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.

Hepático-yeyunostomía estenosada con absceso hepático y fístula bilo pleural / Stenosed hepatic jejunostomy with hepatic abscess and biliary pleural fistula

La iatrogenia biliar cobra una importancia particular en nuestros tiempos, pues después de la era laparoscópica su incidencia no solo se mantiene en altos índices, sino que la envergadura de las lesiones tiende a ser mayor. Se presentan pacientes con complicaciones más complejas que demandan de esfuerzos extraordinarios y de un enfoque multidisciplinario. Se presenta un paciente con una lesión iatrogénica de la vía biliar, complicado con una cirrosis biliar, un absceso hepático con fístula biliopleural y se explica su manejo, con buenos resultados. Con este trabajo se pretende comunicar un caso singularmente complejo que fue enviado a nosotros después de múltiples intentos de reparación y estenosis de una lesión iatrogénica de la vía biliar(AU)

Biliary iatrogeny gains particular importance in our times, since its incidence after laparoscopic era is not only high but the significance of the lesions tends to be greater. More patients with more complex complications demanding extraordinary efforts and multidisciplinary approach appear. Here is a patient suffering iatrogenic lesion of the bile duct, complicated with biliary cirrhosis, a hepatic abscess with biliary pleural fistula was presented, along with the management of the patient with good results. This paper was intended to show a particularly complex case that was referred to our service after a lot of repair attempts and the stenosis of iatrogenic lesion of the bile duct(AU)

Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia / Primary biliary cirrhosis: a thirteen years experience

Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. Aim: To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. Material and methods: Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver transplantation, were recorded. Result: Seventy eight percent of patients were symptomatic at presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in 56%. No clinical or laboratory differences were observed between symptomatic patients or those with positive antimitochondrial antibodies and the rest of the study group. Sjögren syndrome was present in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61% had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred. Conclusions: An early diagnosis and treatment of a progressive disease such as PBC will reduce the incidence of complications and the use of costly treatments.

Cirrose biliar primária: relato de um caso tratado com ácido ursodesoxicólico / Primary biliar cirrhosis: a case report of a treatment with ursodesoxicholic acid

A cirrose biliar primária é de incidência rara e pouco registrada no Brasil. O prurido persistente numa mulher de meia-idade, associado a colestase detectada em laboratório, levou-nos a hipótese da doença, confirmada histologicamente. O uso do ácido ursodesoxicólico teve resposta favorável, tanto no quadro clínico como laboratorial