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Inicio Revista Colombiana de Reumatología Síndrome pulmón-riñón en lupus severo asociado a ANCA: ¿un síndrome de sob...
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Vol. 20. Issue 1.
Pages 64-71 (March 2013)
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Vol. 20. Issue 1.
Pages 64-71 (March 2013)
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Síndrome pulmón-riñón en lupus severo asociado a ANCA: ¿un síndrome de sobreposición?
Lung - kidney syndrome in severe lupus ANCA associated: overlap syndrome?
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Nasser Abdel Polanco Flores1,
Corresponding author
, María Virgilia Soto Abraham2, Francisco Eugenio Rodríguez Castellanos1
1 Servicio de Nefrología, Instituto Nacional de Cardiología Dr. Ignacio Chávez, México D.F
2 Servicio de Patología, Instituto Nacional de Cardiología Dr. Ignacio Chávez, México D.F
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Resumen

Presentamos el caso de una mujer de 30 años, mexicana, que luego de permanecer sin datos de actividad lúpica renal por 13 años, desde su diagnóstico, sin inmunosupresión crónica, experimenta un deterioro general por un estado autoinmune severo desencadenado tras un embarazo a término sin complicaciones, presentando, incluso, hemorragia alveolar difusa en 2 ocasiones, durante 12 meses, en el contexto de anticuerpos anti citoplasma de neutrófilos ANCA (+), volviendo su manejo difícil desde el punto de vista terapéutico con esquemas de inmunosupresión triple y cuádruple. Durante su evolución ha desarrollado microangiopatía cerebral y laringitis, por lo que se plantea la posibilidad de un síndrome de sobreposición lupus eritematoso sistémico – vasculitispauciinmune, una entidad no bien descrita en la literatura, cuyo diagnóstico está plagado de dificultades al no existir criterios definidos, ni la verdadera implicación de los ANCA en un paciente con lupus.

Palabras clave:
Lupus
nefritislúpica
vasculitis
hemorragia pulmonar
ANCA
síndrome de sobreposición
Summary

We report the case of a woman of 30 years, Mexican, who after spending without renal lupus activity data for 13 years after diagnosis, without chronic immunosuppression, experienced a general decline for severe autoimmune condition triggered after an uncomplicated term pregnancy presenting even diffuse alveolar hemorrhage in 2 occasions within 12 months, in the context of Anti neutrophil cytoplasmic ANCA (+), becoming its handling difficult from the point of view of therapeutic immunosuppression regimens triple and quadruple. During its evolution has developed cerebral microangiopathy and laryngitis which raises the possibility of an overlap syndrome lupuserythematosus – ANCA associated vasculitis, an entity not well described in the literature, whose diagnosis is fraught with difficulties in the absence of defined criteria or true ANCA involvement in a patient with lupus.

Key words:
Lupus nephritis
vasculitis
pulmonary hemorrhage
ANCA
overlap syndrome
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