ABSTRACT
El Síndrome Púrpura Trombótico Trombocitopénico/Síndrome Hemolítico Urémico (PTT/SHU) es la principal causa de Microangiopatía Trombótica (MAT) en pacientes con Lupus Eritematoso Sistémico (LES). Entre sus manifestaciones destacan la presencia de anemia hemolítica autoinmune, con trombocitopenia y falla renal en grados variables. No existe correlación entre los niveles de actividad de ADAMTS 13 y MAT. Presentamos un caso clínico de MAT asociado a LES. Se debe tener una alta sospecha diagnóstica por la sobreposición de las manifestaciones clínicas de PTT/SHU y LES. El tratamiento con plasmaféresis ha disminuido la mortalidad de 90 por ciento a 15 por ciento. En casos refractarios se ha reportado el uso de Rituximab, aunque aún falta evidencia que lo avale.
The thrombotic Thrombocytopenic Purpura Syndrome / Hemolytic Uremic Syndrome (TTP/HUS) is the main cause behind Thrombotic Microangiopathy (TMA) in patients with Systemic Lupus Erythematosus (SLE). Among the ways in which it manifests itself is the presence of autoimmune hemolytic anemia (AIHA), with thrombocytopenia and kidney failure in various degrees. There is no co-relation between the levels of activity of ADAMTS13 and TMA. We present a clinical case of TMA associated to SLE. A high suspicion is paramount for diagnose due to the overlapping of clinical manifestations of TTP/HUS and SLE. Treatment with plasmapheresis has decreased mortality from 90 percent to 15 percent. Use of Rituximab in refractory cases has been reported, albeit a lack of supporting evidence.
Subject(s)
Humans , Female , Lupus Erythematosus, Systemic/complications , Thrombotic Microangiopathies/diagnosis , Thrombotic Microangiopathies/therapy , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Lupus Erythematosus, Systemic/therapy , Plasmapheresis , PrognosisABSTRACT
Se presenta el caso de una paciente de 35 años con antecedentes de enfermedad de Basedow-Graves, en tratamiento con propiltiouracilo (PTU), consumidora de pasta base y cocaína, que ingresa por fenómenos trombóticos en extremidades. Presenta en forma brusca falla respiratoria severa, que requiere manejo en UCI. Al laboratorio inmunológico destacan: ANA (+), AntiDNA (+), Anti-Ro (+), Anti-cardiolipinas IgG (+). Se realiza una revisión de las entidades autoinmunes más frecuentes asociadas al uso de drogas.
It presents the case of a 35-year old patient with a history of Basedow Graves disease, under treatment with Propiltiouracile (PTU), crack and cocaine user, who was admitted on thrombotic phenomena in her limbs. She presents sudden severe respiratory failure that requires handling at ICU. Under immunologic lab tests, the following stand out: ANA (+), Anti-DNA (+), Anti-Ro (+), Anti-cardiolipines IgG (+). A checkup is made of the most frequent auto-immune entities associated to drug abuse.