in patients with more severe liver histological involvement and in postmenopausal women. No correlation was found between the duration of postmenopausal period and the degree of osteopenia. It is concluded that patients with PBC have a clear lumbar spine osteopenia and a lower total mineral content and that these parameters worsen in a non significant fashion in subjects along with liver histological involvement and with the length of post menopausal period

Estudo post-mortem da prevalência da litíase vesicular em uma populaçäo cirrótica / Postmortem study of prevalence of vesicular lithiase in a cirrhotic population

Num estudo retrospectivo em necrópsias de 1964 a 1989, foi apreciada a prevalência de litíase vesicular em pacientes com e sem cirrose. Nos cirróticos, a freqüência de litíase foi de 13,23//, em comparaçäo com 8,35// na populaçäo näo cirrótica. Näo houve significância estatística. Entretanto, essa conclusäo näo pode ser considerada como definitiva, por ter sido pequena a populaçäo cirrótica estudada. Encontramos maior prevalência de esplenomegalia entre os cirróticos com litíase do que nos cirróticos sem colelitíase

Cirrose biliar primária associada a esclerodermia e síndrome de Sjögren / Primary biliary cirrhosis associated with scleroderma and Sjogren's syndrome

Relatamos um caso clínico de uma paciente de 49 anos que apresentava cirrose biliar primária, associada a esclerodermia e Síndrome de Sjögren. O diagnóstico da cirrose biliar primária foi confirmado através de dosagem de fosfatase alcalina, anticorpos anti-mitocondriais e biópsia hepática; o de esclerodermia através da clínica e da biópsia da pele. Para a Síndrome de Sjögren realizamos teste de Shirmer, Rosa-bengala e biópsia labial. Pretendemos com este relato levantar a possibilidade de uma etiopatogenia comum para diferentes doenças auto-imunes

Síndrome hepatocutáneo / Hepato-cutaneous syndrome

El síndrome hepato-cutáneo comprende la asociación de hepatopatías autoinmunes, cirrosis biliar primaria o hepatitis crónica activa y lesiones cutáneas de tipo prurigoide debidas a vasculitis alérgicas. La coexistencia con otras entidades de idéntica patogenica le haría formar parte de una enfermedad autoinmune multisistémica