Case Report: Refractory Angioedema in a Patient with Systemic Lupus Erythematosus

Correspondence: Zahra Habibagahi, MD; Department of Rheumatology and Internal Medicine, Internal Medicine, Zand Street, Shiraz, Iran Tel/Fax:+98 71 36474316 Email: [email protected] Received: 29 April 2013 Revised: 8 September 2013 Accepted: 27 October 2013 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.