RESUMO
UNLABELLED: Reversibility of liver fibrosis with immunosuppressive therapy (IT) has been described in autoimmune hepatitis (AIH). OBJECTIVE: To compare initial fibrosis and fibrosis after IT in patients with AIH. METHODS: A total of 54 patients were admitted with positive ANA or AML antibodies, or both, elevated IgG immunoglobulins and who met international criteria for a diagnosis of AIH. The mean age was 39 years (range 13-65) and there were 47 women (87%). Two liver biopsies were taken: one at diagnosis and another at a mean of 28±8 months after initiation of IT with prednisone and azathioprine. The degree of inflammation (0-18) and fibrosis (0-6) according to Ishak score was compared between the initial and the follow-up biopsy. RESULTS: Fibrosis decreased from 2.9±0.3 to 2.2±0.3 (p=0.005) and histological activity index from 6.8±0.45 to 2.6±0.2 (P<.001). In subgroups, fibrosis decreased from 3.6±0.4 to 1.4±0.3 (P<.001) in 22 patients (41%), was unchanged in 27 (50%) and increased in five (9%). There were seven patients with histological cirrhosis at IT initiation. After IT, four showed a reduction in Ishak score (achieving scores of 0-3). Transaminase values were not associated with histological improvement. CONCLUSION: Fibrosis in patients with AIH significantly improved with IT, emphasizing the importance of studying the prognostic factors associated with this favorable response.
Assuntos
Azatioprina/uso terapêutico , Hepatite Autoimune/complicações , Imunossupressores/uso terapêutico , Cirrose Hepática/tratamento farmacológico , Cirrose Hepática/patologia , Prednisona/uso terapêutico , Adolescente , Adulto , Idoso , Feminino , Humanos , Cirrose Hepática/etiologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Indução de Remissão , Adulto JovemRESUMO
UNLABELLED: Celiac disease (CD) has been associated with non-alcoholic fatty liver disease (NAFLD) and other chronic liver diseases (CLD), such as primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC). AIM: To study the frequency of serological markers of CD in patients with NAFLD and CLD and their correlation with duodenal biopsy. PATIENTS AND METHODS: In patients with NAFLD, PBC, AIH and PSC, we studied anti-endomysium (AE) IgA by indirect immunofluorescence and anti-gliadin IgA-IgG (AG) and human anti-tissue transglutaminase (tTG) IgA antibodies by an ELISA technique. Patients with positive serology for at least one marker underwent endoscopy with duodenal biopsies. RESULTS: Positive CD markers were found in 9 of 101 patients (8.9%): 7 patients were positive for tTG alone, 1 for AE and AG, and 1 patient for 3 antibodies. Positivity was as follows: 3/38 (7.9%) in NAFLD, 3/44 (6.8%) in PBC, 2/16 (12.5%) in AIH and 1/3 in PSC. Endoscopy was performed in 8 patients, with normal duodenal biopsy in 7 and 1 patient with Marsh stage 1 CD with NAFLD, positive AE and AG. The only patient with 3 positive markers died during the study without undergoing endoscopy. None of the patients had symptoms suggestive of CD. CONCLUSION: A high prevalence of positive tTG was found in patients with CLD and NAFLD. However, duodenal biopsy should be performed in these patients, given that the results of this procedure were normal in most patients in this study.
Assuntos
Doença Celíaca/complicações , Fígado Gorduroso/complicações , Hepatite Autoimune/complicações , Adolescente , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterized by progressive inflammation and fibrosis of the biliary tract, evolving to cirrhosis. It is commonly associated with inflammatory bowel disease (IBD). AIM: To communicate the clinical characteristics of patient with PSC seen in two reference centers. PATIENTS AND METHODS: Review of medical records of patients with PSC confirmed by liver biopsies. The clinical picture, laboratory abnormalities, imaging studies and treatment were recorded. RESULTS: Thirty three patients (aged 16 to 73 years, 64% female) were identified. They corresponded to 1.7% of liver biopsies done between 1991-2003. Clinical presentation was a cholestatic picture in 40%, right upper abdominal pain in 36%, a dysenteric syndrome in 9% and varied symptoms in 15%. Laboratory tests showed cholestasis in 94% and positive anti ANCA, SMA, ANA and AMA antibodies in 28, 18, 15 and 9% of cases, respectively. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiography were diagnostic in 43 and 58% of patients, respectively. There was an association with ulcerative colitis in 12% of cases. Liver biopsies showed grade I PSC in 76% and grade II-III in 6% of patients. It also showed a concomitant chronic hepatitis and primary biliary cirrhosis in 12 and 6% of cases, respectively. Treatment consisted on ursodeoxycholic acid (UDCA) in 45%, UDCA plus 5-aminosalicylic acid derivatives in 12% and UDCA plus immunosuppresors in 12% of patients. Two patients had to be transplanted. CONCLUSIONS: PSC is an uncommon cause of chronic liver disease. It is suspected in cholestatic patients and confirmed with a liver biopsy. It can be associated with other autoimmune hepatic and extrahepatic diseases.
Assuntos
Colangite Esclerosante/patologia , Adolescente , Adulto , Idoso , Biópsia , Colangite Esclerosante/imunologia , Colangite Esclerosante/terapia , Colestase Intra-Hepática/patologia , Feminino , Hepatite Autoimune/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
The most successful therapy for acute liver failure is liver transplantation. However, due to the low number of donors, organ support therapies need to be used as a bridge to liver transplantation. Molecular Adsorbents Recirculating System (MARS) is a dialysis treatment that uses a recirculating dialysate containing albumin. This allows the removal of both hydrosoluble and albumin-related substances. This system improves hepatic encephalopathy, renal dysfunction and some clinical parameters in acute liver failure, but there is no clear decrease in mortality. We report three women aged 23, 21 and 61 years, that were subjected to liver transplantation, in whom this therapy was successfully used.
Assuntos
Albuminas/uso terapêutico , Encefalopatia Hepática/terapia , Transplante de Fígado , Desintoxicação por Sorção/métodos , Injúria Renal Aguda/terapia , Adulto , Amônia/sangue , Bilirrubina/sangue , Biomarcadores/sangue , Nitrogênio da Ureia Sanguínea , Creatinina/sangue , Feminino , Humanos , Pessoa de Meia-Idade , Diálise Renal/métodosRESUMO
The therapeutic options for treatment of Achalasia of the esophagus include medical treatment, endoscopic and surgical procedures. The latter can be either conservative, such as cardiomyotomy or more aggressive, such as cardioplasty or esophageal resection. In this article, we discuss the early and long term results after the different therapeutic options. We also present the results of our recent surgical experience. The definitive results seem to be better after surgical treatment compared to medical management or endoscopic procedures.