Microangiopatía trombótica y lupus erimatoso sistémico / Thrombotic microangiopathy and systemic lupus erythematosus

RESUMEN

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 periférico, 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. Continuó en tratamiento dialítico crónico

ABSTRACT

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 hematological commitment with anemia and severe thrombocytopenia, schistocytes in peripheral blood smears, haptoglobin and decreased complement. The diagnosis of thrombotic microangiopathy (TMA) of primary versus secondary cause is proposed. For religious reasons MAT is treated with Rituximab, adding later Eculizumab. No pathological renal anatomy was performed, nor was plasmapheresis performed for this reason. It 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, Rituximab and Eculizumab were discontinued. He continued on chronic dialysis treatment