Sansinanea Pierina, Carrica Sebastián Augusto, Marcos Josefina, García Mercedes Argentina
Servicio de Reumatología, Hospital Interzonal General de Agudos (HIGA) General San Martín, La Plata, Buenos Aires, Argentina.
Servicio de Gastroenterología, Hospital Interzonal General de Agudos (HIGA) General San Martín, La Plata, Buenos Aires, Argentina.
Reumatol Clin. 2016 Jan-Feb;12(1):47-9. doi: 10.1016/j.reuma.2015.01.009. Epub 2015 Mar 26.
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.
本文报告了一例17岁女性系统性红斑狼疮（SLE）患者，以蛋白丢失性肠病（PLE）为首发表现，伴有腹水、水肿和低白蛋白血症。SLE的诊断依据包括：颧部红斑、口腔溃疡、血小板减少、抗核抗体、IgM抗心磷脂抗体和狼疮抗凝物。排除了肾脏和肝脏疾病。通过粪便α1-抗胰蛋白酶清除率确诊为PLE。PLE对糖皮质激素、环磷酰胺、硫唑嘌呤和环孢素等不同系列的免疫抑制剂均难治，而利妥昔单抗显示出满意且持续的反应，可减少类固醇用量并实现长期缓解。
