Resumen de Microangiopatía trombótica y lupus eritematoso sistémico

María Laura Argüello Alegre, Cintia Verónica Marin, Marcelo De Rosa, Fernando Segovia

• español

  Paciente de 28 años, Testigo de Jehová, con diagnóstico de lupus eritematoso sistémico con compromiso renal cuatro años antes (nefritis lúpica clase IV con remisión completa). A los 2 años y medio reactiva el compromiso renal y agrega, durante la fase de reinducción, compromiso hematológico con anemia y plaquetopenia severa, esquistocitos en frotis de sangre periférica, haptoglobina y complemento disminuido. Se plantea el diagnóstico de microangiopatía trombótica (MAT) de causa primaria versus secundaria. Por cuestiones religiosas se trata la MAT con Rituximab, sumándose posteriormente Eculizumab. No se realizó anatomía patológica renal, ni se realizó plasmaféresis por dicho motivo. Requirió tratamiento dialítico. El ADAMTS XIII era normal y presentaba importante consumo de C3. A los 6 meses de seguimiento, sin actividad lúpica, plaquetopenia ni anemia hemolítica se discontinuó Rituximab y Eculizumab. Continuóen tratamiento dialítico crónico

• English

  A 28-year-old patient, a Jehovah’s Witness, diagnosed with systemic lupus erythematosus with renal involvement four years earlier (class IV lupus nephritis with complete remission). After 2 and a half years, she reactivates renal involvement and adds during the re-induction phase hema-tological commitment with anemia and severe thrombocytopenia, schistocytes in peripheral blood smears, haptoglobin and decreased comple-ment.The diagnosis of thrombotic microangiopathy (TMA) of primary versus secondary cause is proposed. For religious reasons MAT was treated with Rituximab, adding later Eculizumab. No pathological renal anatomy was performed, nor was plasmapheresis performed for this reason. She required dialytic treatment. ADAMTS XIII was normal and had significant C3 consumption. At 6 months of follow-up, without lupus activity, thrombocytopenia or hemolytic anemia, Ritu-ximab and Eculizumab were discontinued. She continued on chronic dialysis treatment.