Intractable acquired autoimmune angioedema in a patient with systemic lupus erythematosus.
Acquired angioedema is caused by different drugs and lymphoproliferative diseases, and rarely it has also been related to the presence of auto-immune disorders. We report the case of a 47 year old female with systemic lupus erythematosus (SLE) and severe cutaneous involvement who developed recurrent localized angioedema of the face, including lips and eye lids, upper limbs, and thorax, not associated with urticaria, and with reduced levels of C1 esterase inhibitor. Treatment with antimalarials, glucocorticoids, and pulse therapy with methylprednisolone associated with azathioprine did not improve her condition. The patient was also unsuccessfully treated with danazol, and she only showed clinical response after several sessions of plasmapheresis, including resolution of the extensive edema of the gastrointestinal tract.
Núcleo de Reumatologia, Hospital Universitário, Universidade Federal de Santa Catarina.
Vilson
V
Furlanetto
Karina de Souza
Kde S
Giassi
Fabrício de Souza
Fde S
Neves
Adriana Fontes
AF
Zimmermann
Gláucio Ricardo Werner
GR
Castro
Ivânio Alves
IA
Pereira
D000799:Angioedema / Q000209:etiology; D001327:Autoimmune Diseases / Q000209:etiology*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008875:Middle Aged