RESUMO
INTRODUCTION: Several groups have reported associations of primary biliary cholangitis with other autoimmune entities, particularly Sjögren's syndrome and hypothyroidism. Its prevalence and characteristics in Mexican patients is unknown. AIM: To determine the frequency and characteristics of autoimmune diseases in a Mexican cohort of patients with primary biliary cholangitis. MATERIALS AND METHODS: The medical records of patients that presented with primary biliary cholangitis within the time frame of 2005 and 2012 were reviewed and assessed for other autoimmune diseases. RESULTS: Seventy-eight patients, 75 women and 3 men, were included. Their mean age was 55.8 years. Seventy-three cases had positive antimitochondrial antibodies (94.8%) and disease was confirmed in 5 through liver biopsy. Five patients (8%) had anti-smooth muscle antibodies and 55/78 (70.5%) had antinuclear antibodies by indirect immunofluorescence. Forty-nine patients (62.8%) were positive for an autoimmune disease other than primary biliary cholangitis. Among those, 20 patients had one associated disease, 14 had 2, and 15 patients had 3 concomitant diseases. They included: Sjögren's syndrome in 23/78 patients (29.5%), dysthyroidism in 21/78 cases (26.9%), Raynaud syndrome in 11/78 (14.1%), CREST syndrome in 9/78 patients (11.4%), rheumatoid arthritis in 6/78 patients (7.7%), vitiligo in 5/78 (6.4%), scleroderma in 4/78 patients (5.1%), and other diseases in 8 patients. In 12/78 patients (15.4%), there was a documented family background of autoimmune disease. CONCLUSIONS: The presence of autoimmune associations in our cohort was frequent, and similar in characteristics to the information reported by other groups. The clinical implications of those findings remain to be determined.
Assuntos
Doenças Autoimunes/epidemiologia , Doenças Autoimunes/imunologia , Cirrose Hepática Biliar/epidemiologia , Cirrose Hepática Biliar/imunologia , Adulto , Idoso , Autoanticorpos/análise , Doenças Autoimunes/complicações , Estudos de Coortes , Comorbidade , Feminino , Humanos , Cirrose Hepática Biliar/complicações , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Síndrome de SjogrenRESUMO
Eosinophilic gastroenteritis (EGE) is an uncommon disease and has rarely been reported in association with connective tissue diseases as systemic lupus erythematosus. We report a 36-year-old woman who developed recurrent episodes of abdominal pain, nausea, vomiting and melena. Complete blood counts showed elevated eosinophil counts. Ultrasound and CT-scan images studies were significant for bowel wall thickening and ascites. The patient underwent an exploratory laparotomy with a mesenteric biopsy and appendectomy that showed eosinophil infiltration in the muscularis propria, establishing the diagnosis of EGE. The patient developed pleural effusions, with laboratory studies showing haemolytic anaemia, thrombocytopenia, positive antinuclear antibody and anticardiolipin antibodies. The patient was treated with high-dose systemic corticosteroid therapy, with successful resolution of symptoms. Three months later, she developed a new episode of abdominal pain defined as intestinal pseudo-obstruction that was resolved without complications.
Assuntos
Eosinofilia/complicações , Gastroenterite/complicações , Lúpus Eritematoso Sistêmico/complicações , Adulto , Feminino , Gastroenterite/patologia , Humanos , Pseudo-Obstrução Intestinal/complicaçõesRESUMO
La dermatomiositis asociada a neoplasias ha sido reportada en el 11.8 por ciento de los casos. Hasta el momento, solo se ha descrito en la literatura un caso de asociación entre dermatomiositis y adenocarcinoma de vesícula biliar. Presentamos el caso de una paciente de 53 años con debilidad muscular proximal de cinturas escapular y pélvica, manifestaciones cutáneas (Gottron y eritema en heliotropo), e incremento de enzimas musculares, en quién se hizo el diagnóstico de Dermatomiositis y se le prescribió el tratamiento adecuado sin embargo, a los 3 meses de evolución acudió con pérdida de peso, mala respuesta a esteroides y metotrexate, síndrome ictérico, hemorragia de tubo digestivo; se le realizó ultrasonido de hígado y vías biliares reportándose datos compatibles con coledocolitiasis y daño hepático difuso, se le realizó colecistectomía y el reporte histopatológico fue de adenocarcinoma de vesícula biliar. El objetivo de este reporte es la presentación del segundo caso en que asocia la dermatomiositis y el adenocarcinoma de vesícula biliar en la literatura médica internacional.