Seronegative Autoimmune Hepatitis: Case Report / Hepatitis autoinmune seronegativa: reporte de un caso

Abstract Autoimmune hepatitis (AIH) is an immune-mediated inflammatory disease that requires the integration of histological abnormalities, characteristic laboratory findings, autoantibody positivity, and exclusion of other liver diseases for diagnosis. The case of a 28-year-old female patient with a week-long history of generalized jaundice associated with diffuse and intermittent abdominal pain is presented. Liver tests revealed hepatocellular injury (R factor = 17.6) with severe transaminitis (aspartate aminotransferase [AST]: 1.502 IU/L, alanine aminotransferase [ALT]: 2.029 IU/L) and conjugated hyperbilirubinemia (total serum bilirubin: 10.9 mg/dL, direct bilirubin: 8.50 mg/dL). Serological tests for hepatotropic viruses were negative, as were autoantibodies for autoimmune hepatitis. Percutaneous liver biopsy revealed findings compatible with autoimmune hepatitis. The revised 1999 International Autoimmune Hepatitis Group (IAIHG) score was calculated pretreatment, resulting in a score of 16, thus diagnosing it as definitive severe AIH. The patient was treated with oral steroids, obtaining clinical and biochemical improvement, so she was discharged after seven days of hospitalization without incidents. Maintaining a high index of suspicion for AIH, despite the initial negativity of autoantibodies, and complementing the diagnostic approach with percutaneous liver biopsy allow the timely diagnosis and treatment of this group of patients, thus preventing progression to advanced cirrhosis and its complications.

Resumen La hepatitis autoinmune (HAI) es una enfermedad inflamatoria inmunomediada la cual requiere para su diagnóstico la integración de anomalías histológicas, hallazgos de laboratorio característicos, positividad de autoanticuerpos y exclusión de otras enfermedades hepáticas. Se presenta el caso de una paciente femenina de 28 años con un cuadro de ictericia generalizada de una semana de evolución asociado a dolor abdominal difuso e intermitente. Las pruebas hepáticas revelaron lesión hepatocelular (factor R= 17,6) con transaminasemia grave (aspartato-aminotransferasa [AST]: 1,502 UI/L, alanina-aminotransferasa [ALT]: 2,029 UI/L) e hiperbilirrubinemia conjugada (bilirrubina sérica total: 10,9 mg/dL, bilirrubina directa: 8,50 mg/dL). Las pruebas serológicas para virus hepatotropos fueron negativas, así como los autoanticuerpos para hepatitis autoinmune. La biopsia hepática percutánea reveló hallazgos compatibles de hepatitis autoinmune. Se calculó el puntaje revisado del Grupo Internacional de Hepatitis Autoinmune (IAIHG) de 1999 pretratamiento, en el que se obtuvo un puntaje de 16, por lo que se diagnosticó como HAI grave definitiva. La paciente fue manejada con esteroides orales y se obtuvo una mejoría clínica y bioquímica, por lo que fue egresada a los siete días de hospitalización, sin eventualidades. Mantener un alto índice de sospecha de HAI, a pesar de la negatividad inicial de los autoanticuerpos, y complementar el abordaje diagnóstico con biopsia hepática percutánea permite el diagnóstico y tratamiento oportuno de este grupo de pacientes, de modo que se previene la progresión a cirrosis avanzada y sus complicaciones.

An unusually prolonged course of late onset severe OHSS with successful outcome - a rare but avoidable complication.

Ovarian hyperstimulation syndrome (OHSS) is an entity associated with controlled ovarian stimulation (COS) in assisted reproduction. It presents a great challenge for clinicians concerning a timely diagnosis and intervention, because it is a life-threatening condition. We came across a rare case of late onset severe OHSS, which lasted till 19 weeks of gestation (POG) with diagnostic challenge in which the patient had hemorrhagic ascites post embryo transfer, and transaminitis. The case was managed effectively with repeated paracentesis along with replacement therapy, and the transaminitis was also gradually resolved. Severe OHSS may follow an unusually prolonged course associated with transaminitis. Our case suggests that it is crucial to distinguish between the nature and etiology of transaminitis along with OHSS, and other pathologies like drug induced transaminitis should also be kept in mind.