RESUMO
La asociación entre lupus eritematoso sistémico (LES) y púrpura trombótica trombocitopénica (PTT) es una situación descrita pero poco frecuente y suele ocurrir en casos con actividad lúpica y deterioro renal. Presentamos un caso de PTT con disminución de los niveles de ADAMST13 que ocurrió en una paciente afectada de LES pero sin actividad y sin afectación renal. Fue un caso que presentó dificultades diagnósticas iniciales y que, debido a su refractariedad, precisó recambios plasmáticos y tratamientos inmunosupresores, incluyendo rituximab (AU)
The association between systemic lupus erythematosus (SLE) and thrombotic thrombocytopenic purpura (TTP) has been infrequently reported. Usually, patients with TTP have more SLE activity and frequent renal involvement. Here we present a case of TTP associated to low-activity SLE. The absence of renal and major organ involvement increased the difficulty in making the initial diagnosis. ADAMTS13 activity in plasma in this patient was very low, as seen in other similar cases. The evolution of the patient was poor, needing plasma exchanges and immunosuppressive therapy, including the use of rituximab (AU)
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Púrpura Trombocitopênica/complicações , Púrpura Trombocitopênica/diagnóstico , Anemia Hemolítica/complicações , Anemia Hemolítica/diagnóstico , Imunossupressores/uso terapêutico , Corticosteroides/uso terapêutico , Anemia Hemolítica/terapia , Hidroxicloroquina/uso terapêutico , Ciclofosfamida/uso terapêutico , Fibrinolíticos/uso terapêuticoRESUMO
The association between systemic lupus erythematosus (SLE) and thrombotic thrombocytopenic purpura (TTP) has been infrequently reported. Usually, patients with TTP have more SLE activity and frequent renal involvement. Here we present a case of TTP associated to low-activity SLE. The absence of renal and major organ involvement increased the difficulty in making the initial diagnosis. ADAMTS13 activity in plasma in this patient was very low, as seen in other similar cases. The evolution of the patient was poor, needing plasma exchanges and immunosuppressive therapy, including the use of rituximab.
