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.

Esclerodermia y Cirrosis Biliar Primaria (Síndrome de Reynolds): Descripción de un Caso / Scleroderma and Primary Biliary Cirrhosis (Reynolds syndrome): Case Description

El síndrome de Reynolds, se define como cirrosis biliar primaria en pacientes con esclerodermia; este síndrome debe ser sospechado en aquellos pacientes que desarrollen un patrón colestásico. Se reporta una paciente con antecedente de esclerodermia que se presenta con ictericia, a quien se le confirma con estudios inmunológicos y biopsia hepática, el diagnóstico de cirrosis biliar prima­ ria (ahora se denomina colangitis biliar primaria). Se ordena ácido ursodesoxicólico 15mg/día.

Reynolds syndrome is defined as primary biliary cirrhosis in patients with scleroderma; this syndro­me should be suspected in those patients who develop a cholesteric pattern. We report a patient with scleroderma who presented with jaundice. After immunological and liver biopsy, a diagnosis of Primary Biliary Cholangiopathy (new name) was confirmed. Ursodeoxycholic acid 15mg / day was prescribed

LIVER BIOPSY: IMPORTANCE OF SPECIMEN SIZE IN THE DIAGNOSIS AND STAGING OF CHRONIC VIRAL HEPATITIS

Liver biopsy is the gold standard method for the grading and staging of chronic viral hepatitis, but optimal biopsy specimen size remains controversial. The aim of this study was to evaluate the quality of liver specimen (number of portal tracts) and to evaluate the impact of the number of portal tracts in the staging of chronic hepatitis. Material and Methods: 468 liver biopsies from consecutive patients with hepatitis C virus and hepatitis B virus infection from 2009 to 2010 were evaluated. Results: The length of fragment was less than 10 mm in 43 cases (9.3%), between 10 and 14 mm in 114 (24.3%), and ≥ 15 mm in 311 (64.4%); of these, in 39 (8.3%) cases were ≥ 20 mm. The mean representation of portal tracts was 17.6 ± 2.1 (5-40); in specimens ≥ 15 mm the mean portal tract was 13.5 ± 4.7 and in cases ≤ 15 mm was 11.4 ± 5.0 (p = 0.002). Cases with less than 11 portal tracts were associated with F3, and cases with 11 or more portal tracts with F2 (p = 0.001). Conclusion: this study demonstrated the good quality of liver biopsy and a relationship between the macroscopic size of the fragment and the number of portal tracts.

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.

Cirrosis biliar primaria / Primary biliary cirrhosis

La cirrosis Biliar Primaria, es una enfermedad crónica, colestásica del higado, caracterizada por la destrucción inmunoinflamatoria de ductos biliares intrahepáticos. Es de curso progresivo y evoluciona a cirrosis, produciendo muerte por insuficiencia hepática o por hemorragia secundaria a ruptura de várices esófago-gástricas.

Caso clínico: una mujer de 65 años, con prurito y astenia / Clinical case: a sixty five year old woman suffering from itching and fatigue

Se presenta el caso de una mujer con historia de 3 años de evolución clínica caracterizada por purito y astenia acentuada, sin mejoría con el tratamiento médico. El laboratorio mostró alteraciones de la función hepática y el examen físico no relevó alteraciones significativas. El diagnóstico fue aclarado con biopsia hepática y anticuerpos específicos compartibles con Cirrosis biliar primaria. Una rara entidad de etiología autoinmune, con clara predilección por el sexo femenino, que debe ser considerada en el diagnóstico diferencial de las hepatopat¡as crónicas. Se discuten aspectos clínicos epidemiológicos y pronósticos actualizados de esta infrecuente enfermedad

Non-Hodgkin's lymphoma & primary biliary cirrhosis with Sjogren's syndrome

Sjogren's syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of the salivary and lacrimal glands leading to a progressive destruction of these glands due to the production of autoantibodies. This disorder is either isolated (primary SS) or associated with other systemic diseases (secondary SS). The occurrence of B-cell non-Hodgkin's lymphoma (NHL) represents the major complication in the evolution of SS patients. The risk of developing NHL, which is equivalent for both primary and secondary SS, was estimated to be 44 times greater than that observed in a comparable normal population. NHLs in SS patients occur preferentially in the salivary glands and in other mucosa-associated lymphoid tissues (MALT). However, it can also occur in the lymph nodes or bone marrow. We documented a case of low-grade B-cell lymphoma of MALT in the right eyelid and primary biliary cirrhosis (PBC) of a patient with SS. To the best of our knowledge, this is the first case reported in Korea.

Primary biliary cirrhosis and myopathy: an uncommon association

Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases

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.

Primary biliary cirrhosis in a patient with progressive systemic sclerosis, a case report

A 63-year-old woman suffering from progressive systemic sclerosis for about 20 years disclosed symptoms of liver disease within the last three years. Diagnosis of biliary cirrhosis was established on the basis of clinical picture, pathological examination of the hepatic tissue sample, immunological tests, and x-ray studies. Association of systemic sclerosis with primary biliary cirrhosis is briefly reviewed

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

Sinusoidal endothelial hyperplasia in pathogenesis of perilobular fibrosis in rabbit after experimental ligation of common bile duct.

Development of perilobular hepatic fibrosis in rabbits after experimental ligation of the common bile duct was studied by microscopy. Twelve hours after the ligation, the lobular boundaries assumed prominence by appearance of row(s) of congested sinusoids around the distended perilobular canals of Hering. Seven days later, the lobular laminae limitans around such canals of Hering revealed myxomatous ballooning degeneration forming well defined inter-lobular bands (ILBs). On day 15 post-ligation, the residual sinusoidal endothelial-lining cells in the ILBs manifested hyper chromasia and occasional mitotic activity. It was followed, on days 25 and 35, by proliferation (hyperplasia) of the sinusoidal endothelium cells forming the portal tract like fibrotic tissue in the perilobular bands. These findings, showed the course of interlobular bridging necrosis along the bile preductules at the lobular circumferences and subsequently indigenous origin of the fibrotic tissue in the ILBs from the residual cells in the affected parenchyma independent of the portal tracts.