title: Refractory Angioedema in a Patient with Systemic Lupus Erythematosus.
abstract: Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule. Herein we report a new case of lupus nephritis that developed angioedema and a rapid course of disease progression with acute renal failure and alveolar hemorrhage without response to high dose steroid and plasmapheresis.
authors.affiliation: Department of Rheumatology, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran; ; Department of Internal Medicine, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Zahra
authors.initials: Z
authors.lastname: Habibagahi
authors.affiliation: Department of Nephrology, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran; ; Department of Internal Medicine, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Jamshid
authors.initials: J
authors.lastname: Ruzbeh
authors.affiliation: Department of Internal Medicine, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Vahide
authors.initials: V
authors.lastname: Yarmohammadi
authors.affiliation: Department of Internal Medicine, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Malihe
authors.initials: M
authors.lastname: Kamali
authors.affiliation: Department of Internal Medicine, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Mohammad Hassan
authors.initials: MH
authors.lastname: Rastegar
keywords: Angioedema; Enalapril; Systemic lupus erythematosus