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Vol. 12. Issue 1.
Pages 47-49 (January - February 2016)
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Vol. 12. Issue 1.
Pages 47-49 (January - February 2016)
Case Report
DOI: 10.1016/j.reumae.2015.01.010
Protein-losing Enteropathy Associated With Refractory Systemic Lupus Erythematosus With a Good Response to Rituximab
Enteropatía perdedora de proteínas asociada a lupus eritematoso sistémico refractaria con buena respuesta a rituximab
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Pierina Sansinaneaa,
Corresponding author
pierina95@hotmail.com

Corresponding author.
, Sebastián Augusto Carricab, Josefina Marcosa, Mercedes Argentina Garcíaa
a Servicio de Reumatología, Hospital Interzonal General de Agudos (HIGA) General San Martín, La Plata, Buenos Aires, Argentina
b Servicio de Gastroenterología, Hospital Interzonal General de Agudos (HIGA) General San Martín, La Plata, Buenos Aires, Argentina
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Table 1. Changes in Laboratory Parameters and Treatment.
Abstract

A case is presented of a protein-losing enteropathy (PLE) as the initial manifestation of systemic lupus erythematosus (SLE) in a 17 year-old female patient, who presented with ascites, edema and hypoalbuminemia. The diagnosis of SLE was based on the presence of: malar rash, oral ulcers, thrombocytopenia, antinuclear antibodies, IgM anticardiolipin antibody, and lupus anticoagulant. Renal and liver diseases were ruled out. The PLE diagnosis was confirmed with fecal alpha 1-antitrypsin clearance. The PLE was refractory to different lines of immunosuppressive agents like glucocorticoids, cyclophosphamide, azathioprine, and cyclosporine, showing a satisfactory and sustained response with rituximab, allowing steroid sparing and long term remission.

Keywords:
Systemic lupus erythematosus
Protein-losing enteropathy
Rituximab
Refractory
Resumen

Se presenta un caso de enteropatía perdedora de proteínas (EPP)como manifestación inicial del lupus eritematoso sistémico (LES). Se trata de una paciente de sexo femenino, de 17 años de edad, que consulta por síndrome ascítico edematoso e hipoalbuminemia. El diagnóstico de LES se estableció por la presencia de: rash malar, úlceras orales, trombocitopenia, anticuerpos antinucleares, anticardiolipinas IgM y anticoagulante lúpico positivos. Se descartó el compromiso renal y hepático como causa de hipoproteinemia. El diagnóstico de la pérdida enteral de proteínas se realizó con el Clearance de alfa 1 antitripsina. La EPP fue refractaria a distintas líneas de inmunosupresores, como corticoides, ciclofosfamida, azatioprina y ciclosporina, presentando respuesta satisfactoria y sostenida a rituximab, lo que posibilitó la reducción de corticoides y la remisión de la enfermedad por tiempo prolongado.

Palabras clave:
Lupus eritematoso sistémico
Enteropatía perdedora de proteínas
Rituximab
Refractario

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