Síndrome de superposición: lupus eritematoso sistémico y hepatitis autoinmune en una adolescente / An Overlap Syndrome involving systemic lupus erythematosus and autoimmune hepatitis in an adolescent girl

RESUMEN

La superposición del lupus eritematoso sistémico y la hepatitis autoinmune se describe ocasionalmente. Aunque ambas enfermedades pueden compartir ciertos hallazgos, como poliartralgias, hipergammaglobulinemia y anticuerpo antinúcleo positivo, son consideradas dos diferentes. Se presenta a una paciente de 14 años con lupus eritematoso sistémico, que, luego de dos años, consultó por ictericia. Sin antecedentes de ingesta de drogas, alcohol o exposición a virus hepatotropos. Tenia un aumento de las enzimas hepáticas con anticuerpos antinúcleo, anti-ADN de doble cadena y LKM 1 positivos. La biopsia hepática mostró una hepatitis de interfase con infiltrado linfoplasmocitario. De esta manera, cumplia con los criterios diagnósticos tanto para lupus eritematoso sistémico como para hepatitis autoinmune. Tratada con corticoides y micofenolato mofetil, mejoró su clinica y laboratorio. Conclusión. La hepatitis autoinmune puede ocurrir en el curso del lupus eritematoso sistémico. Un diagnóstico temprano es importante para prevenir el avance de la enfermedad; es obligatoria la realización de la biopsia hepática.

ABSTRACT

Overlap of systemic lupus erythematosus and autoimmune hepatitis is a rare condition, occasionally described. Both autoimmune diseases can share common autoimmune features such as polyartralgia, hypergammaglobulinemia and positive antinuclear antibody, but they have been considered as two different entities. We report a 14 year old female patient with systemic lupus erythematosus who developed autoimmune hepatitis two years later. She had jaundice with no history of viral infections, drug intake, alcohol abuses or exposition to blood products. She was positive for antinuclear antibody, double stranded DNA antibody and LKM 1 antibody. Histological examination of the liver showed interface hepatitis with lymphoplasmacytic cell infiltration. The patient satisfied the international criteria for both systemic lupus erythematosus and Type 2 autoimmune hepatitis. Clinical symptoms and laboratory findings improved under treatment with steroids and mycophenolate mofetil. Conclusion. Autoimmune hepatitis may occur during the course of systemic lupus erythematosus and an early diagnosis is important for preventing advanced liver disease. Definitive diagnosis of autoimmune hepatitis requires confirmation by means of a liver biopsy.